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Atlas / Disease / Marginal zone lymphoma

Marginal zone lymphoma

disease
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Also known as lymphoma of marginal zone B cellmarginal zone B cell lymphomamarginal zone B-cell lymphomaMZBCLMZL

Summary

Marginal zone lymphoma (MONDO:0017604) is a cancer with 12 cohort genes (23 GWAS associations across 7 studies; 3 CIViC-evidence somatic drivers) and 341 clinical trials. Top therapeutic interventions include cyclophosphamide anhydrous, ublituximab, and zanubrutinib.

At a glance

  • Classification: Cancer
  • Prevalence: 1-9 / 100 000 (Europe) [Orphanet-validated]
  • Cohort genes: 12
  • GWAS associations: 23
  • Clinical trials: 341

Clinical features

Epidemiology

Prevalence records

3 prevalence record(s), Orphanet:

TypeClassValueGeographyValidation
Annual incidence1-9 / 1 000 0000.3EuropeValidated
Point prevalence1-9 / 100 0007EuropeValidated
Annual incidence1-9 / 100 0002.8FranceValidated

Identifiers

Disease identifiers

FieldValue
Canonical namemarginal zone lymphoma
Mondo IDMONDO:0017604
EFOEFO:1000630
Orphanet300912
DOIDDOID:0050748
NCITC4341
SNOMED CT447100004
UMLSC1367654
MedGen277950
GARD0013237
Is cancer (heuristic)yes

Also known as: lymphoma of marginal zone B cell · marginal zone B cell lymphoma · marginal zone B-cell lymphoma · marginal zone lymphoma · MZBCL · MZL

Data availability: 23 GWAS associations (7 studies).

Disease family

An umbrella term covering 4 Mondo subtypes.

Classification path: disease › human disease › disease by body system or component › immune system disorder › leukocyte disorder › B-cell neoplasm › B-cell non-Hodgkin lymphoma › indolent B-cell non-Hodgkin lymphoma › marginal zone lymphoma

Related subtypes (5): B-cell chronic lymphocytic leukemia, indolent primary cutaneous B-cell lymphoma, lymphoplasmacytic lymphoma without IgM production, follicular lymphoma, Waldenstrom macroglobulinemia

Subtypes (4): MALT lymphoma, splenic diffuse red pulp small B-cell lymphoma, splenic marginal zone lymphoma, nodal marginal zone B-cell lymphoma

Genetics & variants

GWAS landscape

23 GWAS associations across 7 studies. Top hits map to 15 distinct genes (as reported by GWAS).

Top associations by p-value

rsIDp-valueGeneRisk alleleOdds ratio
rs94617414e-15BTNL2, TSBP1-AS1C2.66
rs13642292e-10RNU6-21P - DPPA3P11A1.35
rs29229942e-09HLA-B - RNU6-283PG1.64
rs111871573e-09Y_RNA - EXOC6C1.15
rs169471225e-09FBXW8C1.86
rs13232922e-08RGS2-AS1?1.12
rs27778992e-08VMP1?1.09
rs80217412e-08BATF - FLVCR2-AS1?1.09
rs60623142e-08ZBTB46?1.16
rs14447662e-08KALRNG1.17
rs29415092e-08IKZF3T1.41
rs11073453e-08IL2RA?1.11
rs14391123e-08MGAT5?1.17
rs73821706e-08ATXN1-AS1 - STMND1?1.15
rs12505509e-08ZMIZ1?1.09
rs24257521e-07NCOA5?1.1
rs92686713e-07HLA-DRA - HLA-DRB9?0.75
rs730830743e-07STK38L?12.56
rs170982464e-07PPM1H?0.56
rs583196885e-07PPM1H?0.56
rs767880972e-06PRKCHG1.53
rs765884273e-06LINC02850 - APOBA2.05
rs94216846e-06LINC02655 - RPS7P9A1.14

Top studies (by case count)

StudyLead authorYearCasesControlsTitle
GCST90267402Berndt SI20223,1009,505Distinct germline genetic susceptibility profiles identified for common non-Hodgkin lymphoma subtypes.
GCST002742Vijai J20158256,221A genome-wide association study of marginal zone lymphoma shows association to the HLA region.
GCST008723Din L201974123,367Genetic overlap between autoimmune diseases and non-Hodgkin lymphoma subtypes.
GCST008726Din L201974110,070Genetic overlap between autoimmune diseases and non-Hodgkin lymphoma subtypes.
GCST008729Din L201974121,982Genetic overlap between autoimmune diseases and non-Hodgkin lymphoma subtypes.
GCST90624748Guler M2025623656,255Clustering of lymphoid neoplasms by cell of origin, somatic mutation and drug usage profiles: a multi-trait genome-wide association study.
GCST90704651Kleinstern G2025450European-based polygenic risk score and genome-wide association study of B-cell non-Hodgkin lymphoma subtypes in Israeli Jews and Palestinian Arabs.

Variant details and genetic-evidence tiers

Tier distribution (top 50 variants)

TierVariants
Tier 1: coding0
Tier 2: splice/UTR1
Tier 3: regulatory1
Tier 4: intronic/intergenic21

MAF distribution

BucketVariants
common (>=0.05)20
low_freq (0.01-0.05)2
rare (<0.01)0
unknown1

Functional consequences

ConsequenceCount
intron_variant15
intergenic_variant6
regulatory_region_variant1
3_prime_UTR_variant1

Top variants

rsIDChrPosAllelesMAFConsequenceGenep-valueTier
rs9461741632402810G>A,C,T0.018intron_variantBTNL2, TSBP1-AS14e-15Tier 4: intronic/intergenic
rs13642291662594871T>C,G0.05intergenic_variantRNU6-21P - DPPA3P112e-10Tier 4: intronic/intergenic
rs2922994631368124A>C,G0.113intron_variantHLA-B - RNU6-283P2e-09Tier 4: intronic/intergenic
rs111871571092742487T>C0.05regulatory_region_variantY_RNA - EXOC63e-09Tier 3: regulatory
rs1694712212116928726C>T0.05intron_variantFBXW85e-09Tier 4: intronic/intergenic
rs13232921192571891G>A,C,T0.05intron_variantRGS2-AS12e-08Tier 4: intronic/intergenic
rs27778991759755030T>A,G0.05intron_variantVMP12e-08Tier 4: intronic/intergenic
rs80217411475556057T>G0.05intergenic_variantBATF - FLVCR2-AS12e-08Tier 4: intronic/intergenic
rs60623142063778360C>A,G,T0.05intron_variantZBTB462e-08Tier 4: intronic/intergenic
rs14447663124206424A>G,T0.05intron_variantKALRN2e-08Tier 4: intronic/intergenic
rs29415091739764941T>A,C0.053_prime_UTR_variantIKZF32e-08Tier 2: splice/UTR
rs1107345106045332G>T0.05intron_variantIL2RA3e-08Tier 4: intronic/intergenic
rs14391122134305027G>A0.05intron_variantMGAT53e-08Tier 4: intronic/intergenic
rs7382170616970739C>A0.05intergenic_variantATXN1-AS1 - STMND16e-08Tier 4: intronic/intergenic
rs12505501079300560C>A,G0.05intron_variantZMIZ19e-08Tier 4: intronic/intergenic
rs24257522046073481T>C,G0.05intron_variantNCOA51e-07Tier 4: intronic/intergenic
rs9268671632446513A>C,G,T0.05intron_variantHLA-DRA - HLA-DRB93e-07Tier 4: intronic/intergenic
rs730830741227275024C>Gintron_variantSTK38L3e-07Tier 4: intronic/intergenic
rs170982461262732293G>A0.05intron_variantPPM1H4e-07Tier 4: intronic/intergenic
rs583196881262732665G>A,T0.05intron_variantPPM1H5e-07Tier 4: intronic/intergenic
rs767880971461262775A>G0.062intergenic_variantPRKCH2e-06Tier 4: intronic/intergenic
rs76588427220935927G>A0.017intergenic_variantLINC02850 - APOB3e-06Tier 4: intronic/intergenic
rs94216841080702590G>A0.05intergenic_variantLINC02655 - RPS7P96e-06Tier 4: intronic/intergenic

Genes & proteins

Mendelian disease overlap and somatic drivers

GenCC: 0 · Orphanet: 14 · OMIM-shared: 0 · Dual-evidence (GWAS+Mendelian): 0

Somatic driver evidence (intOGen + CIViC, cohort fanout)

GeneintOGen roleCancer typesCIViC
TCF19CIViC #5654
IKZF3ActCHOL,CLLSLL,DLBCLNOS
HLA-BLoFCESC,DLBCLNOS,ESCA,HNSC,MLYMCIViC #2607

Orphanet rare-disease linkage (cohort genes)

GeneOrphanet IDRare disease
BTNL2Orphanet:797Sarcoidosis
IKZF3Orphanet:67038B-cell chronic lymphocytic leukemia
IKZF3Orphanet:699590Immune dysregulation with immunodeficiency due to AIOLOS haploinsufficiency
IKZF3Orphanet:699593Combined immunodeficiency-lymphopenia-cancer predisposing syndrome due to AIOLOS deficiency
ZMIZ1Orphanet:528084Non-specific syndromic intellectual disability
HLA-BOrphanet:117Behçet disease
HLA-BOrphanet:275798Pulmonary arterial hypertension associated with connective tissue disease
HLA-BOrphanet:29207Reactive arthritis
HLA-BOrphanet:3287Takayasu arteritis
HLA-BOrphanet:36426Stevens-Johnson syndrome
HLA-BOrphanet:397Giant cell arteritis
IL2RAOrphanet:169100Immunodeficiency due to CD25 deficiency
IL2RAOrphanet:85408Rheumatoid factor-negative polyarticular juvenile idiopathic arthritis
IL2RAOrphanet:85410Oligoarticular juvenile idiopathic arthritis

Cohort genes → proteins

12 cohort genes, 12 distinct canonical proteins.

Evidence partition

SubsetGenes
gwas_only12

Cohort genes (full)

SymbolHGNCEnsemblUniProtNameEvidence
BTNL2HGNC:1142ENSG00000204290Q9UIR0Butyrophilin-like protein 2gwas
TCF19HGNC:11629ENSG00000137310Q9Y242Transcription factor 19gwas
IKZF3HGNC:13178ENSG00000161405Q9UKT9Zinc finger protein Aiolosgwas
FBXW8HGNC:13597ENSG00000174989Q8N3Y1F-box/WD repeat-containing protein 8gwas
NCOA5HGNC:15909ENSG00000124160Q9HCD5Nuclear receptor coactivator 5gwas
ZBTB46HGNC:16094ENSG00000130584Q86UZ6Zinc finger and BTB domain-containing protein 46gwas
ZMIZ1HGNC:16493ENSG00000108175Q9ULJ6Zinc finger MIZ domain-containing protein 1gwas
VMP1HGNC:29559ENSG00000062716Q96GC9Vacuole membrane protein 1gwas
KALRNHGNC:4814ENSG00000160145O60229Kaliringwas
HLA-BHGNC:4932ENSG00000234745P01889HLA class I histocompatibility antigen, B alpha chaingwas
IL2RAHGNC:6008ENSG00000134460P01589Interleukin-2 receptor subunit alphagwas
MGAT5HGNC:7049ENSG00000152127Q09328Alpha-1,6-mannosylglycoprotein 6-beta-N-acetylglucosaminyltransferase Agwas

Cohort function summary

Lead sentence per gene, UniProt-curated.

SymbolProtein nameFunction (lead sentence)
BTNL2Butyrophilin-like protein 2Negative regulator of T-cell proliferation.
TCF19Transcription factor 19Potential transcription factor that may play a role in the regulation of genes involved in cell cycle G1/S transition.
IKZF3Zinc finger protein AiolosTranscription factor that plays an important role in the regulation of lymphocyte differentiation.
FBXW8F-box/WD repeat-containing protein 8Substrate-recognition component of the Cul7-RING(FBXW8) ubiquitin ligase complex, which mediates the ubiquitination and subsequent proteasomal degradation of target proteins.
NCOA5Nuclear receptor coactivator 5Nuclear receptor coregulator that can have both coactivator and corepressor functions.
ZBTB46Zinc finger and BTB domain-containing protein 46Transcription regulator that mediates differentiation of conventional and non-conventional dendritic cells, and which is involved in tolerance to gut microbiota.
ZMIZ1Zinc finger MIZ domain-containing protein 1Acts as a transcriptional coactivator.
VMP1Vacuole membrane protein 1Phospholipid scramblase involved in lipid homeostasis and membrane dynamics processes.
KALRNKalirinPromotes the exchange of GDP by GTP.
HLA-BHLA class I histocompatibility antigen, B alpha chainAntigen-presenting major histocompatibility complex class I (MHCI) molecule.
IL2RAInterleukin-2 receptor subunit alphaReceptor for interleukin-2.
MGAT5Alpha-1,6-mannosylglycoprotein 6-beta-N-acetylglucosaminyltransferase ACatalyzes the addition of N-acetylglucosamine (GlcNAc) in beta 1-6 linkage to the alpha-linked mannose of biantennary N-linked oligosaccharides.

Protein-family classification

Druggable: 5 · Difficult: 5 · Unknown: 2 · Druggable fraction: 0.42

Family distribution

Cohort families vs a genome-wide background (hypergeometric, BH-FDR; fold = observed/expected). Counts kept; sorted by enrichment, so the catch-all Other/Unknown bucket no longer leads.

FamilyGenesFoldFDR
Complement122.3×0.144
Antibody/Immunoglobulin24.9×0.144
Transcription factor42.8×0.144
Kinase12.3×0.624
Scaffold/PPI11.4×0.715
Enzyme (other)11.0×0.756
Other/Unknown20.3×0.999

Per-gene assignment

SymbolFamilyDruggable?ECInterPro (top 3)
BTNL2Antibody/ImmunoglobulinyesIg_C1-set, Ig_sub, Ig-like_dom
TCF19Transcription factornoFHA_dom, Znf_PHD, SMAD_FHA_dom_sf
IKZF3Transcription factornoZnf_C2H2_type, Znf_C2H2_sf, Ikaros_C2H2-ZF
FBXW8Scaffold/PPInoWD40_rpt, F-box_dom, Quinoprotein_ADH-like_sf
NCOA5Other/UnknownnoAnticodon-bd_dom_sf, Nuc_rcpt_coact/corep
ZBTB46Transcription factornoBTB/POZ_dom, SKP1/BTB/POZ_sf, Znf_C2H2_type
ZMIZ1Transcription factornoZnf_MIZ, Znf_RING/FYVE/PHD, ZMIZ1_N
VMP1Other/Unknownno
KALRNKinaseyesDH_dom, Prot_kinase_dom, CRAL-TRIO_dom
HLA-BAntibody/ImmunoglobulinyesMHC_I_a_a1/a2, Ig/MHC_CS, Ig_C1-set
IL2RAComplementyesSushi_SCR_CCP_dom, IL-2_rcpt_alpha, Sushi/SCR/CCP_sf
MGAT5Enzyme (other)yes2.4.1.155GT18_cat, MGT5A-like_N, MGAT5_Glycosyltransferase

Expression context

Cohort genes with no expression data: 0.

8 cohort genes are a single-cell marker in ≥1 SCXA experiment.

Breadth distribution (Bgee present_calls)

BucketGenes
narrow (1-5 tissues)0
moderate (6-20)0
broad (>20)12
unknown0

Top tissues across cohort

TissueCohort genes
primordial germ cell in gonad3
ventricular zone3
lymph node3
granulocyte2
oviduct epithelium2
blood2
sural nerve1
epithelium of nasopharynx1
stromal cell of endometrium1
cortical plate1
ileal mucosa1
right hemisphere of cerebellum1
tendon of biceps brachii1
dorsal motor nucleus of vagus nerve1
seminal vesicle1
tibia1
monocyte1
mononuclear cell1
frontal pole1
oocyte1

Per-gene tissue summary (top 30)

SymbolBgee breadthFANTOM5 breadthSCXATop tissues
BTNL2106yessural nerve, ventricular zone, primordial germ cell in gonad
TCF19133ubiquitousyesprimordial germ cell in gonad, ventricular zone, lymph node
IKZF3155broadmarkergranulocyte, lymph node, epithelium of nasopharynx
FBXW8175ubiquitousyesstromal cell of endometrium, primordial germ cell in gonad, ventricular zone
NCOA5231ubiquitousmarkeroviduct epithelium, ileal mucosa, cortical plate
ZBTB46215ubiquitousyesoviduct epithelium, tendon of biceps brachii, right hemisphere of cerebellum
ZMIZ1295ubiquitousmarkerdorsal motor nucleus of vagus nerve, tibia, seminal vesicle
VMP1295ubiquitousmarkerblood, monocyte, mononuclear cell
KALRN257ubiquitousmarkersecondary oocyte, oocyte, frontal pole
HLA-B134ubiquitousmarkerblood, spleen, granulocyte
IL2RA153broadmarkerlymph node, vermiform appendix, caecum
MGAT5282ubiquitousmarkerrenal glomerulus, metanephric glomerulus, middle temporal gyrus

Protein interactions among cohort

Intra-cohort edges: 0.

Hub genes (top 10 by interactor count)

SymbolInteractor count
IKZF33,285
HLA-B3,209
IL2RA2,557
NCOA52,462
MGAT52,286
TCF191,774
ZMIZ11,773
FBXW81,617
KALRN1,603
VMP11,567

Structural data

PDB: 7 · AlphaFold-only: 5 · No structure: 0

Cohort genes with PDB structures (top 30)

SymbolUniProtPDB entries
HLA-BP01889237
MGAT5Q0932814
KALRNO6022913
IL2RAP0158910
NCOA5Q9HCD54
FBXW8Q8N3Y11
ZMIZ1Q9ULJ61

AlphaFold-only cohort genes (top 30 by pLDDT)

SymbolUniProtpLDDT
BTNL2Q9UIR085.97
VMP1Q96GC978.64
TCF19Q9Y24263.17
ZBTB46Q86UZ655.56
IKZF3Q9UKT948.06

Function

Pathway analysis

Distinct Reactome pathways touched by cohort: 55. Enrichment computed across 12 evidence-associated genes (8 with Reactome annotation).

Pathways by enrichment

Over-representation of cohort genes vs the genome-wide background (hypergeometric test, Benjamini-Hochberg FDR; fold = observed/expected over 8 annotated cohort genes). Counts and members are kept as ground-truth; sorted by enrichment.

PathwayCohort genesFoldFDRSample cohort genes
Interaction of NuRD complexes with transcription factors231.7×0.092TCF19, IKZF3
RUNX1 and FOXP3 control the development of regulatory T lymphocytes (Tregs)1142.8×0.096IL2RA
Endosomal/Vacuolar pathway1129.8×0.096HLA-B
Interleukin-2 signaling1119.0×0.096IL2RA
Butyrophilin (BTN) family interactions1109.8×0.096BTNL2
N-Glycan antennae elongation195.2×0.096MGAT5
N-glycan antennae elongation in the medial/trans-Golgi171.4×0.101MGAT5
DAP12 interactions159.5×0.101HLA-B
Interleukin receptor SHC signaling151.0×0.101IL2RA
Translation of Structural Proteins151.0×0.101MGAT5
Antigen Presentation: Folding, assembly and peptide loading of class I MHC149.2×0.101HLA-B
Late SARS-CoV-2 Infection Events136.6×0.117MGAT5
EPHB-mediated forward signaling133.2×0.117KALRN
Maturation of spike protein133.2×0.117MGAT5
Cell death signalling via NRAGE, NRIF and NADE127.4×0.131KALRN
p75 NTR receptor-mediated signalling123.4×0.136KALRN
NRAGE signals death through JNK123.0×0.136KALRN
EPH-Ephrin signaling120.7×0.136KALRN
Interferon alpha/beta signaling119.0×0.136HLA-B
RHOG GTPase cycle118.5×0.136KALRN
Death Receptor Signaling117.4×0.136KALRN
G alpha (12/13) signalling events117.2×0.136KALRN
MAPK6/MAPK4 signaling117.0×0.136KALRN
ER-Phagosome pathway116.2×0.136HLA-B
Interferon gamma signaling115.7×0.136HLA-B
MAPK family signaling cascades112.9×0.153KALRN
Transport to the Golgi and subsequent modification112.9×0.153MGAT5
SARS-CoV-2 activates/modulates innate and adaptive immune responses111.2×0.167HLA-B
Immunoregulatory interactions between a Lymphoid and a non-Lymphoid cell110.9×0.167HLA-B
SARS-CoV-2 Infection110.1×0.175MGAT5

GO biological processes by enrichment

Over-representation of cohort genes vs the genome-wide background (hypergeometric test, Benjamini-Hochberg FDR; fold = observed/expected over 12 annotated cohort genes). Counts and members are kept as ground-truth; sorted by enrichment.

GO termCohort genesFoldFDRSample cohort genes
regulation of T cell tolerance induction11404.3×0.018IL2RA
plasmacytoid dendritic cell differentiation1702.2×0.018ZBTB46
positive regulation of ATPase-coupled calcium transmembrane transporter activity1702.2×0.018VMP1
regulation of CD4-positive, alpha-beta T cell proliferation1702.2×0.018IL2RA
negative regulation of leukocyte activation1468.1×0.018ZBTB46
pyramidal neuron migration to cerebral cortex1468.1×0.018ZMIZ1
regulation of lymphocyte differentiation1468.1×0.018IKZF3
regulation of T cell homeostatic proliferation1468.1×0.018IL2RA
organelle localization by membrane tethering1468.1×0.018VMP1
regulation of dendritic cell differentiation1468.1×0.018HLA-B
tolerance induction in gut-associated lymphoid tissue1351.1×0.018ZBTB46
regulation of T cell anergy1351.1×0.018HLA-B
regulation of B cell proliferation1351.1×0.018IKZF3
regulation of interleukin-12 production1351.1×0.018HLA-B
positive regulation of dendritic cell differentiation1351.1×0.018ZBTB46
positive regulation of T cell differentiation275.9×0.018ZMIZ1, IL2RA
negative regulation of insulin receptor signaling pathway262.4×0.018FBXW8, NCOA5
vitellogenesis1280.9×0.020ZMIZ1
Golgi organization222.3×0.020FBXW8, VMP1
protection from natural killer cell mediated cytotoxicity1234.1×0.021HLA-B
activation-induced cell death of T cells1200.6×0.021IL2RA
autophagosome membrane docking1200.6×0.021VMP1
negative regulation of monocyte differentiation1200.6×0.021ZBTB46
negative regulation of dendritic cell differentiation1200.6×0.021ZBTB46
negative regulation of granulocyte differentiation1175.5×0.021ZBTB46
interleukin-2-mediated signaling pathway1175.5×0.021IL2RA
regulatory T cell differentiation1175.5×0.021ZBTB46
negative regulation of macrophage differentiation1175.5×0.021ZBTB46
obsolete mitochondrion-endoplasmic reticulum membrane tethering1175.5×0.021VMP1
activated T cell proliferation1156.0×0.023IL2RA

Therapeutics

Drugs indicated for this disease

0 approved, 8 in late-stage (phase 3) trials. Disease-direct ChEMBL indications, not inferred from the associated-gene cohort below.

DrugDevelopment status
BendamustinePhase 3 (in late-stage trials)
DextrosePhase 3 (in late-stage trials)
DoxorubicinPhase 3 (in late-stage trials)
IbrutinibPhase 3 (in late-stage trials)
LenalidomidePhase 3 (in late-stage trials)
OrelabrutinibPhase 3 (in late-stage trials)
PrednisonePhase 3 (in late-stage trials)
RituximabPhase 3 (in late-stage trials)

Earlier-phase candidates (phase 2, investigational — efficacy not yet established): Acalabrutinib, Copanlisib, Everolimus, Mosunetuzumab, Obinutuzumab, Pembrolizumab, Pirtobrutinib, Polatuzumab Vedotin, Tafasitamab, Venetoclax, Zanubrutinib.

Drug target analysis

Approved (phase 4): 1 · Phase ≥3: 1 · Phased (≥1): 1 · Undrugged: 11

Druggability breadth: 5 of 12 evidence-associated genes (42%) have a ChEMBL target (buckets above are over the deeply-mined display cohort).

Genes with an approved drug

The molecule shown is one approved compound that hits the gene — not necessarily a drug of choice or one indicated for this disease.

SymbolExample approved molecule
IKZF3POMALIDOMIDE

Top cohort targets by molecule count

SymbolMoleculesMax phase
IKZF354
BTNL200
TCF1900
FBXW800
NCOA500
ZBTB4600
ZMIZ100
VMP100
KALRN00
HLA-B00

Drugs targeting cohort genes (top 30)

MoleculeMax phaseTargets in cohort
POMALIDOMIDE4IKZF3
LENALIDOMIDE4IKZF3
THALIDOMIDE4IKZF3
IBERDOMIDE3IKZF3
AVADOMIDE2IKZF3

Bioactivity and enzyme data

Enzyme cohort genes (≥1 EC): 1.

Cohort genes with ChEMBL bioactivity (full, sorted by assay count)

SymbolAssaysType breakdown
IKZF3101Binding:100, Functional:1
IL2RA2Binding:2
VMP11Binding:1
HLA-B1Binding:1
MGAT51Binding:1

Cohort enzymes (BRENDA EC)

SymbolEC numbersNames
MGAT52.4.1.155alpha-1,6-mannosyl-glycoprotein 6-beta-N-acetylglucosaminyltransferase

Cohort genes with high screening signal

≥100 ChEMBL assays — a studied-ness signal; see Therapeutics for approved-drug status.

SymbolChEMBL assays
IKZF3101

Pharmacogenomics

Cohort genes with a PharmGKB record: 12; with CPIC/DPWG dosing guidelines: 1.

Cohort genes with a CPIC/DPWG dosing guideline

SymbolCPIC guidelines
HLA-B1

Drug repurposing candidates

5 approved/phased drugs hit cohort targets but don’t yet appear in disease-level clinical trials. Target-inhibition rationale is strongest for cancer driver genes; a bioactivity hit is a screening signal, not a treatment claim.

CompoundMax phaseCohort target (bioactivity)
POMALIDOMIDE4IKZF3
LENALIDOMIDE4IKZF3
THALIDOMIDE4IKZF3
IBERDOMIDE3IKZF3
AVADOMIDE2IKZF3

Druggability pyramid

Cohort genes binned by druggability tier (high → low):

TierDefinitionGenesSymbols
AApproved (phase 4 drug)1IKZF3
BPhased (≥1) drug, not yet approved0
CDruggable family + PDB, no drug4KALRN, HLA-B, IL2RA, MGAT5
DDruggable family + AlphaFold only, no drug1BTNL2
EDifficult family or no structure, no drug6TCF19, FBXW8, NCOA5, ZBTB46, ZMIZ1, VMP1

Undrugged target profiles

11 cohort genes are undrugged. Ranked by ‘starting-point quality’ (assay depth + drugged-partner adjacency).

SymbolChEMBL assaysDrugged partners (top 3)
BTNL20—
TCF190—
FBXW80—
NCOA50—
ZBTB460—
ZMIZ10—
VMP11—
KALRN0—
HLA-B1—
IL2RA2—
MGAT51—

Clinical trials & evidence

Clinical trials

Clinical trials: 341.

Phase distribution (across all retrieved trials)

PhaseTrials
PHASE2118
PHASE1105
PHASE1/PHASE252
Not specified46
PHASE314
EARLY_PHASE14
PHASE41
PHASE2/PHASE31

Top trials by phase / activity

NCTPhaseStatusTitle
NCT07355699PHASE4RECRUITINGApplication of Orelabrutinib With or Without CD20 Monoclonal Antibody in Previously Untreated Marginal Zone Lymphoma
NCT00877214PHASE3ACTIVE_NOT_RECRUITINGSignificance of Duration of Maintenance Therapy With Rituximab in Non-Hodgkin Lymphomas
NCT04212013PHASE3ACTIVE_NOT_RECRUITINGA Study of Ibrutinib With Rituximab in People With Untreated Marginal Zone Lymphoma
NCT04680052PHASE3ACTIVE_NOT_RECRUITINGA Phase 3 Study to Assess Efficacy and Safety of Tafasitamab Plus Lenalidomide and Rituximab Compared to Placebo Plus Lenalidomide and Rituximab in Patients With Relapsed/Refractory (R/R) Follicular Lymphoma or Marginal Zone Lymphoma.
NCT05100862PHASE3RECRUITINGA Study of Zanubrutinib Plus Anti-CD20 Versus Lenalidomide Plus Rituximab in Participants With Relapsed/Refractory Follicular or Marginal Zone Lymphoma
NCT06006117PHASE3RECRUITINGMosunetuzumab-Lenalidomide Versus Investigator Choices in Patients With Relapsed or Refractory Marginal Zone Lymphoma
NCT07029217PHASE3RECRUITINGA Study of Reduced Dose Radiation Therapy for People With B-Cell Lymphomas
NCT00799461PHASE3COMPLETEDInternet-Based Program With or Without Telephone-Based Problem-Solving Training in Helping Long-Term Survivors of Hematopoietic Stem Cell Transplant Cope With Late Complications
NCT00801281PHASE3COMPLETEDFirst-line R-CVP vs R-CHOP Induction Immunochemotherapy for Indolent Lymphoma and R Maintenance.
NCT00991211PHASE3COMPLETEDBendamustine Plus Rituximab Versus CHOP Plus Rituximab
NCT02576275PHASE3WITHDRAWNA Study of Duvelisib in Combination With Rituximab and Bendamustine vs Placebo in Combination With Rituximab and Bendamustine in Subjects With Previously-Treated Indolent Non-Hodgkin Lymphoma (BRAVURA)
NCT02793583PHASE2/PHASE3TERMINATEDStudy to Assess the Efficacy and Safety of Ublituximab + Umbralisib With or Without Bendamustine and Umbralisib Alone in Patients With Previously Treated Non-Hodgkins Lymphoma
NCT03078855PHASE3COMPLETEDA Study to Evaluate the Effect of Vitamin D on PFS in Indolent Non-Hodgkin’s Lymphoma
NCT04745832PHASE3TERMINATEDPhase 3 Study of Zandelisib (ME-401) in Combination With Rituximab in Patients With iNHL - (COASTAL)
NCT04796922PHASE3WITHDRAWNTo Evaluate Efficacy and Safety of Parsaclisib Plus Either Rituximab or Obinutuzumab in R/R Follicular Lymphoma (FL) and Marginal Zone Lymphoma (MZL) (CITADEL-302)
NCT06125028PHASE3TERMINATED[68Ga]Ga-PentixaFor-PET Imaging for Staging of Marginal Zone Lymphoma
NCT02339922PHASE2ACTIVE_NOT_RECRUITINGIxazomib Citrate and Rituximab in Treating Patients With Indolent B-cell Non-Hodgkin Lymphoma
NCT02952508PHASE2ACTIVE_NOT_RECRUITINGStudy of Iopofosine I-131 (CLR 131) in Select B-Cell Malignancies (CLOVER-1) With Expansion in Waldenstrom
NCT03015896PHASE1/PHASE2ACTIVE_NOT_RECRUITINGNivolumab and Lenalidomide in Treating Patients With Relapsed or Refractory Non-Hodgkin or Hodgkin Lymphoma
NCT03147885PHASE1/PHASE2ACTIVE_NOT_RECRUITINGSelinexor Plus Combination Chemotherapy in Treating Patients With Advanced B Cell Non-Hodgkin Lymphoma
NCT03162536PHASE1/PHASE2ACTIVE_NOT_RECRUITINGA Study of Nemtabrutinib (MK-1026) in Participants With Relapsed or Refractory Hematologic Malignancies (ARQ 531-101/MK-1026-001)
NCT03277729PHASE1/PHASE2ACTIVE_NOT_RECRUITINGA Phase I/II Study to Evaluate the Safety of Cellular Immunotherapy Using Autologous T Cells Engineered to Express a CD20-Specific Chimeric Antigen Receptor for Patients With Relapsed or Refractory B Cell Non-Hodgkin Lymphomas
NCT03314974PHASE2RECRUITINGMyeloablative Allo HSCT With Related or Unrelated Donor for Heme Disorders
NCT03322865PHASE2ACTIVE_NOT_RECRUITINGObinutuzumab in Marginal Zone Lymphoma
NCT03474744PHASE2ACTIVE_NOT_RECRUITINGCopanlisib and Rituximab in Marginal Zone Lymphoma Patients
NCT03625037PHASE1/PHASE2ACTIVE_NOT_RECRUITINGFirst-in-Human (FIH) Trial in Patients With Relapsed, Progressive or Refractory B-Cell Lymphoma
NCT03697512PHASE2ACTIVE_NOT_RECRUITINGMALIBU Trial - Combination of Ibrutinib and Rituximab in Untreated Marginal Zone Lymphomas
NCT04186520PHASE1/PHASE2RECRUITINGCAR-20/19-T Cells in Patients With Relapsed Refractory B Cell Malignancies
NCT04195633PHASE2RECRUITINGDonor Stem Cell Transplant With Treosulfan, Fludarabine, and Total-Body Irradiation for the Treatment of Hematological Malignancies
NCT04416451PHASE2ACTIVE_NOT_RECRUITINGA Phase II Study Using Rituximab Plus Venetoclax in the Front Line Treatment of Marginal Zone Lymphoma
NCT04491370PHASE1/PHASE2RECRUITINGAutologous Stem Cell Transplant Followed by Polatuzumab Vedotin in Patients With B-cell Non-Hodgkin and Hodgkin Lymphoma
NCT04542824PHASE1/PHASE2ACTIVE_NOT_RECRUITINGTrial of the Safety and Efficacy of Epcoritamab in Japanese Subjects With Relapsed or Refractory (R/R) B-Cell Non-Hodgkin Lymphoma (R/R B-NHL)
NCT04646395PHASE2ACTIVE_NOT_RECRUITINGStudy of Acalabrutinib and Tafasitamab in MZL Patients
NCT04669171PHASE1/PHASE2RECRUITINGA Novel Vaccine (EO2463) as Monotherapy and in Combination, for Treatment of Patients With Indolent Non-Hodgkin Lymphoma
NCT04792502PHASE2RECRUITINGMosunetuzumab With Lenalidomide Augmentation as First-line Therapy for Follicular and Marginal Zone Lymphoma
NCT04883437PHASE2RECRUITINGAcalabrutinib and Obinutuzumab for the Treatment of Previously Untreated Follicular Lymphoma or Other Indolent Non-Hodgkin Lymphomas
NCT05006716PHASE1/PHASE2RECRUITINGA Dose-Escalation and Expansion Study of BGB-16673 in Participants With B-Cell Malignancies
NCT05025800PHASE1/PHASE2ACTIVE_NOT_RECRUITINGALX148, Rituximab and Lenalidomide for the Treatment of Indolent and Aggressive B-cell Non-Hodgkin Lymphoma
NCT05281809PHASE2RECRUITINGLocal Manufacture of CAR T-Cell Products for the Treatment of B-Cell Lymphoma and B-Acute Lymphoblastic Leukemia
NCT05294731PHASE1/PHASE2RECRUITINGTreatment of Chinese Participants With B-Cell Malignancies With BGB-16673, a Bruton Tyrosine Kinase-Targeted Protein-Degrader

Drugs tested across these trials (top 30)

MoleculeMax phaseTrials referencing
CYCLOPHOSPHAMIDE ANHYDROUS430
UBLITUXIMAB46
ZANUBRUTINIB46
BENDAMUSTINE45
UMBRALISIB45
OBINUTUZUMAB44
TAFASITAMAB44
YTTRIUM Y 90 IBRITUMOMAB TIUXETAN44
DUVELISIB43
IBRUTINIB43
MOSUNETUZUMAB43
ALEMTUZUMAB42
DOXORUBICIN42
NELARABINE41
OPRELVEKIN41
VINCRISTINE41
ORELABRUTINIB312
ZANDELISIB33
OBLIMERSEN SODIUM32
INDIUM IN 111 IBRITUMOMAB TIUXETAN31
PARSACLISIB31
BRYOSTATIN 121
EDODEKIN ALFA21
CHEMBL519312807
CHEMBL517032005
CHEMBL380534803
CHEMBL453868403
CHEMBL364796403
CHEMBL446620503

About this page

Generated deterministically by Sugi Atlas from primary biomedical databases via BioBTree. Every record on this page traces to a primary source.

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Sources 73

This page pulls from 73 datasets indexed by BioBTree:

alphafoldantibodybgeebgee_evidencebindingdbbiogrid_interactionbrendabrenda_kineticscellosauruschembl_activitychembl_assaychembl_documentchembl_moleculechembl_targetciviccivic_evidenceclingen_dosageclinical_trialsclinvarctd_gene_interactiondbsnpdepmapdiamond_similaritydoidefoensemblentrezesm2_similarityfantom5_genefantom5_promotergardgenccgenerifgogtopdbgtopdb_interactiongwasgwas_studyhgncintactinterprointogenmedgenmeshmimmondomondochildmondoparentncitorphanetpatent_compoundpdbpfampharmgkb_clinicalpharmgkb_genepharmgkb_guidelinepharmgkb_variantpubchempubchem_activitypubchem_assayreactomerefseqrhearnacentralsctidscxa_expressionscxa_gene_experimentsignorstring_interactiontranscriptufeatureumlsuniprot