RIPK1

gene
On this page

Also known as RIPRIP1RIP-1

Summary

RIPK1 (receptor interacting serine/threonine kinase 1, HGNC:10019) is a protein-coding gene on chromosome 6p25.2, encoding Receptor-interacting serine/threonine-protein kinase 1 (Q13546). Serine-threonine kinase which is a key regulator of TNF-mediated apoptosis, necroptosis and inflammatory pathways.

This gene encodes a member of the receptor-interacting protein (RIP) family of serine/threonine protein kinases. The encoded protein plays a role in inflammation and cell death in response to tissue damage, pathogen recognition, and as part of developmental regulation. RIPK1/RIPK3 kinase-mediated necrosis is referred to as necroptosis. Genetic disruption of this gene in mice results in death shortly after birth.

Source: NCBI Gene 8737 — RefSeq curated summary.

At a glance

  • Gene–disease (curated): immunodeficiency 57 (Definitive, ClinGen) — +2 more curated relationships
  • GWAS associations: 2
  • Clinical variants (ClinVar): 588 total — 22 pathogenic, 7 likely-pathogenic
  • Phenotypes (HPO): 28
  • Druggable target: yes — 24 molecules with ChEMBL bioactivity
  • Cancer driver (intOGen): loss-of-function (tumor-suppressor-like) across 1 cancer types
  • MANE Select transcript: NM_001354930

Identifiers

Gene identifiers

FieldValue
HGNC IDHGNC:10019
Approved symbolRIPK1
Namereceptor interacting serine/threonine kinase 1
Location6p25.2
Locus typegene with protein product
StatusApproved
AliasesRIP, RIP1, RIP-1
Ensembl geneENSG00000137275
Ensembl biotypeprotein_coding
OMIM603453
Entrez8737

Gene structure

Transcript identifiers

Ensembl transcripts: 36 — 19 protein_coding, 8 nonsense_mediated_decay, 5 protein_coding_CDS_not_defined, 4 retained_intron

ENST00000259808, ENST00000380409, ENST00000479389, ENST00000676591, ENST00000676618, ENST00000676758, ENST00000676965, ENST00000676995, ENST00000677261, ENST00000677361, ENST00000677799, ENST00000678084, ENST00000678809, ENST00000678874, ENST00000679118, ENST00000679335, ENST00000679677, ENST00000679775, ENST00000680389, ENST00000858490, ENST00000858491, ENST00000858492, ENST00000858493, ENST00000858494, ENST00000858495, ENST00000919965, ENST00000919966, ENST00000967576, ENST00000967577, ENST00000967578, ENST00000967579, ENST00000967580, ENST00000967581, ENST00000967582, ENST00000967583, ENST00000967584

RefSeq mRNA: 7 — MANE Select: NM_001354930 NM_001317061, NM_001354930, NM_001354931, NM_001354932, NM_001354933, NM_001354934, NM_003804

CCDS: CCDS4482, CCDS93851

Canonical transcript exons

ENST00000259808 — 11 exons

ExonStartEnd
ENSE0000092860331108033110955
ENSE0000092861130767643076987
ENSE0000148481830684943068661
ENSE0000152980731130533115187
ENSE0000353385531054823106051
ENSE0000355497730777793077935
ENSE0000357587330809793081116
ENSE0000358616230830853083313
ENSE0000361877331042253104315
ENSE0000363781930852593085408
ENSE0000364721130895813089657

Expression profiles

Bgee: expression breadth ubiquitous, 238 present calls, max score 91.05.

FANTOM5 (CAGE): breadth ubiquitous, TPM avg 23.8449 / max 1044.9606, expressed in 1820 samples.

FANTOM5 promoters (5 alternative TSS)

Promoter IDTPM avgSamples expressed
6551817.31451817
655155.35521048
655160.7808146
655190.2557107
655170.138769

Top tissues by expression

277 total, by Bgee expression score (0-100, higher = more expressed):

TissueAnatomy IDExpression scoreQuality
granulocyteCL:000009491.05gold quality
sural nerveUBERON:001548890.39gold quality
right lobe of liverUBERON:000111488.92gold quality
stromal cell of endometriumCL:000225588.60gold quality
mucosa of stomachUBERON:000119988.48gold quality
calcaneal tendonUBERON:000370188.37gold quality
small intestine Peyer’s patchUBERON:000345488.07gold quality
right coronary arteryUBERON:000162588.02gold quality
skin of abdomenUBERON:000141687.97gold quality
omental fat padUBERON:001041487.88gold quality
peritoneumUBERON:000235887.85gold quality
skin of legUBERON:000151187.75gold quality
lower esophagus muscularis layerUBERON:003583387.70gold quality
lower esophagusUBERON:001347387.69gold quality
apex of heartUBERON:000209887.67gold quality
descending thoracic aortaUBERON:000234587.64gold quality
transverse colonUBERON:000115787.45gold quality
rectumUBERON:000105287.39gold quality
tibial arteryUBERON:000761087.38gold quality
popliteal arteryUBERON:000225087.36gold quality
mucosa of transverse colonUBERON:000499187.35gold quality
esophagogastric junction muscularis propriaUBERON:003584187.31gold quality
subcutaneous adipose tissueUBERON:000219087.24gold quality
muscle layer of sigmoid colonUBERON:003580587.21gold quality
esophagusUBERON:000104387.08gold quality
adipose tissue of abdominal regionUBERON:000780887.08gold quality
body of stomachUBERON:000116187.03gold quality
aortaUBERON:000094787.00gold quality
gall bladderUBERON:000211086.82gold quality
small intestineUBERON:000210886.73gold quality

Single-cell (SCXA)

Detected in 2 experiment(s), a significant marker in 1.

ExperimentMarker?Max mean expression
E-CURD-10no146.63
E-ANND-3no0.00

Regulation

Is transcription factor: no

Upstream regulators (CollecTRI, top): AP1, NFKB1, PDX1, REL, SP1, STAT1, ZGLP1

miRNA regulators (miRDB)

49 targeting RIPK1, top 30 by miRDB confidence (max_score; target_count = how many genes the miRNA targets in total — lower means more specific):

miRNAMax scoreAvg scoremiRNA target_count
HSA-MIR-4682100.0068.891258
HSA-MIR-432-3P100.0067.86705
HSA-MIR-318599.9968.121959
HSA-MIR-1213699.9872.815713
HSA-MIR-3065-5P99.9771.563281
HSA-MIR-153-5P99.8973.866317
HSA-MIR-449299.8768.253611
HSA-MIR-548AZ-5P99.8369.943230
HSA-MIR-548T-5P99.8369.913220
HSA-MIR-5004-5P99.6866.631294
HSA-MIR-317599.6566.302031
HSA-MIR-3679-3P99.6469.881599
HSA-MIR-426199.5970.303415
HSA-MIR-7159-5P99.5372.122472
HSA-MIR-642A-5P99.5165.101152
HSA-MIR-6740-3P99.4868.491392
HSA-MIR-766-3P99.4765.241811
HSA-MIR-147B-5P99.4570.622432
HSA-MIR-4797-5P99.3968.011354
HSA-MIR-450599.2767.812678
HSA-MIR-569399.2466.671106
HSA-MIR-664A-3P99.2271.082696
HSA-MIR-578799.2267.862628
HSA-MIR-21-3P99.2168.951312
HSA-MIR-122B-3P99.2168.901333
HSA-MIR-66199.0965.942062
HSA-MIR-465698.7966.221306
HSA-MIR-5006-5P98.7966.921246
HSA-MIR-502-5P98.7766.51906
HSA-MIR-4700-5P98.6367.431915

Literature-anchored findings (GeneRIF, showing 40)

  • cells pre-stimulated through TNFR-2 prior to subsequent activation of TNFR-1, showed enhanced cell death and recruitment of RIP to the TNFR-1 complex; deficiency of RIP rescued the infected cells from TNF-induced cytotoxicity (PMID:14532286)
  • FAK overexpression in human tumors provides a survival signal function by binding to RIP and inhibiting its interaction with the death receptor complex. (PMID:15121855)
  • RIP-dependent recruitment of MEKK3 plays a specific role in TNF-alpha signaling. (PMID:15572679)
  • TAK1 is recruited to the TNF-R1 complex via RIP and likely cooperates with MEKK3 to activate NF-kappaB in TNF-alpha signaling (PMID:16260783)
  • This study provides the first genetic evidence that the ubiquitination of RIP is required for TNFalpha-induced NF-kappaB activation. (PMID:16543241)
  • Activation of IKK by TNF-alpha requires site-specific ubiquitination of RIP1. (PMID:16603398)
  • TRADD and RIP1 compete for recruitment to the TNFR1 signaling complex and the distinct programs of cell death. (PMID:16611992)
  • RIP is one of the critical components involved in mediating DNA damage-induced, p53-independent cell death (PMID:16825191)
  • The expression of receptor-interacting protein 1 (RIP1) in peripheral blood mononuclear cells was significantly decreased in SLE patients. (PMID:17235653)
  • RIP may serve as a general activator of IRF7, responding to and transmitting the signals from various stimuli, and that ubiquitination may be a general mechanism for enhancing the activity of IRF7. (PMID:17296724)
  • We describe how RIP1 acts as a key integrator of signalling pathways initiated by stimulation of death receptors, bacterial or viral infection, genotoxic stress and T-cell homeostasis. (PMID:17301840)
  • findings show ubiquitination of RIP1 inhibits TNF-induced apoptosis first through an NF-kappaB-independent mechanism, then through an NF-kappaB-dependent mechanism; in absence of ubiquitination, RIP1 serves as a proapoptotic signaling molecule (PMID:17306544)
  • TNF-induced TRAF2-RIP1-AIP1-ASK1 complex formation and for the activation of ASK1-JNK/p38 apoptotic signaling. (PMID:17389591)
  • TNFRSF is upregulated after radical prostatectomy for prostatic neoplasms. (PMID:17448597)
  • RIP and c-FLIP-mediated assembly of the death-inducing signaling complex in nonrafts is a critical upstream event in TRAIL resistance (PMID:17638906)
  • These results suggested that HPB-ALL cells have a caspase-independent RIP kinasedependent pathway for Fas ligation. (PMID:17951993)
  • In response to autocrine TNFalpha signaling, the Smac mimetic promotes formation of a RIPK1-dependent caspase-8-activating complex, leading to apoptosis (PMID:17996648)
  • Data demonstrated that CK1alpha interacted with and phosphorylated RIP1 at the intermediate domain. Finally, we showed that CK1alpha enhanced RIP1-mediated NF-kappaB activation. (PMID:18067272)
  • Cezanne forms a novel negative feedback loop in pro-inflammatory signaling and that it suppresses NF-kappaB activation by targeting RIP1 signaling intermediaries for deubiquitination (PMID:18178551)
  • cytomegalovirus M45 protein provides a direct cell type-dependent replication benefit to the virus while modulating other biological processes signaling via the RIP1 adaptor such as activation of Toll-like receptor (TLR)3 and other mediators of cell death (PMID:18442983)
  • activation and formation of TICAM-1 signalosomes with NF-kappaB and interferon regulatory factor-3 requires oligomerization induced at two different sites and RIP1 binding (PMID:18450748)
  • cIAP1 and cIAP2 promote cancer cell survival by functioning as E3 ubiquitin ligases that maintain constitutive ubiquitination of the RIP1 adaptor protein. (PMID:18570872)
  • c-IAP1 and c-IAP2 are required for TNFalpha-stimulated RIP1 ubiquitination and NF-kappaB activation. (PMID:18621737)
  • The protein encoded by the hybrid transcript lacks the putative kinase domain of RIP1, but potently stimulates NFkappaB, AP-1 and Ets-1 activity. (PMID:18635966)
  • the simultaneous downregulation of uPAR and MMP-9 induces apoptosome-mediated apoptosis through FADD-associated protein RIP and caspase 9 (PMID:18813792)
  • Induction of NF-kappa B depends upon the adaptor receptor-interacting protein kinase (RIP)1, acting via a RIP homotypic interaction motif-dependent interaction with DNA-dependent activator of IFN regulatory factors (DAI). (PMID:18941233)
  • glomerular and tubular expression of TNF-alpha, (TNF receptor-associated death domain protein)TRADD, RIP(receptor-interacting protein) and TRAF-2 (TNF receptor-associated factor-) was significantly up-regulated in Lupus nephritis (PMID:19151112)
  • Data show that the polymorphisms of LTA (rs1041981) and RIPK1 (rs2272990) may correlate with TTP in patients with DLBCL treated with R-CHOP. (PMID:19629486)
  • RIP1 is important in maintaining resistance to TRAIL-induced apoptosis in breast tumor cells and highlight the potential therapeutic benefit of the combination of Hsp90 inhibitors and TRAIL against breast tumor cells. (PMID:19632771)
  • RIP1 is necessary for the most efficient activation of downstream caspases by Fas when treated with membrane-bound Fas ligand, but not with agonistic antibodies or cross-linked soluble Fas ligand. (PMID:19641134)
  • Both RIP and caspase-8 may play important roles in the occurrence and progression of oral squamous cell carcinoma and oral precancerous lesions. (PMID:19778795)
  • USP21 plays an important role in the down-regulation of TNFalpha-induced NF-kappaB activation through deubiquitinating RIP1. (PMID:19910467)
  • RIP1 and RIP3 have roles in TNF-induced necrosis (PMID:20354226)
  • Data show that miR-155 can inhibit necrosis and improve cell survival by repressing the receptor interacting protein1, RIP1, without affecting cardiomyogenic differentiation potential. (PMID:20550618)
  • The RIPK1 and CASP7 polymorphisms can be considered as possible prognostic markers for survival after curative resection in patients with colorectal cancer. (PMID:20567846)
  • Results indicate an important role of RIP1 in apoptosis induced by combined treatment with TNFalpha and MeBS. (PMID:20825417)
  • The authors demonstrate that c-IAP1 and UbcH5 family promote K11-linked polyubiquitination of receptor-interacting protein 1 (RIP1) in vitro and in vivo. (PMID:21113135)
  • RIP1-dependent caspase activation contributes to polyinosinic-polycytidylic acid-induced soluble tumor necrosis factor receptor 1 (TNFR1) shedding indicative of innate immune responses to viral infections in human airway epithelial (NCI-H292) cells. (PMID:21148036)
  • TNFalpha-duced NF-kappaB activation is attenuated by RIP1 through stabilization of TRAF2. (PMID:21266470)
  • The death domain kinase RIP1 links the immunoregulatory CD40 receptor to apoptotic signaling in carcinomas (PMID:21282461)

Cross-species orthologs

3 orthologs

OrganismSymbolGene ID
danio_rerioripk1lENSDARG00000006677
mus_musculusRipk1ENSMUSG00000021408
rattus_norvegicusRipk1ENSRNOG00000017787

Paralogs (23): MAP3K9 (ENSG00000006432), TESK2 (ENSG00000070759), MAP3K13 (ENSG00000073803), ARAF (ENSG00000078061), MAP3K20 (ENSG00000091436), RIPK2 (ENSG00000104312), LIMK1 (ENSG00000106683), TESK1 (ENSG00000107140), TNNI3K (ENSG00000116783), RIPK3 (ENSG00000129465), MAP3K10 (ENSG00000130758), RAF1 (ENSG00000132155), MAP3K12 (ENSG00000139625), KSR1 (ENSG00000141068), MAP3K21 (ENSG00000143674), BRAF (ENSG00000157764), ILK (ENSG00000166333), MLKL (ENSG00000168404), KSR2 (ENSG00000171435), MOS (ENSG00000172680), MAP3K11 (ENSG00000173327), LIMK2 (ENSG00000182541), LRRK2 (ENSG00000188906)

Protein

Protein identifiers

Receptor-interacting serine/threonine-protein kinase 1Q13546 (reviewed: Q13546)

Alternative names: Cell death protein RIP, Receptor-interacting protein 1

All UniProt accessions (3): Q13546, A0A7I2V404, A0A7I2V5Y1

UniProt curated annotations — full annotation on UniProt →

Function. Serine-threonine kinase which is a key regulator of TNF-mediated apoptosis, necroptosis and inflammatory pathways. Exhibits kinase activity-dependent functions that regulate cell death and kinase-independent scaffold functions regulating inflammatory signaling and cell survival. Has kinase-independent scaffold functions: upon binding of TNF to TNFR1, RIPK1 is recruited to the TNF-R1 signaling complex (TNF-RSC also known as complex I) where it acts as a scaffold protein promoting cell survival, in part, by activating the canonical NF-kappa-B pathway. Kinase activity is essential to regulate necroptosis and apoptosis, two parallel forms of cell death: upon activation of its protein kinase activity, regulates assembly of two death-inducing complexes, namely complex IIa (RIPK1-FADD-CASP8), which drives apoptosis, and the complex IIb (RIPK1-RIPK3-MLKL), which drives necroptosis. RIPK1 is required to limit CASP8-dependent TNFR1-induced apoptosis. In normal conditions, RIPK1 acts as an inhibitor of RIPK3-dependent necroptosis, a process mediated by RIPK3 component of complex IIb, which catalyzes phosphorylation of MLKL upon induction by ZBP1. Inhibits RIPK3-mediated necroptosis via FADD-mediated recruitment of CASP8, which cleaves RIPK1 and limits TNF-induced necroptosis. Required to inhibit apoptosis and necroptosis during embryonic development: acts by preventing the interaction of TRADD with FADD thereby limiting aberrant activation of CASP8. In addition to apoptosis and necroptosis, also involved in inflammatory response by promoting transcriptional production of pro-inflammatory cytokines, such as interleukin-6 (IL6). Phosphorylates RIPK3: RIPK1 and RIPK3 undergo reciprocal auto- and trans-phosphorylation. Phosphorylates DAB2IP at ‘Ser-728’ in a TNF-dependent manner, and thereby activates the MAP3K5-JNK apoptotic cascade. Required for ZBP1-induced NF-kappa-B activation in response to DNA damage.

Subunit / interactions. Homodimer. Interacts (via RIP homotypic interaction motif) with RIPK3 (via RIP homotypic interaction motif); this interaction induces RIPK1 phosphorylation and formation of a RIPK1-RIPK3 necroptosis-inducing complex. Upon TNF-induced necrosis, the RIPK1-RIPK3 dimer further interacts with PGAM5 and MLKL; the formation of this complex leads to PGAM5 phosphorylation and increase in PGAM5 phosphatase activity. Interacts (via the death domain) with TNFRSF6 (via the death domain) and TRADD (via the death domain). Is recruited by TRADD to TNFRSF1A in a TNF-dependent process. Binds RNF216, EGFR, IKBKG, TRAF1, TRAF2 and TRAF3. Interacts with BNLF1. Interacts with SQSTM1 upon TNF stimulation. May interact with MAVS/IPS1. Interacts with ZFAND5. Interacts with RBCK1. Interacts with ZBP1. Interacts with BIRC2/c-IAP1, BIRC3/c-IAP2 and XIAP/BIRC4. Interacts (via kinase domain) with DAB2IP (via Ras-GAP domain); the interaction occurs in a TNF-dependent manner. Interacts with ARHGEF2. Interacts (via protein kinase domain) with RFFL; involved in RIPK1 ubiquitination. Interacts with RNF34; involved in RIPK1 ubiquitination (Ref.35). Interacts with TICAM1 and this interaction is enhanced in the presence of WDFY1. Interacts with PELI1. Interacts (via death domain) with CRADD (via death domain); the interaction is direct. Component of complex IIa composed of at least RIPK1, FADD and CASP8. Component of the AIM2 PANoptosome complex, a multiprotein complex that drives inflammatory cell death (PANoptosis). Interacts with MAP3K7, CFLAR, CASP8, FADD and NEMO. Interacts with TAX1BP1; this interaction negatively regulates RIPK1 ubiquitination. Interacts with GRB2. Interacts with DDX24; this interaction disrupts RLR signaling activation of IFN-dependent transcription factor IRF7. (Microbial infection) Interacts with mumps virus protein SH; this interaction inhibits downstream NF-kappa-B pathway activation. (Microbial infection) Interacts with Murid herpesvirus 1 protein RIR1. (Microbial infection) Interacts (via RIP homotypic interaction motif) with herpes simplex virus 1/HHV-1 protein RIR1/ICP6 (via RIP homotypic interaction motif); this interaction prevents necroptosis activation. (Microbial infection) Interacts (via RIP homotypic interaction motif) with herpes simplex virus 2/HHV-2 protein RIR1/ICP10 (via RIP homotypic interaction motif); this interaction prevents necroptosis activation.

Subcellular location. Cytoplasm. Cell membrane.

Post-translational modifications. (Microbial infection) Proteolytically cleaved by S.flexneri OspD3 within the RIP homotypic interaction motif (RHIM), leading to its degradation and inhibition of necroptosis. Proteolytically cleaved by CASP8 at Asp-324. Cleavage is crucial for limiting TNF-induced apoptosis, necroptosis and inflammatory response. Cleavage abolishes NF-kappa-B activation and enhances the interaction of TRADD with FADD. Proteolytically cleaved by CASP6 during intrinsic apoptosis. RIPK1 and RIPK3 undergo reciprocal auto- and trans-phosphorylation. Phosphorylation of Ser-161 by RIPK3 is necessary for the formation of the necroptosis-inducing complex. Phosphorylation at Ser-25 represses its kinase activity and consequently prevents TNF-mediated RIPK1-dependent cell death. Phosphorylated at Ser-320 by MAP3K7 which requires prior ubiquitination with ‘Lys-63’-linked chains by BIRC2/c-IAP1 and BIRC3/c-IAP2. This phosphorylation positively regulates RIPK1 interaction with RIPK3 to promote necroptosis but negatively regulates RIPK1 kinase activity and its interaction with FADD to mediate apoptosis. Deubiquitinated by USP7; this modification is required for TNF-induced apoptosis. Ubiquitinated with ‘Lys-11’-, ‘Lys-48’-, ‘Lys-63’- and linear-linked type ubiquitin (PubMed:15258597, PubMed:16603398, PubMed:17703191, PubMed:18450452, PubMed:21455173, PubMed:21931591, PubMed:29883609, Ref.35). Polyubiquitination with ‘Lys-63’-linked chains by TRAF2 induces association with the IKK complex. Deubiquitination of ‘Lys-63’-linked chains and polyubiquitination with ‘Lys-48’-linked chains by TNFAIP3 leads to RIPK1 proteasomal degradation and consequently down-regulates TNF-induced NF-kappa-B signaling. ‘Lys-48’-linked polyubiquitination by RFFL or RNF34 also promotes proteasomal degradation and negatively regulates TNF-induced NF-kappa-B signaling (PubMed:18450452, Ref.35). Linear polyubiquitinated; the head-to-tail linear polyubiquitination (‘Met-1’-linked) is mediated by the LUBAC complex and decreases protein kinase activity. Deubiquitination of linear polyubiquitin by CYLD promotes the kinase activity. Polyubiquitinated with ‘Lys-48’ and ‘Lys-63’-linked chains by BIRC2/c-IAP1 and BIRC3/c-IAP2, leading to activation of NF-kappa-B. Ubiquitinated with ‘Lys-63’-linked chains by PELI1. Ubiquitination at Lys-377 with ‘Lys-63’-linked chains by BIRC2/c-IAP1 and BIRC3/c-IAP2 is essential for its phosphorylation at Ser-320 mediated by MAP3K7. This ubiquitination is required for NF-kB activation, suppresses RIPK1 kinase activity and plays a critical role in preventing cell death during embryonic development. (Microbial infection) Glycosylated at Arg-603 by enteropathogenic E.coli protein NleB1: arginine GlcNAcylation prevents homotypic/heterotypic death domain interactions.

Disease relevance. Immunodeficiency 57 with autoinflammation (IMD57) [MIM:618108] An autosomal recessive primary immunodeficiency characterized by lymphopenia and recurrent viral, bacterial, and fungal infections. Patients exhibit early-onset inflammatory bowel disease involving the upper and lower gastrointestinal tract, and develop progressive polyarthritis. The disease is caused by variants affecting the gene represented in this entry. RIPK1-deficient immune cells from IMD57 patients have impaired proinflammatory signaling leading to dysregulated cytokine secretion and are prone to necroptosis. Autoinflammation with episodic fever and lymphadenopathy (AIEFL) [MIM:618852] An autosomal dominant immunologic disorder characterized by early onset of recurrent episodes of unexplained fever, lymphadenopathy, hepatosplenomegaly, and increased levels of inflammatory cytokines and chemokines in patient serum. The disease is caused by variants affecting the gene represented in this entry.

Activity regulation. Serine-threonine kinase activity is inhibited by linear polyubiquitination (‘Met-1’-linked) by the LUBAC complex. Inhibited by necrostatins, including necrostatin-1, necrostatin-3 and necrostatin-4.

Domain organisation. The RIP homotypic interaction motif (RHIM) mediates interaction with the RHIM motif of RIPK1. Both motifs form a hetero-amyloid serpentine fold, stabilized by hydrophobic packing and featuring an unusual Cys-Ser ladder of alternating Ser (from RIPK1) and Cys (from RIPK3). The death domain mediates dimerization and activation of its kinase activity during necroptosis and apoptosis. It engages other DD-containing proteins as well as a central (intermediate) region important for NF-kB activation and RHIM-dependent signaling.

Similarity. Belongs to the protein kinase superfamily. TKL Ser/Thr protein kinase family.

Isoforms (2)

UniProt IDNamesCanonical?
Q13546-11yes
Q13546-22

RefSeq proteins (7): NP_001303990, NP_001341859, NP_001341860, NP_001341861, NP_001341862, NP_001341863, NP_003795 (=MANE)

Domains & families (InterPro)

IDNameType
IPR000488Death_domDomain
IPR000719Prot_kinase_domDomain
IPR001245Ser-Thr/Tyr_kinase_cat_domDomain
IPR008271Ser/Thr_kinase_ASActive_site
IPR011009Kinase-like_dom_sfHomologous_superfamily
IPR011029DEATH-like_dom_sfHomologous_superfamily
IPR037934RIP1_DeathDomain
IPR051681Ser/Thr_Kinases-PseudokinasesFamily

Pfam: PF00531, PF07714

Enzyme classification (BRENDA):

  • EC 2.7.10.2 — non-specific protein-tyrosine kinase (BRENDA: 41 organisms, 396 substrates, 479 inhibitors, 43 Km, 32 kcat entries)

Substrate kinetics (BRENDA)

6 substrates with measured Km, best-characterized 6. Km ranges are aggregated across organisms/conditions.

SubstrateKm (mM)Measurements
ATP0.014–17.6412
[KDSRC KINASE]-L-TYROSINE0.0057–0.2412
POLY(GLU4-TYR)0.018–0.65910
EEEEYIQ[DP]-8-HYDROXY-5-(N,N-DIMETHYLSULFONAMIDO0.0571
S1 PEPTIDE0.0371
EEEEY0

Catalyzed reactions (Rhea), 2 shown:

  • L-seryl-[protein] + ATP = O-phospho-L-seryl-[protein] + ADP + H(+) (RHEA:17989)
  • L-threonyl-[protein] + ATP = O-phospho-L-threonyl-[protein] + ADP + H(+) (RHEA:46608)

UniProt features (94 total): helix 25, strand 14, sequence variant 12, modified residue 10, mutagenesis site 10, turn 4, region of interest 3, sequence conflict 3, domain 2, binding site 2, compositionally biased region 2, chain 1, site 1, glycosylation site 1, cross-link 1, splice variant 1, short sequence motif 1, active site 1

Structure

Experimental structures (PDB)

39 structures, top 30 by resolution.

PDBMethodResolution (Å)
5ZMZX-RAY DIFFRACTION1.4
6AC5X-RAY DIFFRACTION1.45
4ITJX-RAY DIFFRACTION1.8
6NW2X-RAY DIFFRACTION2
7FD0X-RAY DIFFRACTION2
24IBX-RAY DIFFRACTION2.05
9Q31X-RAY DIFFRACTION2.05
6NYHX-RAY DIFFRACTION2.1
9GTYX-RAY DIFFRACTION2.15
7FCZX-RAY DIFFRACTION2.21
5HX6X-RAY DIFFRACTION2.23
4ITHX-RAY DIFFRACTION2.25
9GTGX-RAY DIFFRACTION2.25
8I2NX-RAY DIFFRACTION2.29
9HY9X-RAY DIFFRACTION2.29
9MZYX-RAY DIFFRACTION2.32
7XMKX-RAY DIFFRACTION2.38
9Q32X-RAY DIFFRACTION2.49
6C4DX-RAY DIFFRACTION2.52
9MZXX-RAY DIFFRACTION2.53
5TX5X-RAY DIFFRACTION2.56
4NEUX-RAY DIFFRACTION2.57
9HR6ELECTRON MICROSCOPY2.57
6C3EX-RAY DIFFRACTION2.6
7YDXX-RAY DIFFRACTION2.64
9MZZX-RAY DIFFRACTION2.68
24ICX-RAY DIFFRACTION2.7
6OCQX-RAY DIFFRACTION2.79
7CJBX-RAY DIFFRACTION2.8
4ITIX-RAY DIFFRACTION2.86

Predicted structure (AlphaFold)

ModelpLDDTFraction very-high
AF-Q13546-F170.380.33

Functional residue map

Curated UniProt residues grouped by drug-discovery relevance — catalytic, ligand-binding, modification, and mutation-validated positions. Source: UniProtKB sequence features.

Catalytic / active sites (2): 324–325 (cleavage; by casp8); 138 (proton acceptor)

Ligand- & substrate-binding residues (2): 23–31; 45

Post-translational modifications (11): 6, 20, 25, 161, 166, 303, 320, 331, 333, 384, 377

Glycosylation sites (1): 603

Mutagenesis-validated functional residues (10):

PositionPhenotype
25phophomimetic mutant. significant loss of kinase activity.
45abolishes kinase activity.
161decreases ripk1 kinase activity.
161no effect on ripk1 autophosphorylation.
324abolishes cleavage by caspase-8.
377abolishes rip-mediated nf-kappa-b activation.
536strongly reduced homodimerization and interaction with ripk3.
539–542abolished cleavage by s.flexneri ospd3.
599blocks homodimerization, necroptosis and apoptosis.
603abolished glcnacylation by e.coli nleb1.

Function

Pathways and Gene Ontology

Reactome pathways

24 pathways

IDPathway
R-HSA-140534Caspase activation via Death Receptors in the presence of ligand
R-HSA-168927TICAM1, RIP1-mediated IKK complex recruitment
R-HSA-1810476RIP-mediated NFkB activation via ZBP1
R-HSA-2562578TRIF-mediated programmed cell death
R-HSA-3295583TRP channels
R-HSA-3371378Regulation by c-FLIP
R-HSA-5213460RIPK1-mediated regulated necrosis
R-HSA-5218900CASP8 activity is inhibited
R-HSA-5357786TNFR1-induced proapoptotic signaling
R-HSA-5357905Regulation of TNFR1 signaling
R-HSA-5357956TNFR1-induced NF-kappa-B signaling pathway
R-HSA-5675482Regulation of necroptotic cell death
R-HSA-5689880Ub-specific processing proteases
R-HSA-5689896Ovarian tumor domain proteases
R-HSA-69416Dimerization of procaspase-8
R-HSA-75893TNF signaling
R-HSA-9013957TLR3-mediated TICAM1-dependent programmed cell death
R-HSA-933543NF-kB activation through FADD/RIP-1 pathway mediated by caspase-8 and -10
R-HSA-937041IKK complex recruitment mediated by RIP1
R-HSA-9679191Potential therapeutics for SARS
R-HSA-9686347Microbial modulation of RIPK1-mediated regulated necrosis
R-HSA-9692913SARS-CoV-1-mediated effects on programmed cell death
R-HSA-9693928Defective RIPK1-mediated regulated necrosis
R-HSA-9920951Dengue virus modulates apoptosis

MSigDB gene sets: 0 (showing top):

GO Biological Process (56): positive regulation of protein phosphorylation (GO:0001934), apoptotic process (GO:0006915), inflammatory response (GO:0006954), response to oxidative stress (GO:0006979), canonical NF-kappaB signal transduction (GO:0007249), positive regulation of gene expression (GO:0010628), protein catabolic process (GO:0030163), positive regulation of interleukin-8 production (GO:0032757), positive regulation of tumor necrosis factor production (GO:0032760), tumor necrosis factor-mediated signaling pathway (GO:0033209), response to tumor necrosis factor (GO:0034612), intracellular signal transduction (GO:0035556), peptidyl-serine autophosphorylation (GO:0036289), positive regulation of apoptotic process (GO:0043065), negative regulation of apoptotic process (GO:0043066), positive regulation of programmed cell death (GO:0043068), positive regulation of canonical NF-kappaB signal transduction (GO:0043123), negative regulation of canonical NF-kappaB signal transduction (GO:0043124), positive regulation of neuron apoptotic process (GO:0043525), positive regulation of macrophage differentiation (GO:0045651), positive regulation of transcription by RNA polymerase II (GO:0045944), positive regulation of JNK cascade (GO:0046330), protein autophosphorylation (GO:0046777), positive regulation of inflammatory response (GO:0050729), obsolete positive regulation of NF-kappaB transcription factor activity (GO:0051092), positive regulation of necroptotic process (GO:0060545), negative regulation of necroptotic process (GO:0060546), positive regulation of programmed necrotic cell death (GO:0062100), positive regulation of interleukin-6-mediated signaling pathway (GO:0070105), T cell apoptotic process (GO:0070231), necroptotic process (GO:0070266), cellular response to hydrogen peroxide (GO:0070301), cellular response to tumor necrosis factor (GO:0071356), cellular response to growth factor stimulus (GO:0071363), extrinsic apoptotic signaling pathway (GO:0097191), programmed necrotic cell death (GO:0097300), ripoptosome assembly (GO:0097343), necroptotic signaling pathway (GO:0097527), positive regulation of execution phase of apoptosis (GO:1900119), ripoptosome assembly involved in necroptotic process (GO:1901026)

GO Molecular Function (17): protein kinase activity (GO:0004672), protein serine/threonine kinase activity (GO:0004674), JUN kinase kinase kinase activity (GO:0004706), death receptor binding (GO:0005123), ATP binding (GO:0005524), ubiquitin protein ligase binding (GO:0031625), signaling adaptor activity (GO:0035591), identical protein binding (GO:0042802), protein homodimerization activity (GO:0042803), protein-containing complex binding (GO:0044877), death domain binding (GO:0070513), protein serine kinase activity (GO:0106310), protein serine/threonine kinase binding (GO:0120283), nucleotide binding (GO:0000166), protein binding (GO:0005515), kinase activity (GO:0016301), transferase activity (GO:0016740)

GO Cellular Component (10): cytoplasm (GO:0005737), mitochondrion (GO:0005739), cytosol (GO:0005829), plasma membrane (GO:0005886), endosome membrane (GO:0010008), death-inducing signaling complex (GO:0031264), protein-containing complex (GO:0032991), signaling receptor complex (GO:0043235), ripoptosome (GO:0097342), membrane (GO:0016020)

Reactome top-level categories

Rollup of top-14 pathways:

CategoryPathways
TNF signaling3
Toll Like Receptor 3 (TLR3) Cascade2
TRIF (TICAM1)-mediated TLR4 signaling2
Caspase activation via Death Receptors in the presence of ligand2
Deubiquitination2
Caspase activation via extrinsic apoptotic signalling pathway1
ZBP1(DAI) mediated induction of type I IFNs1
Stimuli-sensing channels1
Regulated Necrosis1
Regulation of necroptotic cell death1
RIPK1-mediated regulated necrosis1
Death Receptor Signaling1
DDX58/IFIH1-mediated induction of interferon-alpha/beta1
SARS-CoV Infections1

GO top-level categories

Rollup of top GO terms by namespace:

CategoryTerms
cellular anatomical structure3
programmed cell death2
intracellular anatomical structure2
apoptotic process2
regulation of apoptotic process2
canonical NF-kappaB signal transduction2
regulation of canonical NF-kappaB signal transduction2
protein kinase activity2
binding2
cytoplasm2
regulation of protein phosphorylation1
protein phosphorylation1
positive regulation of protein modification process1
positive regulation of phosphorylation1
apoptotic signaling pathway1
execution phase of apoptosis1
defense response1
response to stress1
intracellular signaling cassette1
gene expression1
regulation of gene expression1
positive regulation of macromolecule biosynthetic process1
macromolecule catabolic process1
protein metabolic process1
positive regulation of cytokine production1
interleukin-8 production1
regulation of interleukin-8 production1
tumor necrosis factor production1
regulation of tumor necrosis factor production1
positive regulation of tumor necrosis factor superfamily cytokine production1
cytokine-mediated signaling pathway1
cellular response to tumor necrosis factor1
response to cytokine1
signal transduction1
peptidyl-serine phosphorylation1
protein autophosphorylation1
positive regulation of programmed cell death1
negative regulation of programmed cell death1
regulation of programmed cell death1
positive regulation of cellular process1

Protein interactions and networks

STRING

3240 interactions, top by confidence (×1000):

Protein AProtein BPartner UniProtScore
RIPK1FADDQ13158998
RIPK1CASP8Q14790998
RIPK1TRADDQ15628998
RIPK1TNFRSF1AP19438997
RIPK1BIRC2Q13490996
RIPK1CFLARO15519995
RIPK1TRAF2Q12933994
RIPK1IKBKGQ9Y6K9994
RIPK1BIRC3Q13489993
RIPK1TRAF5O00463992
RIPK1RIPK3Q9Y572989
RIPK1MLKLQ8NB16989
RIPK1TRAF6Q9Y4K3987
RIPK1TAB2Q9NYJ8982
RIPK1IFNB1P01574971

IntAct

280 interactions, top by confidence:

ABTypeScore
CASP8FADDpsi-mi:“MI:0914”(association)0.980
IKBKGIKBKBpsi-mi:“MI:0914”(association)0.980
CASP8FADDpsi-mi:“MI:0915”(physical association)0.980
RIPK1RIPK3psi-mi:“MI:0914”(association)0.970
RIPK3RIPK1psi-mi:“MI:0915”(physical association)0.970
RIPK1RIPK3psi-mi:“MI:0915”(physical association)0.970
RIPK1RIPK3psi-mi:“MI:0217”(phosphorylation reaction)0.970
RIPK3RIPK1psi-mi:“MI:0914”(association)0.970
TNFTNFRSF1Apsi-mi:“MI:0914”(association)0.960
CASP8RIPK1psi-mi:“MI:0914”(association)0.960

BioGRID (609): RIPK1 (Affinity Capture-Western), RIPK1 (Biochemical Activity), RIPK1 (Affinity Capture-Western), RFFL (Affinity Capture-Western), RIPK1 (Affinity Capture-Western), RIPK1 (Two-hybrid), RIPK1 (Two-hybrid), RIPK1 (Two-hybrid), RIPK1 (Reconstituted Complex), RIPK1 (Biochemical Activity), RIPK1 (Affinity Capture-Western), RIPK1 (Affinity Capture-Western), RIPK1 (Affinity Capture-Western), UBC (Affinity Capture-Western), UBC (Affinity Capture-Western)

ESM2 similar proteins: A1YG61, A2T737, A4IGQ8, B5XCB8, O60934, O70273, P28715, Q08B72, Q0III0, Q13546, Q28J92, Q2KJE0, Q32LN0, Q3B8D4, Q3T0G1, Q3US16, Q4R3Q6, Q5R4U3, Q5RCE4, Q5RCV3, Q5SZJ8, Q5U208, Q5U560, Q5VVJ2, Q60855, Q69Z66, Q6DDT6, Q6DIN8, Q6IR68, Q6PFX2, Q6TGZ4, Q7L4P6, Q7ZYM8, Q86VP1, Q86VV4, Q8BSV3, Q8C6D4, Q8CHW1, Q8IW35, Q8N302

Diamond homologs: A0A509AH51, A1CAF0, A1CL96, A1D624, A2QU77, A2YMV6, A8BPK8, B1H3E1, B5VNQ3, C4YGK0, D0Z5N4, O18209, O19004, O34507, O45797, O61661, P04627, P09251, P0CS76, P0CS77, P10398, P10533, P12965, P14056, P15056, P19026, P23293, P27966, P28028, P28926, P32516, P32866, P34908, P41808, P51953, P52304, P84390, Q00772, Q01577, Q04543

SIGNOR signaling

64 interactions.

AEffectBMechanism
RIPK1“up-regulates activity”CASP8binding
RIPK1“up-regulates activity”MAP3K1phosphorylation
RIPK1up-regulatesMAP3K1phosphorylation
RIPK1up-regulatesNecrosis
RIPK1“up-regulates activity”TAB2binding
BIRC2“up-regulates activity”RIPK1ubiquitination
BIRC3“up-regulates activity”RIPK1ubiquitination
RIPK1“up-regulates activity”IKBKGbinding
RIPK1“up-regulates activity”TAB3binding
FADD“up-regulates activity”RIPK1binding
RIPK1“up-regulates activity”FASbinding
RIPK1up-regulatesTNFRSF10A
TRAF2“up-regulates activity”RIPK1ubiquitination
5-(1H-indol-3-ylmethyl)-3-methyl-2-sulfanylidene-4-imidazolidinonedown-regulatesRIPK1“chemical inhibition”
TICAM1“up-regulates activity”RIPK1binding
RIPK1“up-regulates activity”TRAF6binding
RIPK1“up-regulates activity”DAB2IPphosphorylation
RIPK1“up-regulates activity”MAP3K7binding
TRAF2up-regulatesRIPK1
“Caspase 8 complex”“down-regulates activity”RIPK1cleavage
TNFAIP3“down-regulates quantity”RIPK1ubiquitination
ponatinib“down-regulates activity”RIPK1“chemical inhibition”

Enriched among interaction partners

Reactome pathways and GO biological processes over-represented among this gene’s 104 IntAct physical interaction partners (hypergeometric vs the genome-wide background, BH-FDR, gene-set size 15–500, ranked by fold). A functional readout of the neighbourhood — distinct from this gene’s own memberships above, and biased toward well-studied / hub proteins, so read it as themes rather than proof.

Reactome pathways:

PathwayPartnersFoldFDR
RIPK1-mediated regulated necrosis1168.8×3e-16
TICAM1, RIP1-mediated IKK complex recruitment865.9×1e-11
Caspase activation via Death Receptors in the presence of ligand662.6×1e-08
TNFR1-induced proapoptotic signaling1060.2×4e-14
Regulation of necroptotic cell death1060.2×4e-14
TNFR1-induced NF-kappa-B signaling pathway1359.8×3e-18
IKK complex recruitment mediated by RIP1854.4×6e-11
Regulation of TNFR1 signaling1752.1×6e-23

GO biological processes:

GO termPartnersFoldFDR
non-canonical NF-kappaB signal transduction548.4×4e-06
protein refolding643.0×7e-07
tumor necrosis factor-mediated signaling pathway1141.8×1e-12
regulation of tumor necrosis factor-mediated signaling pathway540.4×1e-05
canonical NF-kappaB signal transduction937.9×5e-10
extrinsic apoptotic signaling pathway via death domain receptors836.9×7e-09
negative regulation of canonical NF-kappaB signal transduction1121.7×6e-10
extrinsic apoptotic signaling pathway621.1×3e-05

Disease & clinical

Cancer significance

From intOGen — cancer-driver classification: loss-of-function (tumor-suppressor-like) across 1 cancer types — CLLSLL.

Clinical variants and AI predictions

ClinVar

588 variants total. Per-class counts are floors (≥ shown; pagination cap):

ClassificationCount (floor)
Pathogenic22
Likely pathogenic7
Uncertain significance329
Likely benign187
Benign12

Top pathogenic / likely-pathogenic (29)

Variant IDHGVSClassification
1350924NC_000006.11:g.(?3077058)(3078189_?)delPathogenic
1424981NM_001354930.2(RIPK1):c.187G>T (p.Glu63Ter)Pathogenic
1455831NM_001354930.2(RIPK1):c.1632C>G (p.Tyr544Ter)Pathogenic
1456594NC_000006.11:g.(?3083299)(3085662_?)delPathogenic
1457300NM_001354930.2(RIPK1):c.528_532del (p.Arg177fs)Pathogenic
1459670NC_000006.11:g.(?3104439)(3106305_?)delPathogenic
151603GRCh38/hg38 6p25.3-22.3(chr6:155807-17058414)x3Pathogenic
1949108NM_001354930.2(RIPK1):c.388del (p.His130fs)Pathogenic
2822654NM_001354930.2(RIPK1):c.569_585del (p.Leu190fs)Pathogenic
2858656NM_001354930.2(RIPK1):c.1542dup (p.Pro515fs)Pathogenic
3246172NC_000006.11:g.(?3083299)(3106305_?)delPathogenic
3621187NM_001354930.2(RIPK1):c.775G>T (p.Glu259Ter)Pathogenic
561164NM_001354930.2(RIPK1):c.867_870del (p.Phe288_Tyr289insTer)Pathogenic
561165NM_001354930.2(RIPK1):c.688_688+20delPathogenic
561166NM_001354930.2(RIPK1):c.460-133_689-244delPathogenic
598789NM_001354930.2(RIPK1):c.1278C>A (p.Tyr426Ter)Pathogenic
598791NM_001354930.2(RIPK1):c.1802G>A (p.Cys601Tyr)Pathogenic
870218NM_001354930.2(RIPK1):c.970G>A (p.Asp324Asn)Pathogenic
870219NM_001354930.2(RIPK1):c.970G>C (p.Asp324His)Pathogenic
870220NM_001354930.2(RIPK1):c.970G>T (p.Asp324Tyr)Pathogenic
870221NM_001354930.2(RIPK1):c.971A>T (p.Asp324Val)Pathogenic
981836NM_001354930.2(RIPK1):c.1802_1805del (p.Cys601fs)Pathogenic
1498963NM_001354930.2(RIPK1):c.322-1G>CLikely pathogenic
1690605NM_001354930.2(RIPK1):c.1458_1459del (p.Arg487fs)Likely pathogenic
2809856NM_001354930.2(RIPK1):c.915+1G>ALikely pathogenic
3246170NC_000006.11:g.(?3081193)(3081370_?)delLikely pathogenic
3630393NM_001354930.2(RIPK1):c.1576+2T>GLikely pathogenic
3765421NM_001354930.2(RIPK1):c.1577-1G>ALikely pathogenic
4154692NM_001354930.2(RIPK1):c.971A>G (p.Asp324Gly)Likely pathogenic

SpliceAI

1951 predictions. Top by Δscore:

VariantEffectΔscore
6:3076911:A:Tdonor_gain1.0000
6:3076988:G:GGdonor_gain1.0000
6:3077872:G:GTdonor_gain1.0000
6:3077872:G:Tdonor_gain1.0000
6:3077931:CCGAG:Cdonor_loss1.0000
6:3077932:CGAGG:Cdonor_loss1.0000
6:3077933:GAGG:Gdonor_loss1.0000
6:3077934:AG:Adonor_loss1.0000
6:3077935:GGTA:Gdonor_loss1.0000
6:3077936:GTAGA:Gdonor_loss1.0000
6:3077937:T:Adonor_loss1.0000
6:3083246:G:GTdonor_gain1.0000
6:3083272:T:TAdonor_gain1.0000
6:3083273:A:AAdonor_gain1.0000
6:3083310:GAAA:Gdonor_gain1.0000
6:3083311:A:Tdonor_gain1.0000
6:3083312:AAGT:Adonor_loss1.0000
6:3083313:AGT:Adonor_loss1.0000
6:3083314:G:GGdonor_gain1.0000
6:3083314:G:Tdonor_loss1.0000
6:3083315:T:Gdonor_loss1.0000
6:3104217:A:AGacceptor_gain1.0000
6:3104218:A:Gacceptor_gain1.0000
6:3104223:A:AGacceptor_gain1.0000
6:3104224:G:GAacceptor_gain1.0000
6:3104224:GAA:Gacceptor_gain1.0000
6:3104224:GAAA:Gacceptor_gain1.0000
6:3104311:TTCAG:Tdonor_loss1.0000
6:3104312:TCAG:Tdonor_loss1.0000
6:3104313:CAG:Cdonor_loss1.0000

AlphaMissense

4492 scored. Top likely-pathogenic:

VariantProtein changeam_pathogenicity
6:3113115:T:AW598R0.998
6:3113115:T:CW598R0.998
6:3113226:T:AW635R0.997
6:3113226:T:CW635R0.997
6:3113128:C:AA602D0.996
6:3113117:G:CW598C0.994
6:3113117:G:TW598C0.994
6:3113281:T:CL653P0.993
6:3083280:T:AW219R0.992
6:3083280:T:CW219R0.992
6:3113271:G:CA650P0.992
6:3113218:T:CL632P0.991
6:3113269:T:CL649P0.989
6:3083262:A:CS213R0.988
6:3083264:C:AS213R0.988
6:3083264:C:GS213R0.988
6:3113137:T:CL605P0.987
6:3113215:T:GM631R0.986
6:3113272:C:AA650D0.986
6:3113215:T:AM631K0.985
6:3113228:G:CW635C0.985
6:3113228:G:TW635C0.985
6:3113131:G:CR603P0.984
6:3113116:G:CW598S0.983
6:3113127:G:CA602P0.983
6:3113277:G:CA652P0.983
6:3113281:T:AL653H0.981
6:3085372:T:AW268R0.980
6:3085372:T:CW268R0.980
6:3113083:T:CL587P0.979

dbSNP variants (sampled 300 via entrez): RS1000095415 (6:3068930 T>G), RS1000126978 (6:3109862 C>G,T), RS1000148853 (6:3076986 G>C), RS1000229128 (6:3068837 C>A,T), RS1000240255 (6:3096713 C>T), RS1000247018 (6:3115621 C>G), RS1000305249 (6:3073247 TATA>T), RS1000375654 (6:3082577 T>A,G), RS1000412588 (6:3078407 G>A), RS1000485531 (6:3078305 A>T), RS1000537604 (6:3078547 T>A,C), RS1000557451 (6:3097336 G>C,T), RS1000715194 (6:3089416 A>C,G), RS1000837523 (6:3098118 G>C), RS1000902546 (6:3105149 G>A)

Disease associations

OMIM: gene MIM:603453 | disease phenotypes: MIM:618108, MIM:618852

GenCC curated gene-disease

DiseaseClassificationInheritance
immunodeficiency 57DefinitiveAutosomal recessive
autoinflammation with episodic fever and lymphadenopathyStrongAutosomal dominant
immune dysregulation-inflammatory bowel disease-arthritis-recurrent infections-lymphopenia syndromeSupportiveAutosomal recessive

ClinGen Gene-Disease Validity (1)

Expert-panel classifications — Definitive > Strong > Moderate > Limited > Disputed > Refuted.

DiseaseClassificationInheritance
immunodeficiency 57DefinitiveAR

Mondo (6): immunodeficiency 57 (MONDO:0020849), autoinflammation with episodic fever and lymphadenopathy (MONDO:0030018), inborn error of immunity (MONDO:0003778), IL10-related early-onset inflammatory bowel disease (MONDO:0016542), hereditary breast ovarian cancer syndrome (MONDO:0003582), immune dysregulation-inflammatory bowel disease-arthritis-recurrent infections-lymphopenia syndrome (MONDO:0033968)

Orphanet (3): Primary immunodeficiency (Orphanet:101997), Immune dysregulation-inflammatory bowel disease-arthritis-recurrent infections syndrome (Orphanet:238569), Hereditary breast and/or ovarian cancer syndrome (Orphanet:145)

HPO phenotypes

28 total (28 of 28 shown, HPO-id order):

HPOTerm
HP:0000006Autosomal dominant inheritance
HP:0000007Autosomal recessive inheritance
HP:0000155Oral ulcer
HP:0000988Skin rash
HP:0001508Failure to thrive
HP:0001744Splenomegaly
HP:0001935Microcytic anemia
HP:0001954Recurrent fever
HP:0002014Diarrhea
HP:0002027Abdominal pain
HP:0002037Inflammation of the large intestine
HP:0002110Bronchiectasis
HP:0002205Recurrent respiratory infections
HP:0002240Hepatomegaly
HP:0002716Lymphadenopathy
HP:0002923Rheumatoid factor positive
HP:0003493Antinuclear antibody positivity
HP:0003593Infantile onset
HP:0003623Neonatal onset
HP:0004313Decreased circulating immunoglobulin concentration
HP:0005263Gastritis
HP:0005403Decreased total T cell count
HP:0006528Chronic lung disease
HP:0009789Perianal abscess
HP:0010976Decreased total B cell count
HP:0011110Recurrent tonsillitis
HP:0040218Reduced total natural killer cell count
HP:0410297Partial absence of specific antibody response to tetanus vaccine

GWAS associations

2 associations (top):

StudyTraitp-value
GCST003918_6Idiopathic osteonecrosis of the femoral head5.000000e-06
GCST009206_5Cerebral white matter volume3.000000e-06

EFO canonical traits (2, from GWAS)

EFO IDTrait name
EFO:1001930idiopathic osteonecrosis of the femoral head
EFO:0008320white matter volume measurement

MeSH disease descriptors (2)

DescriptorNameTree numbers
D061325Hereditary Breast and Ovarian Cancer SyndromeC04.588.180.483; C04.588.322.455.431; C04.700.517; C12.050.351.500.056.630.705.431; C12.050.351.937.418.685.431; C12.100.250.056.630.705.431; C12.900.418.685.431; C16.320.700.517; C17.800.090.500.483; C19.344.410.431; C19.391.630.705.431
D007153Immunologic Deficiency SyndromesC20.673

Drugs & pharmacology

Drug and pharmacology data

Is drug target: yes

ChEMBL targets (4): CHEMBL3885602 (PROTEIN FAMILY), CHEMBL5464 (SINGLE PROTEIN), CHEMBL5465217 (PROTEIN-PROTEIN INTERACTION), CHEMBL6066130 (PROTEIN-PROTEIN INTERACTION)

Molecules with ChEMBL bioactivity

24 molecules (phase ≥1), by development phase (incl. off-target/promiscuous compounds). Patent mentions across the top 20 by phase: 263,504 (via chembl_molecule»patent_compound — counts attach to the compound, not the gene–compound relationship, so off-target/promiscuous molecules can dominate).

MoleculeNamePhasePatents
CHEMBL1171837PONATINIB48,955
CHEMBL1287853FEDRATINIB43,554
CHEMBL1289926AXITINIB415,732
CHEMBL1336SORAFENIB486,060
CHEMBL2028663DABRAFENIB412,430
CHEMBL477772PAZOPANIB415,540
CHEMBL502835NINTEDANIB48,545
CHEMBL535SUNITINIB479,020
CHEMBL576982QUIZARTINIB44,432
CHEMBL601719CRIZOTINIB414,403
CHEMBL223360LINIFANIB33,925
CHEMBL522892DOVITINIB34,944
CHEMBL1230609FORETINIB23,096
CHEMBL1721885SU-0148132363
CHEMBL1738757REBASTINIB21,478
CHEMBL3348846FEXAGRATINIB2221
CHEMBL4071864GSK29827722483
CHEMBL4861471ECLITASERTIB2259
CHEMBL5314462FLIZASERTIB221
CHEMBL5416250OCADUSERTIB243
CHEMBL558752RAF-2652
CHEMBL572878TOZASERTIB2
CHEMBL1908397KW-24491
CHEMBL574738AST-4871

PharmGKB: 1 entry (VIP=true, CPIC=false)

GtoPdb / IUPHAR curated pharmacology

(IUPHAR/BPS Guide to Pharmacology — expert-curated)

Target class: enzyme — Receptor interacting protein kinase (RIPK) family

Most potent curated ligand interactions (25 total), top 25:

LigandActionAffinityParameter
eclitasertibInhibition10.0pIC50
oditrasertibInhibition10.0pIC50
GSK963Inhibition9.1pIC50
GSK2982772Inhibition9.0pIC50
compound 21 [PMID: 24900635]Inhibition8.89pIC50
WJH-C19Inhibition8.24pIC50
GSK3145095Inhibition8.2pIC50
SZM679Inhibition8.07pKd
GSK'547Inhibition8.0pIC50
RIPK1 inhibitor 22bInhibition7.96pIC50
ponatinibInhibition7.92pIC50
compound 27 [PMID: 24900635]Inhibition7.89pIC50
RIPA-56Inhibition7.89pIC50
GNE684Inhibition7.68pKi
necrostatin-1sInhibition7.43pKd
ocadusertibInhibition7.4pIC50
PN10Inhibition7.05pIC50
SZM594Inhibition7.01pKd
SIR1-365Inhibition7.0pIC50
flizasertibInhibition6.66pKi
6E11Inhibition6.4pKd
TAK-632Inhibition6.32pKd
necrostatin-1Inhibition6.31pIC50
RIPK3 inhibitor 42Inhibition5.3pKd
RIPK3 inhibitor 18Inhibition5.26pIC50

Binding affinities (BindingDB)

574 measured of 653 human assays (653 total across all organisms); most potent 50 below. Values come from heterogeneous assays and are not directly comparable.

LigandMeasureValuePatent
8-(2-Amino-[1,2,4]triazolo[1,5-a]pyridin-7-yl)-2-(2-fluoro-5-(trifluoromethoxy)benzyl)-2,3,4,5-tetrahydro-1H-benzo[c]azepin-1-one 2IC502.7 nMUS-20250197390: FUSED HETEROCYCLIC COMPOUND, AND PREPARATION METHOD THEREFOR AND MEDICAL USE THEREOF
[(5S,7S)-5-(3-chlorophenyl)-7-fluoro-6,7-dihydro-5H-pyrrolo[1,2-b][1,2,4]triazol-2-yl]-cyclopropylmethanoneKI6.5 nMUS-12344612: Bicyclic ketone compounds and methods of use thereof
5-[[3-[3-(3,5-difluorophenyl)-3,4-dihydropyrazole-2-carbonyl]-1-bicyclo[1.1.1]pentanyl]methoxy]pyrazine-2-carbonitrileIC508.3 nMUS-20250214942: INHIBITORS OF RECEPTOR INTERACTING PROTEIN KINASE I FOR THE TREATMENT OF DISEASE
Cyclopropyl-[(5R,7R)-7-fluoro-5-(5-fluoro-3-pyridyl)-6,7-dihydro-5H-pyrrolo[1,2-b][1,2,4]triazol-2-yl]methanone and cyclopropyl-[(5S,7S)-7-fluoro-5-(5-fluoro-3-pyridyl)-6,7-dihydro-5H-pyrrolo[1,2-b][1,2,4]triazol-2-yl]methanoneKI9 nMUS-12344612: Bicyclic ketone compounds and methods of use thereof
1-((3-(5-(5-cyanopyridin-3- yl)-4,5-dihydro-1H-pyrazole- 1-carbonyl)bicyclo[1.1.1]- pentan-1-yl)methyl)-1H- indazole-5-carbonitrileIC509.1 nMUS-20250214942: INHIBITORS OF RECEPTOR INTERACTING PROTEIN KINASE I FOR THE TREATMENT OF DISEASE
1-((3-(5-(3,5-difluorophenyl)- 4,5-dihydro-1H-pyrazole-1- carbonyl)bicyclo[1.1.1]pentan- 1-yl)methyl)-1H-pyrrolo[3,2- b]pyridine-5-carbonitrileIC509.4 nMUS-20250214942: INHIBITORS OF RECEPTOR INTERACTING PROTEIN KINASE I FOR THE TREATMENT OF DISEASE
1-((3-(5-(3-fluorophenyl)- 4,5-dihydro-1H-pyrazole-1- carbonyl)bicyclo[1.1.1]- pentan-1-yl)methyl)-1H- pyrazolo[4,3-b]pyridine-5- carbonitrileIC509.6 nMUS-20250214942: INHIBITORS OF RECEPTOR INTERACTING PROTEIN KINASE I FOR THE TREATMENT OF DISEASE
1-((3-(5-(3,5-difluorophenyl)- 4,5-dihydro-1H-pyrazole-1- carbonyl)bicyclo[1.1.1]- pentan-1-yl)methyl)-1H- indazole-5-carbonitrileIC5010 nMUS-20250214942: INHIBITORS OF RECEPTOR INTERACTING PROTEIN KINASE I FOR THE TREATMENT OF DISEASE
5-benzyl-N-[(3S)-7-[2-(3-hydroxyoxetan-3-yl)ethynyl]-5-methyl-4-oxo-2,3-dihydro-1,5-benzoxazepin-3-yl]-1H-1,2,4-triazole-3-carboxamideIC5011 nMUS-10815206: RIP1 inhibitory compounds and methods for making and using the same
[3-(3,5-difluorophenyl)-3,4-dihydropyrazol-2-yl]-[3-[(4-fluoroindazol-2-yl)methyl]-1-bicyclo[1.1.1]pentanyl]methanoneIC5011 nMUS-20250214942: INHIBITORS OF RECEPTOR INTERACTING PROTEIN KINASE I FOR THE TREATMENT OF DISEASE
(3-((1H-pyrazolo[4,3-b]- pyridin-1-yl)methyl)bicyclo- [1.1.1]pentan-1-yl)(5-(3,5- difluorophenyl)-4,5-dihydro- 1H-pyrazol-1-yl)methanoneIC5011 nMUS-20250214942: INHIBITORS OF RECEPTOR INTERACTING PROTEIN KINASE I FOR THE TREATMENT OF DISEASE
(3-((4-fluoro-1H-indazol-1- yl)methyl)bicyclo[1.1.1]- pentan-1-yl)(5-phenyl-4,5- dihydro-1H-pyrazol-1-yl)- methanoneIC5011 nMUS-20250214942: INHIBITORS OF RECEPTOR INTERACTING PROTEIN KINASE I FOR THE TREATMENT OF DISEASE
3-(1-(3-((4-fluoro-1H- indazol-1-yl)methyl)bicyclo- [1.1.1]pentane-1-carbonyl)- 4,5-dihydro-1H-pyrazol-5- yl)benzonitrileIC5011 nMUS-20250214942: INHIBITORS OF RECEPTOR INTERACTING PROTEIN KINASE I FOR THE TREATMENT OF DISEASE
1-((3-(5-(3,5-difluorophenyl)- 4,5-dihydro-1H-pyrazole-1- carbonyl)bicyclo[1.1.1]pentan- 1-yl)methyl)-1H-pyrrolo[2,3- b]pyridine-5-carbonitrileIC5011 nMUS-20250214942: INHIBITORS OF RECEPTOR INTERACTING PROTEIN KINASE I FOR THE TREATMENT OF DISEASE
(3-((4-bromo-2-chloro- phenoxy)methyl)bicyclo- [1.1.1]pentan-1-yl)(5-(3,5- difluorophenyl)-4,5-dihydro- 1H-pyrazol-1-yl)methanoneIC5011 nMUS-20250214942: INHIBITORS OF RECEPTOR INTERACTING PROTEIN KINASE I FOR THE TREATMENT OF DISEASE
[3-[(4-fluoroindazol-1-yl)methyl]-1-bicyclo[1.1.1]pentanyl]-[3-(5-fluoro-3-pyridinyl)-3,4-dihydropyrazol-2-yl]methanoneIC5012 nMUS-20250214942: INHIBITORS OF RECEPTOR INTERACTING PROTEIN KINASE I FOR THE TREATMENT OF DISEASE
(3-((4-bromo-2-fluoro- phenoxy)methyl)bicyclo- [1.1.1]pentan-1-yl)(5-(3,5- difluorophenyl)-4,5-dihydro- 1H-pyrazol-1-yl)methanoneIC5013 nMUS-20250214942: INHIBITORS OF RECEPTOR INTERACTING PROTEIN KINASE I FOR THE TREATMENT OF DISEASE
TC001262KI13.7 nMUS-10092529: Necrosis inhibitors
[4-fluoro-2-(3-fluorophenyl)pyrrolidin-1-yl]-[3-[(5-fluoroindazol-1-yl)methyl]-1-bicyclo[1.1.1]pentanyl]methanoneIC5014 nMUS-20250214942: INHIBITORS OF RECEPTOR INTERACTING PROTEIN KINASE I FOR THE TREATMENT OF DISEASE
5-benzyl-N-[(3S)-7-[3-(1H-indazol-5-ylamino)-3-oxopropyl]-5-methyl-4-oxo-2,3-dihydro-1,5-benzoxazepin-3-yl]-1H-1,2,4-triazole-3-carboxamideIC5015.7 nMUS-10815206: RIP1 inhibitory compounds and methods for making and using the same
5-benzyl-N-[7-[3-(1H-indazol-5-ylamino)-3-oxopropyl]-5-methyl-4-oxo-2,3-dihydro-1,5-benzoxazepin-3-yl]-1H-1,2,4-triazole-3-carboxamideIC5015.7 nMUS-11407736: RIP1 inhibitory compounds and methods for making and using the same
6-[[3-[3-(3,5-difluorophenyl)-3,4-dihydropyrazole-2-carbonyl]-1-bicyclo[1.1.1]pentanyl]methoxy]pyrimidine-4-carbonitrileIC5016 nMUS-20250214942: INHIBITORS OF RECEPTOR INTERACTING PROTEIN KINASE I FOR THE TREATMENT OF DISEASE
1-[[3-[3-(6-methylpyrazin-2-yl)-3,4-dihydropyrazole-2-carbonyl]-1-bicyclo[1.1.1]pentanyl]methyl]indazole-5-carbonitrileIC5016 nMUS-20250214942: INHIBITORS OF RECEPTOR INTERACTING PROTEIN KINASE I FOR THE TREATMENT OF DISEASE
1-((3-(5-(3,5-difluorophenyl)- 4,5-dihydro-1H-pyrazole-1- carbonyl)bicyclo[1.1.1]- pentan-1-yl)methyl)-1H- indazole-6-carbonitrileIC5016 nMUS-20250214942: INHIBITORS OF RECEPTOR INTERACTING PROTEIN KINASE I FOR THE TREATMENT OF DISEASE
(3-((4-fluoro-1H-indazol-1- yl)methyl)bicyclo[1.1.1]- pentan-1-yl)(4-fluoro-2-(3- fluorophenyl)pyrrolidin-1- yl)methanoneIC5016 nMUS-20250214942: INHIBITORS OF RECEPTOR INTERACTING PROTEIN KINASE I FOR THE TREATMENT OF DISEASE
1-((3-(5-(3-cyanophenyl)-4,5- dihydro-1H-pyrazole-1- carbonyl)bicyclo[1.1.1]- pentan-1-yl)methyl)-1H- indazole-5-carbonitrileIC5016 nMUS-20250214942: INHIBITORS OF RECEPTOR INTERACTING PROTEIN KINASE I FOR THE TREATMENT OF DISEASE
4-((3-(5-(3,5-difluorophenyl)- 4,5-dihydro-1H-pyrazole-1- carbonyl)bicyclo[1.1.1]pentan- 1-yl)methoxy)-3-fluoro- benzonitrileIC5016 nMUS-20250214942: INHIBITORS OF RECEPTOR INTERACTING PROTEIN KINASE I FOR THE TREATMENT OF DISEASE
[3-(3,5-difluorophenyl)-3,4-dihydropyrazol-2-yl]-[3-(fluoromethyl)-1-bicyclo[1.1.1]pentanyl]methanoneIC5017 nMUS-20250214942: INHIBITORS OF RECEPTOR INTERACTING PROTEIN KINASE I FOR THE TREATMENT OF DISEASE
1-((3-(5-(5-fluoropyridin-3- yl)-4,5-dihydro-1H-pyrazole- 1-carbonyl)bicyclo[1.1.1]- pentan-1-yl)methyl)-1H- indazole-5-carbonitrileIC5017 nMUS-20250214942: INHIBITORS OF RECEPTOR INTERACTING PROTEIN KINASE I FOR THE TREATMENT OF DISEASE
(5R)-5-[(7-chloro-1H-indol-3-yl)methyl]-3-methyl-2,4-Imidazolidinedione, 11EC5018 nM
(5-(3,5-difluorophenyl)-4,5- dihydro-1H-pyrazol-1-yl)(3- ((5-fluoro-2H-benzo[d]- [1,2,3]triazol-2-yl)methyl)- bicyclo[1.1.1]pentan-1-yl)- methanoneIC5018 nMUS-20250214942: INHIBITORS OF RECEPTOR INTERACTING PROTEIN KINASE I FOR THE TREATMENT OF DISEASE
(3-((1H-pyrazolo[3,4-b]- pyrazin-1-yl)methyl)- bicyclo[1.1.1]pentan-1-yl)(5- (3,5-difluorophenyl)-4,5- dihydro-1H-pyrazol-1- yl)methanoneIC5018 nMUS-20250214942: INHIBITORS OF RECEPTOR INTERACTING PROTEIN KINASE I FOR THE TREATMENT OF DISEASE
(5-(3,5-difluorophenyl)-4,5- dihydro-1H-pyrazol-1-yl)(3- (((3,5-difluoropyridin-2-yl)- oxy)methyl)bicyclo[1.1.1]- pentan-1-yl)methanoneIC5018 nMUS-20250214942: INHIBITORS OF RECEPTOR INTERACTING PROTEIN KINASE I FOR THE TREATMENT OF DISEASE
(3-((2-chloro-4-fluoro- phenoxy)methyl)bicyclo- [1.1.1]pentan-1-yl)(5-(3,5- difluorophenyl)-4,5-dihydro- 1H-pyrazol-1-yl)methanoneIC5018 nMUS-20250214942: INHIBITORS OF RECEPTOR INTERACTING PROTEIN KINASE I FOR THE TREATMENT OF DISEASE
(3-((2,4-difluorophenoxy)- methyl)bicyclo[1.1.1]pentan-1- yl)(5-(3,5-difluorophenyl)-4,5- dihydro-1H-pyrazol-1- yl)methanoneIC5018 nMUS-20250214942: INHIBITORS OF RECEPTOR INTERACTING PROTEIN KINASE I FOR THE TREATMENT OF DISEASE
5-benzyl-N-[(3S)-7-(4-methoxy-3-oxobutyl)-5-methyl-4-oxo-2,3-dihydro-1,5-benzoxazepin-3-yl]-1H-1,2,4-triazole-3-carboxamideIC5019 nMUS-10815206: RIP1 inhibitory compounds and methods for making and using the same
US10815206, Compound I-64IC5019 nMUS-10815206: RIP1 inhibitory compounds and methods for making and using the same
ethyl 3-[3-[(5-benzyl-1H-1,2,4-triazole-3-carbonyl)amino]-5-methyl-4-oxo-2,3-dihydro-1,5-benzoxazepin-7-yl]propanoateIC5019 nMUS-11407736: RIP1 inhibitory compounds and methods for making and using the same
5-benzyl-N-(7-ethynyl-5-methyl-4-oxo-2,3-dihydro-1,5-benzoxazepin-3-yl)-1H-1,2,4-triazole-3-carboxamideIC5019 nMUS-11407736: RIP1 inhibitory compounds and methods for making and using the same
[3-(3,5-difluorophenyl)-3,4-dihydropyrazol-2-yl]-[3-[(5-fluoroindazol-2-yl)methyl]-1-bicyclo[1.1.1]pentanyl]methanoneIC5019 nMUS-20250214942: INHIBITORS OF RECEPTOR INTERACTING PROTEIN KINASE I FOR THE TREATMENT OF DISEASE
2-((3-(5-(3,5-difluorophenyl)- 4,5-dihydro-1H-pyrazole-1- carbonyl)bicyclo[1.1.1]- pentan-1-yl)methyl)-2H- indazole-6-carbonitrileIC5019 nMUS-20250214942: INHIBITORS OF RECEPTOR INTERACTING PROTEIN KINASE I FOR THE TREATMENT OF DISEASE
1-((3-(5-(3,5-difluorophenyl)- 4,5-dihydro-1H-pyrazole-1- carbonyl)bicyclo[1.1.1]- pentan-1-yl)methyl)-1H- indazole-4-carbonitrileIC5019 nMUS-20250214942: INHIBITORS OF RECEPTOR INTERACTING PROTEIN KINASE I FOR THE TREATMENT OF DISEASE
(3-((2H-benzo[d][1,2,3]- triazol-2-yl)methyl)bicyclo- [1.1.1]pentan-1-yl)(5-(3,5- difluorophenyl)-4,5-dihydro- 1H-pyrazol-1-yl)methanoneIC5019 nMUS-20250214942: INHIBITORS OF RECEPTOR INTERACTING PROTEIN KINASE I FOR THE TREATMENT OF DISEASE
1-((3-(4-fluoro-2-(3-fluoro- phenyl)pyrrolidine-1- carbonyl)bicyclo[1.1.1]- pentan-1-yl)methyl)-1H- indazole-5-carbonitrileIC5019 nMUS-20250214942: INHIBITORS OF RECEPTOR INTERACTING PROTEIN KINASE I FOR THE TREATMENT OF DISEASE
6-((3-(5-(3,5-difluorophenyl)- 4,5-dihydro-1H-pyrazole-1- carbonyl)bicyclo[1.1.1]pentan- 1-yl)methoxy)nicotinonitrileIC5019 nMUS-20250214942: INHIBITORS OF RECEPTOR INTERACTING PROTEIN KINASE I FOR THE TREATMENT OF DISEASE
(3-((2-chloro-3-fluoro- phenoxy)methyl)bicyclo- [1.1.1]pentan-1-yl)(5-(3,5- difluorophenyl)-4,5-dihydro- 1H-pyrazol-1-yl)methanoneIC5019 nMUS-20250214942: INHIBITORS OF RECEPTOR INTERACTING PROTEIN KINASE I FOR THE TREATMENT OF DISEASE
TC001207KI19.8 nMUS-10092529: Necrosis inhibitors
[3-(chloromethyl)-1-bicyclo[1.1.1]pentanyl]-[3-(3,5-difluorophenyl)-3,4-dihydropyrazol-2-yl]methanoneIC5020 nMUS-20250214942: INHIBITORS OF RECEPTOR INTERACTING PROTEIN KINASE I FOR THE TREATMENT OF DISEASE
(3-((7-chloro-1H-pyrazolo- [4,3-c]pyridin-1-yl)methyl)- bicyclo[1.1.1]pentan-1-yl)(5- (3,5-difluorophenyl)-4,5- dihydro-1H-pyrazol-1- yl)methanoneIC5020 nMUS-20250214942: INHIBITORS OF RECEPTOR INTERACTING PROTEIN KINASE I FOR THE TREATMENT OF DISEASE
(5-(3,5-difluorophenyl)-4,5- dihydro-1H-pyrazol-1-yl)(3- ((5-fluoro-1H-indol-1-yl)- methyl)bicyclo[1.1.1]pentan- 1-yl)methanoneIC5020 nMUS-20250214942: INHIBITORS OF RECEPTOR INTERACTING PROTEIN KINASE I FOR THE TREATMENT OF DISEASE

ChEMBL bioactivities

4555 potent at pChembl≥5 of 5316 total, top 50 by pChembl (potency: 10 = 0.1 nM, 6 = 1 µM).

pChemblTypeValueUnitMolecule
11.00IC500.01nMCHEMBL6193201
11.00IC500.01nMCHEMBL6191419
11.00IC500.01nMCHEMBL6192383
10.30IC500.05nMCHEMBL4067372
10.20IC500.063nMCHEMBL3785745
10.10IC500.079nMCHEMBL4093220
9.70IC500.2nMCHEMBL3786997
9.55Kd0.28nMCHEMBL5177284
9.52IC500.3nMCHEMBL4439913
9.52Ki0.3nMCHEMBL5885803
9.52IC500.3nMCHEMBL6177790
9.49IC500.32nMCHEMBL4068954
9.49IC500.32nMCHEMBL4096224
9.40IC500.4nMCHEMBL3785482
9.40IC500.4nMCHEMBL4069318
9.40IC500.4nMCHEMBL4060644
9.40IC500.4nMGSK2982772
9.40IC500.4nMCHEMBL4452233
9.40IC500.4nMCHEMBL6165847
9.39Ki0.4074nMCHEMBL4075976
9.37Ki0.43nMTP-030-2
9.30IC500.5nMCHEMBL4089558
9.30IC500.5nMCHEMBL4099004
9.30IC500.5nMGSK2982772
9.30IC500.5nMCHEMBL4452233
9.30IC500.5nMCHEMBL4537171
9.30EC500.5nMCHEMBL5093571
9.22IC500.6nMCHEMBL6175053
9.22IC500.6nMCHEMBL6176716
9.20IC500.63nMCHEMBL4081685
9.20IC500.63nMCHEMBL4065561
9.15IC500.7nMCHEMBL6177473
9.10Ki0.8nMCHEMBL3785703
9.10IC500.79nMCHEMBL3786078
9.10IC500.79nMCHEMBL4088857
9.10IC500.8nMCHEMBL6177790
9.07Ki0.8511nMCHEMBL4061975
9.05IC500.9nMCHEMBL4439913
9.05IC500.9nMCHEMBL6163751
9.05IC500.9nMCHEMBL6173421
9.04Ki0.912nMCHEMBL4100398
9.04Ki0.912nMCHEMBL4069537
9.04Ki0.91nMCHEMBL6145720
9.03IC500.94nMCHEMBL3785745
9.01Ki0.9772nMGSK2982772
9.00IC501nMGSK2982772
9.00IC501nMCHEMBL4099698
9.00IC501nMCHEMBL4080978
9.00IC501nMCHEMBL4067372
9.00IC501nMCHEMBL4514271

PubChem BioAssay actives

974 with measured affinity, of 1891 total; 50 most potent distinct compounds. Largely complementary to BindingDB; screening values are coarse (µM, 4 dp), so sub-nM hits tie at the floor.

CompoundAssayTypeValueUnit
2-benzyl-N-[(3S)-5-methyl-4-oxo-2,3-dihydro-1,5-benzoxazepin-3-yl]tetrazole-5-carboxamide1432226: Inhibition of human RIP1 (1 to 375 residues) expressed in baculovirus infected insect cells preincubated for 1 hr followed by ATP addition measured after 5 hrs by ADP-Glo luminescence assayic500.0001uM
5-benzyl-N-[(3S)-5-methyl-4-oxo-2,3-dihydro-1,5-benzoxazepin-3-yl]-1,3-oxazole-2-carboxamide1432226: Inhibition of human RIP1 (1 to 375 residues) expressed in baculovirus infected insect cells preincubated for 1 hr followed by ATP addition measured after 5 hrs by ADP-Glo luminescence assayic500.0001uM
5-benzyl-N-[(3R)-5-methyl-4-oxo-2,3-dihydro-1,5-benzothiazepin-3-yl]-1,2-oxazole-3-carboxamide1432226: Inhibition of human RIP1 (1 to 375 residues) expressed in baculovirus infected insect cells preincubated for 1 hr followed by ATP addition measured after 5 hrs by ADP-Glo luminescence assayic500.0001uM
5-benzyl-N-[(3S)-1-methyl-2-oxo-4,5-dihydro-3H-1-benzazepin-3-yl]-1,2-oxazole-3-carboxamide1290032: Inhibition of Flag-tagged human RIP1 (1 to 324 residues) after 30 mins by ADP-Glo reagent based assayic500.0002uM
methyl N-[(3S)-3-[(5-benzyl-1,2-oxazole-3-carbonyl)amino]-5-methyl-4-oxo-2,3-dihydro-1,5-benzoxazepin-7-yl]carbamate1432226: Inhibition of human RIP1 (1 to 375 residues) expressed in baculovirus infected insect cells preincubated for 1 hr followed by ATP addition measured after 5 hrs by ADP-Glo luminescence assayic500.0003uM
tert-butyl 2-[4-[5-(methylcarbamoyl)benzimidazol-1-yl]phenyl]acetate1580388: Inhibition of human RIPK1 by ADP-Glo assayic500.0003uM
7-(4-aminocyclohexyl)-5-(2-fluoro-4-methylphenyl)pyrrolo[2,3-d]pyrimidin-4-amine1880918: Binding affinity to human RIPK1 in human Jurkat cellskd0.0003uM
N-[(3S)-7-acetamido-5-methyl-4-oxo-2,3-dihydro-1,5-benzoxazepin-3-yl]-5-benzyl-1,2-oxazole-3-carboxamide1432226: Inhibition of human RIP1 (1 to 375 residues) expressed in baculovirus infected insect cells preincubated for 1 hr followed by ATP addition measured after 5 hrs by ADP-Glo luminescence assayic500.0003uM
5-benzyl-N-[(3S)-5-methyl-4-oxo-2,3-dihydro-1,5-benzoxazepin-3-yl]-1H-pyrazole-3-carboxamide1290032: Inhibition of Flag-tagged human RIP1 (1 to 324 residues) after 30 mins by ADP-Glo reagent based assayic500.0004uM
1-benzyl-N-[(3S)-5-methyl-4-oxo-2,3-dihydro-1,5-benzoxazepin-3-yl]imidazole-4-carboxamide1432226: Inhibition of human RIP1 (1 to 375 residues) expressed in baculovirus infected insect cells preincubated for 1 hr followed by ATP addition measured after 5 hrs by ADP-Glo luminescence assayic500.0004uM
5-benzyl-N-[(3S)-7,9-difluoro-2-oxo-1,3,4,5-tetrahydro-1-benzazepin-3-yl]-1H-1,2,4-triazole-3-carboxamide1509723: Inhibition of RIP1 in human neutrophils assessed as inhibition of TNF-alpha/QVD-Oph/RMT5265-stimulated MIP-1 beta cytokine overproduction measured at 21 hrs post stimulation by sandwich ELISAic500.0004uM
(3S)-3-(2-benzyl-3-chloro-7-oxo-4,5-dihydropyrazolo[3,4-c]pyridin-6-yl)-5-methyl-2,3-dihydro-1,5-benzoxazepin-4-one1469067: Inhibition of fluorescent-labeled 3-(3-((3-(4-amino-5-(4-(3-(2-fluoro-5-(trifluoromethyl)phenyl)ureido)-phenyl)-7H-pyrrolo[2,3-d]pyrimidin-7-yl)propyl)amino)-3-oxopropyl)-5,5-difluoro-7,9-dimethyl-5H-dipyrrolo[1,2-c:2’,1’-f]-[1,3,2]diazaborinin-4-ium-5-uide binding to human N-terminal GST/His-tagged RIPK1 (1 to 375 residues) expressed in baculovirus expression system by TR-FRET assayki0.0004uM
5-benzyl-1-methyl-N-[(3S)-5-methyl-4-oxo-2,3-dihydro-1,5-benzoxazepin-3-yl]pyrazole-3-carboxamide1432226: Inhibition of human RIP1 (1 to 375 residues) expressed in baculovirus infected insect cells preincubated for 1 hr followed by ATP addition measured after 5 hrs by ADP-Glo luminescence assayic500.0004uM
5-benzyl-N-[(3S)-5-methyl-4-oxo-2,3-dihydro-1,5-benzoxazepin-3-yl]-1H-1,2,4-triazole-3-carboxamide1432234: Inhibition of RIP1 in human primary neutrophils assessed as reduction in TNFalpha/QCD-OPh/SMAC mimetic RMT 5265-induced necroptosis at 21 hrs post-stimulation by LDH release assayic500.0004uM
5-benzyl-N-[(3S)-5-methyl-4-oxo-7-(2-oxo-3H-1,3,4-oxadiazol-5-yl)-2,3-dihydro-1,5-benzoxazepin-3-yl]-1,2-oxazole-3-carboxamide1432226: Inhibition of human RIP1 (1 to 375 residues) expressed in baculovirus infected insect cells preincubated for 1 hr followed by ATP addition measured after 5 hrs by ADP-Glo luminescence assayic500.0005uM
1-[3-(3-fluorophenyl)-3,4-dihydropyrazol-2-yl]-2,2-dimethylpropan-1-one1554569: Inhibition of human GST/His-tagged RIP1 (1 to 375 residues) expressed in baculovirus expression system assessed as reduction in autophosphorylation measured after 4 hrs in presence of ATP by ADP-Glo luminescence assayic500.0005uM
(3-phenoxyazetidin-1-yl)-[(3S)-3-phenyl-3,4-dihydropyrazol-2-yl]methanone1819797: Inhibition of recombinant N-terminal GST-tagged human RIPK1 (1 to 327 residues) expressed in baculovirus infected Sf9 insect cells preincubated for 40 mins followed by substrate addition and measured after 60 mins by ADP-glo luminescence assayec500.0005uM
5-benzyl-N-[(3S)-5-methyl-4-oxo-2,3-dihydro-1,5-benzoxazepin-3-yl]-1,2,4-oxadiazole-3-carboxamide1432226: Inhibition of human RIP1 (1 to 375 residues) expressed in baculovirus infected insect cells preincubated for 1 hr followed by ATP addition measured after 5 hrs by ADP-Glo luminescence assayic500.0005uM
1-benzyl-N-[(3S)-5-methyl-4-oxo-2,3-dihydro-1,5-benzoxazepin-3-yl]-1,2,4-triazole-3-carboxamide1432226: Inhibition of human RIP1 (1 to 375 residues) expressed in baculovirus infected insect cells preincubated for 1 hr followed by ATP addition measured after 5 hrs by ADP-Glo luminescence assayic500.0006uM
5-benzyl-N-[(3S)-7-(methanesulfonamido)-5-methyl-4-oxo-2,3-dihydro-1,5-benzoxazepin-3-yl]-1,2-oxazole-3-carboxamide1432226: Inhibition of human RIP1 (1 to 375 residues) expressed in baculovirus infected insect cells preincubated for 1 hr followed by ATP addition measured after 5 hrs by ADP-Glo luminescence assayic500.0006uM
5-benzyl-N-[(3S)-5-methyl-4-oxo-2,3-dihydro-1,5-benzoxazepin-3-yl]-1,3,4-oxadiazole-2-carboxamide1432226: Inhibition of human RIP1 (1 to 375 residues) expressed in baculovirus infected insect cells preincubated for 1 hr followed by ATP addition measured after 5 hrs by ADP-Glo luminescence assayic500.0008uM
5-benzyl-N-[(3S)-5-methyl-4-oxo-2,3-dihydro-1H-1,5-benzodiazepin-3-yl]-1,2-oxazole-3-carboxamide1290023: Inhibition of human RIP1 (1 to 375 residues) after 4 hrs by ADP-Glo reagent based assayic500.0008uM
5-benzyl-N-[(3S)-5-methyl-4-oxo-2,3-dihydro-1,5-benzoxazepin-3-yl]-1,2-oxazole-3-carboxamide1290029: Competitive inhibition of human RIP1 (1 to 375 residues) in presence of increasing ATP by ADP-Glo reagent based assayki0.0008uM
(3S)-3-(2-benzyl-3-chloro-7-oxo-4,5-dihydropyrazolo[3,4-c]pyridin-6-yl)-5-methyl-4-oxo-2,3-dihydro-1,5-benzoxazepine-8-carbonitrile1469067: Inhibition of fluorescent-labeled 3-(3-((3-(4-amino-5-(4-(3-(2-fluoro-5-(trifluoromethyl)phenyl)ureido)-phenyl)-7H-pyrrolo[2,3-d]pyrimidin-7-yl)propyl)amino)-3-oxopropyl)-5,5-difluoro-7,9-dimethyl-5H-dipyrrolo[1,2-c:2’,1’-f]-[1,3,2]diazaborinin-4-ium-5-uide binding to human N-terminal GST/His-tagged RIPK1 (1 to 375 residues) expressed in baculovirus expression system by TR-FRET assayki0.0009uM
(3S)-3-(2-benzyl-3-chloro-7-oxo-4,5-dihydropyrazolo[3,4-c]pyridin-6-yl)-N,5-dimethyl-4-oxo-2,3-dihydro-1,5-benzoxazepine-8-carboxamide1469067: Inhibition of fluorescent-labeled 3-(3-((3-(4-amino-5-(4-(3-(2-fluoro-5-(trifluoromethyl)phenyl)ureido)-phenyl)-7H-pyrrolo[2,3-d]pyrimidin-7-yl)propyl)amino)-3-oxopropyl)-5,5-difluoro-7,9-dimethyl-5H-dipyrrolo[1,2-c:2’,1’-f]-[1,3,2]diazaborinin-4-ium-5-uide binding to human N-terminal GST/His-tagged RIPK1 (1 to 375 residues) expressed in baculovirus expression system by TR-FRET assayki0.0009uM
(3S)-3-(2-benzyl-3-chloro-7-oxo-4,5-dihydropyrazolo[3,4-c]pyridin-6-yl)-5-methyl-4-oxo-2,3-dihydro-1,5-benzoxazepine-7-carbonitrile1469067: Inhibition of fluorescent-labeled 3-(3-((3-(4-amino-5-(4-(3-(2-fluoro-5-(trifluoromethyl)phenyl)ureido)-phenyl)-7H-pyrrolo[2,3-d]pyrimidin-7-yl)propyl)amino)-3-oxopropyl)-5,5-difluoro-7,9-dimethyl-5H-dipyrrolo[1,2-c:2’,1’-f]-[1,3,2]diazaborinin-4-ium-5-uide binding to human N-terminal GST/His-tagged RIPK1 (1 to 375 residues) expressed in baculovirus expression system by TR-FRET assayki0.0009uM
1-benzyl-N-[(3S)-5-methyl-4-oxo-2,3-dihydro-1,5-benzoxazepin-3-yl]triazole-4-carboxamide1432226: Inhibition of human RIP1 (1 to 375 residues) expressed in baculovirus infected insect cells preincubated for 1 hr followed by ATP addition measured after 5 hrs by ADP-Glo luminescence assayic500.0010uM
5-benzyl-N-[(3S)-5-methyl-4-oxo-7-(1H-pyrazol-5-yl)-2,3-dihydro-1,5-benzoxazepin-3-yl]-1,2-oxazole-3-carboxamide1432226: Inhibition of human RIP1 (1 to 375 residues) expressed in baculovirus infected insect cells preincubated for 1 hr followed by ATP addition measured after 5 hrs by ADP-Glo luminescence assayic500.0010uM
6-[4-[(3S)-3-(3,5-difluorophenyl)-3,4-dihydropyrazole-2-carbonyl]piperidin-1-yl]pyrimidine-4-carbonitrile1554569: Inhibition of human GST/His-tagged RIP1 (1 to 375 residues) expressed in baculovirus expression system assessed as reduction in autophosphorylation measured after 4 hrs in presence of ATP by ADP-Glo luminescence assayic500.0010uM
(3S)-3-[2-[(2,6-difluorophenyl)methyl]-7-oxo-4,5-dihydropyrazolo[3,4-c]pyridin-6-yl]-5-methyl-2,3-dihydro-1,5-benzoxazepin-4-one1469067: Inhibition of fluorescent-labeled 3-(3-((3-(4-amino-5-(4-(3-(2-fluoro-5-(trifluoromethyl)phenyl)ureido)-phenyl)-7H-pyrrolo[2,3-d]pyrimidin-7-yl)propyl)amino)-3-oxopropyl)-5,5-difluoro-7,9-dimethyl-5H-dipyrrolo[1,2-c:2’,1’-f]-[1,3,2]diazaborinin-4-ium-5-uide binding to human N-terminal GST/His-tagged RIPK1 (1 to 375 residues) expressed in baculovirus expression system by TR-FRET assayki0.0011uM
2-benzyl-6-[(3S)-5-methyl-4-oxo-2,3-dihydro-1,5-benzoxazepin-3-yl]-7-oxo-4,5-dihydropyrazolo[3,4-c]pyridine-3-carbonitrile1469067: Inhibition of fluorescent-labeled 3-(3-((3-(4-amino-5-(4-(3-(2-fluoro-5-(trifluoromethyl)phenyl)ureido)-phenyl)-7H-pyrrolo[2,3-d]pyrimidin-7-yl)propyl)amino)-3-oxopropyl)-5,5-difluoro-7,9-dimethyl-5H-dipyrrolo[1,2-c:2’,1’-f]-[1,3,2]diazaborinin-4-ium-5-uide binding to human N-terminal GST/His-tagged RIPK1 (1 to 375 residues) expressed in baculovirus expression system by TR-FRET assayki0.0011uM
N-(6-propan-2-ylsulfonylquinolin-4-yl)-1,3-benzothiazol-6-amine1580388: Inhibition of human RIPK1 by ADP-Glo assayic500.0013uM
1-[3-(3,5-difluorophenyl)-3,4-dihydropyrazol-2-yl]-2,2-dimethylpropan-1-one1554569: Inhibition of human GST/His-tagged RIP1 (1 to 375 residues) expressed in baculovirus expression system assessed as reduction in autophosphorylation measured after 4 hrs in presence of ATP by ADP-Glo luminescence assayic500.0013uM
1-[3-[5-(3-aminophenyl)-1H-pyrrolo[2,3-b]pyridin-3-yl]phenyl]-3-[2-fluoro-5-(trifluoromethyl)phenyl]urea1065034: Displacement of (14-(2-{[3-({2-{[4-(cyanomethyl)phenyl]amino}-6-[(5-cyclopropyl-1H-pyrazol-3-yl)amino]-4-pyrimidinyl}amino)propyl]amino}-2-oxoethyl)-16,16,18,18-tetramethyl-6,7,7a,8a,9,10,16,18-octahydrobenzo[2’’,3’’]indolizino[8’’,7’’:5’,6’]pyrano[3’,2’:3,4]pyrido[1,2-a]indol-5-ium-2-sulfonate from human N-terminal His-GST-TEV-fused RIP1 kinase domain (1 to 375) expressed in baculovirus infected insect Sf9 cells preincubated for 10 mins followed by fluorescent ligand addition measured after 15 mins by fluorescence polarization assayic500.0013uM
1-[3-(3-fluoro-4-methylphenyl)-3,4-dihydropyrazol-2-yl]-2,2-dimethylpropan-1-one2127037: Binding affinity to recombinant RIPK1 (unknown origin) assessed as dissociation constant by KINOMEscan assaykd0.0016uM
2,2-dimethyl-1-[(3S)-3-phenyl-3,4-dihydropyrazol-2-yl]propan-1-one1554569: Inhibition of human GST/His-tagged RIP1 (1 to 375 residues) expressed in baculovirus expression system assessed as reduction in autophosphorylation measured after 4 hrs in presence of ATP by ADP-Glo luminescence assayic500.0016uM
[(3S)-3-phenyl-3,4-dihydropyrazol-2-yl]-(1-pyrimidin-2-ylpiperidin-4-yl)methanone1554569: Inhibition of human GST/His-tagged RIP1 (1 to 375 residues) expressed in baculovirus expression system assessed as reduction in autophosphorylation measured after 4 hrs in presence of ATP by ADP-Glo luminescence assayic500.0016uM
cyclohexyl-(3-phenyl-3,4-dihydropyrazol-2-yl)methanone1554569: Inhibition of human GST/His-tagged RIP1 (1 to 375 residues) expressed in baculovirus expression system assessed as reduction in autophosphorylation measured after 4 hrs in presence of ATP by ADP-Glo luminescence assayic500.0016uM
(3S)-3-[2-[(2-fluorophenyl)methyl]-7-oxo-4,5-dihydropyrazolo[3,4-c]pyridin-6-yl]-5-methyl-2,3-dihydro-1,5-benzoxazepin-4-one1469067: Inhibition of fluorescent-labeled 3-(3-((3-(4-amino-5-(4-(3-(2-fluoro-5-(trifluoromethyl)phenyl)ureido)-phenyl)-7H-pyrrolo[2,3-d]pyrimidin-7-yl)propyl)amino)-3-oxopropyl)-5,5-difluoro-7,9-dimethyl-5H-dipyrrolo[1,2-c:2’,1’-f]-[1,3,2]diazaborinin-4-ium-5-uide binding to human N-terminal GST/His-tagged RIPK1 (1 to 375 residues) expressed in baculovirus expression system by TR-FRET assayki0.0017uM
(3S)-3-[2-[(2,6-difluorophenyl)methyl]-7-oxo-4,5-dihydropyrazolo[3,4-c]pyridin-6-yl]-5-methyl-4-oxo-2,3-dihydro-1,5-benzoxazepine-8-carbonitrile1469067: Inhibition of fluorescent-labeled 3-(3-((3-(4-amino-5-(4-(3-(2-fluoro-5-(trifluoromethyl)phenyl)ureido)-phenyl)-7H-pyrrolo[2,3-d]pyrimidin-7-yl)propyl)amino)-3-oxopropyl)-5,5-difluoro-7,9-dimethyl-5H-dipyrrolo[1,2-c:2’,1’-f]-[1,3,2]diazaborinin-4-ium-5-uide binding to human N-terminal GST/His-tagged RIPK1 (1 to 375 residues) expressed in baculovirus expression system by TR-FRET assayki0.0018uM
N-(5-benzyl-1,3-thiazol-2-yl)-1-methyl-2-oxo-4,5-dihydro-3H-1-benzazepine-3-carboxamide2139608: Inhibition of human RIPK1 assessed as inhibition constant by ADP-Glo Kinase assayki0.0019uM
1-[4-(4-aminofuro[2,3-d]pyrimidin-5-yl)phenyl]-3-[2-fluoro-5-(trifluoromethyl)phenyl]urea1469067: Inhibition of fluorescent-labeled 3-(3-((3-(4-amino-5-(4-(3-(2-fluoro-5-(trifluoromethyl)phenyl)ureido)-phenyl)-7H-pyrrolo[2,3-d]pyrimidin-7-yl)propyl)amino)-3-oxopropyl)-5,5-difluoro-7,9-dimethyl-5H-dipyrrolo[1,2-c:2’,1’-f]-[1,3,2]diazaborinin-4-ium-5-uide binding to human N-terminal GST/His-tagged RIPK1 (1 to 375 residues) expressed in baculovirus expression system by TR-FRET assayki0.0020uM
4-[4-[(3S)-3-(3,5-difluorophenyl)-3,4-dihydropyrazole-2-carbonyl]piperidin-1-yl]pyrimidine-2-carbonitrile1554570: Inhibition of RIP1 in human U937 cells assessed as reduction in TNFalpha/QVD-Oph-induced necroptosis measured after 24 hrs by cell titer-glo luminescent cell viability assayic500.0020uM
5-[4-[(3S)-3-(3,5-difluorophenyl)-3,4-dihydropyrazole-2-carbonyl]piperidin-1-yl]pyrazine-2-carbonitrile1554569: Inhibition of human GST/His-tagged RIP1 (1 to 375 residues) expressed in baculovirus expression system assessed as reduction in autophosphorylation measured after 4 hrs in presence of ATP by ADP-Glo luminescence assayic500.0020uM
4-benzyl-N-[(3S)-5-methyl-4-oxo-2,3-dihydro-1,5-benzoxazepin-3-yl]pyridine-2-carboxamide1432226: Inhibition of human RIP1 (1 to 375 residues) expressed in baculovirus infected insect cells preincubated for 1 hr followed by ATP addition measured after 5 hrs by ADP-Glo luminescence assayic500.0020uM
(3-phenyl-3,4-dihydropyrazol-2-yl)-[1-(pyridine-2-carbonyl)piperidin-4-yl]methanone1554569: Inhibition of human GST/His-tagged RIP1 (1 to 375 residues) expressed in baculovirus expression system assessed as reduction in autophosphorylation measured after 4 hrs in presence of ATP by ADP-Glo luminescence assayic500.0025uM
[(3S)-3-phenyl-3,4-dihydropyrazol-2-yl]-(1-pyridin-2-ylpiperidin-4-yl)methanone1554569: Inhibition of human GST/His-tagged RIP1 (1 to 375 residues) expressed in baculovirus expression system assessed as reduction in autophosphorylation measured after 4 hrs in presence of ATP by ADP-Glo luminescence assayic500.0025uM
2-benzyl-6-[(3S)-5-methyl-4-oxo-2,3-dihydro-1,5-benzoxazepin-3-yl]-7-oxo-4,5-dihydropyrazolo[3,4-c]pyridine-3-carboxamide1469067: Inhibition of fluorescent-labeled 3-(3-((3-(4-amino-5-(4-(3-(2-fluoro-5-(trifluoromethyl)phenyl)ureido)-phenyl)-7H-pyrrolo[2,3-d]pyrimidin-7-yl)propyl)amino)-3-oxopropyl)-5,5-difluoro-7,9-dimethyl-5H-dipyrrolo[1,2-c:2’,1’-f]-[1,3,2]diazaborinin-4-ium-5-uide binding to human N-terminal GST/His-tagged RIPK1 (1 to 375 residues) expressed in baculovirus expression system by TR-FRET assayki0.0025uM
5-benzyl-N-[(3S)-6,8-difluoro-5-methyl-4-oxo-2,3-dihydro-1,5-benzoxazepin-3-yl]-1H-1,2,4-triazole-3-carboxamide1532932: Binding affinity to RIP1 (unknown origin) by fluorescence polarization assayic500.0025uM
[1-(1,3-oxazole-5-carbonyl)piperidin-4-yl]-[(3S)-3-phenyl-3,4-dihydropyrazol-2-yl]methanone1554569: Inhibition of human GST/His-tagged RIP1 (1 to 375 residues) expressed in baculovirus expression system assessed as reduction in autophosphorylation measured after 4 hrs in presence of ATP by ADP-Glo luminescence assayic500.0025uM

CTD chemical–gene interactions

115 total (human), top 30 by PubMed support.

ChemicalActions (top 5)PubMed papers
necrostatin-1increases reaction, increases phosphorylation, affects binding, affects cotreatment, affects reaction (+2 more)7
bisphenol Adecreases reaction, increases expression, increases reaction, increases methylation3
benzyloxycarbonyl-valyl-alanyl-aspartic acidaffects cotreatment, affects localization, decreases reaction, increases expression, increases reaction3
Arsenic Trioxideincreases expression, decreases expression, increases cleavage3
Cadmiumaffects binding, increases abundance, increases expression, increases phosphorylation, affects reaction (+4 more)3
moringinaffects cotreatment, decreases expression2
2,2’,4,4’-tetrabromodiphenyl etherdecreases expression, affects cotreatment, affects reaction, decreases reaction2
Resveratrolaffects cotreatment, increases expression, decreases reaction2
Acetylcysteinedecreases reaction, increases expression, increases reaction2
Ethanolaffects binding, increases reaction, increases expression, decreases reaction2
Benzo(a)pyreneincreases expression, increases methylation2
Cannabidioldecreases expression, increases expression, affects cotreatment2
Plant Extractsaffects cotreatment, increases expression, decreases reaction2
3-((6-(2-methoxyphenyl)pyrimidin-4-yl)amino)phenyl)methane sulfonamidedecreases expression1
1-hydroxyalantolactonedecreases reaction, increases ubiquitination1
CBLC137increases expression1
GSK872affects cotreatment, affects reaction, decreases expression, decreases reaction1
dihydrotanshinone Iincreases phosphorylation, decreases reaction1
3-monochloropropane-1, 2 diol esterincreases expression, decreases reaction1
dimethoxondecreases expression1
2-anisidineincreases expression1
propylparabenincreases expression1
beta-lapachoneincreases expression1
methamidophosdecreases expression1
sodium arseniteincreases expression1
acacetinincreases expression1
sodium sulfiteincreases expression1
ochratoxin Adecreases expression1
palmitoyl glyceroldecreases expression1
ferrous chloridedecreases expression1

ChEMBL screening assays

400 unique, capped per target: 391 binding, 7 admet, 2 functional

Representative assays (with source publication via chembl_document):

Assay IDTypeDescriptionSource paper
CHEMBL2416956BindingRatio of necrostatin-21 EC50 to compound EC50 for RIP1/JNK2 (unknown origin)-mediated necrosisA novel necroptosis inhibitor-necrostatin-21 and its SAR study. — Bioorg Med Chem Lett
CHEMBL4344882ADMETDisplacement of fluorescein labeled probe from full length human GST-tagged RIPK1 expressed in Sf9 cells incubated for 60 mins by HTRF assayIdentification of RIPK3 Type II Inhibitors Using High-Throughput Mechanistic Studies in Hit Triage. — ACS Med Chem Lett
CHEMBL5210011FunctionalAffinity Phenotypic Cellular interaction (CytoTox 96 nonradioactive cytotoxicity assay (necroptosis in HT29 cells; Promega)) EUB0000719a RIPK1Affinity Phenotypic Cellular Literature for EUbOPEN Chemogenomics Library wave 3

Cellosaurus cell lines

10 cell lines: 9 cancer cell line, 1 transformed cell line

First 10 cell lines (id-ordered, not curated):

CellosaurusNameCategorySex
CVCL_B1AJAbcam THP-1 RIPK1 KOCancer cell lineMale
CVCL_D7ZFUbigene A-549 RIPK1 KOCancer cell lineMale
CVCL_D8UMUbigene HCT 116 RIPK1 KOCancer cell lineMale
CVCL_D9QJUbigene HEK293 RIPK1 KOTransformed cell lineFemale
CVCL_D9WEUbigene HT-1080 RIPK1 KOCancer cell lineMale
CVCL_E0MSUbigene HeLa RIPK1 KOCancer cell lineFemale
CVCL_E0YPUbigene MDA-MB-231 RIPK1 KOCancer cell lineFemale
CVCL_E1CBUbigene SK-OV-3 RIPK1 KOCancer cell lineFemale
CVCL_TJ10HAP1 RIPK1 (-) 1Cancer cell lineMale
CVCL_TJ11HAP1 RIPK1 (-) 2Cancer cell lineMale

Clinical trials (associated diseases)

99 trials via MONDO — disease-level, not drug-specific.

TrialPhaseStatusTitle
NCT03677557PHASE4UNKNOWNSafety, Tolerability, Patient Satisfaction and Cost of 16.5% Subcutaneous Immunoglobulin (Cutaquig®) Treatment
NCT02562170PHASE4COMPLETEDProtexa® Versus TiLoopBra® in Immediate Breast Reconstruction- A Pilot Study
NCT00001646PHASE3COMPLETEDVoriconazole vs. Amphotericin B in the Treatment of Invasive Aspergillosis
NCT00220766PHASE3COMPLETEDRapid Infusion of Immune Globulin Intravenous (Human) In Primary Immunodeficiency Patients
NCT00468273PHASE3COMPLETEDA Clinical Study of Intravenous Immunoglobulin
NCT00811174PHASE3TERMINATEDEfficacy, Safety and Kinetics Study of Octagam 10% in Primary Immunodeficiency Diseases
NCT01012323PHASE3COMPLETEDA Study of NewGam, Human Immunoglobulin 10%, in Patients With Primary Immunodeficiency Diseases
NCT01313507PHASE3COMPLETEDHigh Infusion Rate Study of Immunoglobulin Intravenous (Human) 10% (NewGam)
NCT01406470PHASE3COMPLETEDPhase 3 Study of Immune Globulin Intravenous (Human)IVIG-SN™ in Subjects With Primary Immunodeficiency
NCT02783482PHASE3COMPLETEDStudy of Immune Globulin Intravenous (Human) GC5107 in Subjects With Primary Humoral Immunodeficiency
NCT02810444PHASE3COMPLETEDStudy to Investigate Efficacy, Safety and Pharmacokinetics of BT595 in Subjects With PID
NCT03961009PHASE3COMPLETEDClinical Assessment of Pharmacokinetics, Efficacy, and Safety of 10% IVIg in PID Patients
NCT04842643PHASE3COMPLETEDAn Extension Study of TAK-664 for Japanese People With Primary Immunodeficiency Disease
NCT04944979PHASE3ACTIVE_NOT_RECRUITINGClinical Assessment of Pharmacokinetics, Efficacy, and Safety of 10% IVIg in Pediatric PID Patients (KIDCARES10)
NCT06089122PHASE3UNKNOWNEfficacy, Safety, and Pharmacokinetics of Shu Yang IVIG
NCT06150833PHASE3UNKNOWNEfficacy and Safety and Pharmacokinetics of Boya IVIG
NCT07346859PHASE3RECRUITINGStudy of BP-SCIG 20% in Patients With Primary Immunodeficiency (PID)
NCT00673335PHASE3COMPLETEDLetrozole in Preventing Breast Cancer in Postmenopausal Women With a BRCA1 or BRCA2 Mutation
NCT00685256PHASE3COMPLETEDStandard Genetic Counseling With or Without a Decision Guide in Improving Communication Between Mothers Undergoing BRCA1/2 Testing and Their Minor-Age Children
NCT03162276PHASE3UNKNOWNTrial of Inquiry Based Stress Reduction (IBSR) Program for BRCA1/2 Mutation Carriers
NCT00001438PHASE2COMPLETEDA Pilot Study of the Combination of Retinoic Acid and Interferon-Alpha2a for the Treatment of Lymphoproliferative Disorders in Children With Immunodeficiency Syndromes
NCT00176865PHASE2COMPLETEDStem Cell Transplant for Immunologic or Histiocytic Disorders
NCT00389324PHASE2COMPLETEDA Trial of the Pharmacokinetics, Safety, and Tolerability of Subcutaneous Gamunex® in Primary Immunodeficiency
NCT00598481PHASE2COMPLETEDADA Gene Transfer Into Hematopoietic Stem/Progenitor Cells for the Treatment of ADA-SCID
NCT01856582PHASE2TERMINATEDCD34+ Stem Cell Infusion to Augment Graft Function
NCT06199427PHASE2RECRUITINGPTCy and and Ruxolitinib for GVHD Prophylaxis After HSCT With Thymoglobulin in Conditioning Regimen in Patients With Inborn Errors of Immunity
NCT00253539PHASE2COMPLETEDArzoxifene or Tamoxifen in Preventing Breast Cancer in Premenopausal Women at High Risk for Breast Cancer
NCT00305695PHASE2COMPLETEDZoledronate or Observation in Maintaining Bone Mineral Density in Patients Who Are Undergoing Surgery to Remove Both Ovaries
NCT00321633PHASE2COMPLETEDCarboplatin or Docetaxel in Treating Women With Metastatic Genetic Breast Cancer
NCT01333748PHASE2COMPLETEDSearch Allelic Imbalance of Expression of BRCA Genes in Hereditary Risk of Breast and/or Ovarian Cancer
NCT01367639PHASE2COMPLETEDTrial of Inquiry Based Stress Reduction (IBSR) Program for BRCA1/2 Mutation Carriers
NCT00535119PHASE1COMPLETEDVeliparib, Carboplatin, and Paclitaxel in Treating Patients With Advanced Solid Cancer
NCT00892736PHASE1COMPLETEDVeliparib in Treating Patients With Malignant Solid Tumors That Do Not Respond to Previous Therapy
NCT00001158Not specifiedCOMPLETEDStudies of the Immune Response in Normal Subjects and Patients With Disorders of the Immune System
NCT00001336Not specifiedCOMPLETEDIn Vitro Studies of Immunological and Stem Cell Function in Peripheral Blood Mononuclear Cells in Patients
NCT00001788Not specifiedTERMINATEDGenetic Basis of Primary Immunodeficiencies
NCT00006054Not specifiedTERMINATEDAllogeneic Bone Marrow Transplantation in Patients With Primary Immunodeficiencies
NCT00006131Not specifiedCOMPLETEDRandomized Study of Two Doses of Oral Valacyclovir in Immunocompromised Patients With Uncomplicated Herpes Zoster
NCT01150240Not specifiedUNKNOWNClinical and Laboratory Online Patient- and Research Database for Primary Immunodeficiencies in Switzerland
NCT01727895Not specifiedCOMPLETEDEffects of Orally Administered Beta-glucan on Leukocyte Function in Humans