SETD9

Summary

SETD9 (SET domain containing 9, HGNC:28508) is a protein-coding gene on chromosome 5q11.2, encoding SET domain-containing protein 9 (Q8NE22).

Predicted to enable lysine N-methyltransferase activity. Predicted to be involved in regulation of signal transduction by p53 class mediator. Located in mitochondrion.

Source: NCBI Gene 133383 — RefSeq curated summary.

At a glance

• GWAS associations: 8
• Clinical variants (ClinVar): 44 total
• MANE Select transcript: NM_153706

Identifiers

Gene identifiers

Field	Value
HGNC ID	HGNC:28508
Approved symbol	SETD9
Name	SET domain containing 9
Location	5q11.2
Locus type	gene with protein product
Status	Approved
Aliases	MGC33648
Ensembl gene	ENSG00000155542
Ensembl biotype	protein_coding
Entrez	133383

Gene structure

Transcript identifiers

Ensembl transcripts: 17 — 9 protein_coding, 6 protein_coding_CDS_not_defined, 1 nonsense_mediated_decay, 1 TEC

ENST00000285947, ENST00000418299, ENST00000423328, ENST00000463805, ENST00000472636, ENST00000475908, ENST00000477359, ENST00000480414, ENST00000498322, ENST00000624733, ENST00000628593, ENST00000886482, ENST00000886483, ENST00000918989, ENST00000918990, ENST00000918991, ENST00000941154

RefSeq mRNA: 5 — MANE Select: NM_153706 NM_001171990, NM_001323018, NM_001323019, NM_001323022, NM_153706

CCDS: CCDS3972, CCDS93713

Canonical transcript exons

ENST00000285947 — 6 exons

Exon	Start	End
ENSE00001021435	56911169	56911536
ENSE00001228259	56916815	56917348
ENSE00001228265	56909596	56909743
ENSE00003533911	56914861	56914966
ENSE00003603017	56913874	56913989
ENSE00003652345	56913011	56913134

Expression profiles

Bgee: expression breadth ubiquitous, 219 present calls, max score 96.26.

FANTOM5 (CAGE): breadth ubiquitous, TPM avg 5.0054 / max 112.9415, expressed in 1694 samples.

FANTOM5 promoters (5 alternative TSS)

Promoter ID	TPM avg	Samples expressed
56518	3.3159	1474
56519	0.9068	502
56517	0.7118	363
56515	0.0456	7
56516	0.0253	5

Top tissues by expression

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

Tissue	Anatomy ID	Expression score	Quality
sperm	CL:0000019	96.26	gold quality
primordial germ cell in gonad	CL:0000670 ∩ UBERON:0000991	93.58	gold quality
male germ line stem cell (sensu Vertebrata) in testis	CL:0000089 ∩ UBERON:0000473	93.15	gold quality
left testis	UBERON:0004533	93.01	gold quality
right testis	UBERON:0004534	92.84	gold quality
testis	UBERON:0000473	91.62	gold quality
calcaneal tendon	UBERON:0003701	84.88	gold quality
C1 segment of cervical spinal cord	UBERON:0006469	84.29	gold quality
palpebral conjunctiva	UBERON:0001812	83.79	gold quality
monocyte	CL:0000576	83.35	gold quality
mucosa of transverse colon	UBERON:0004991	83.06	gold quality
leukocyte	CL:0000738	82.82	gold quality
heart left ventricle	UBERON:0002084	82.26	gold quality
right lobe of liver	UBERON:0001114	82.11	gold quality
adult organism	UBERON:0007023	81.94	gold quality
ileal mucosa	UBERON:0000331	81.89	gold quality
cardiac ventricle	UBERON:0002082	81.72	gold quality
jejunal mucosa	UBERON:0000399	81.34	gold quality
spinal cord	UBERON:0002240	81.33	gold quality
rectum	UBERON:0001052	80.81	gold quality
transverse colon	UBERON:0001157	80.77	gold quality
muscle of leg	UBERON:0001383	80.63	gold quality
gastrocnemius	UBERON:0001388	80.55	gold quality
hindlimb stylopod muscle	UBERON:0004252	80.54	gold quality
islet of Langerhans	UBERON:0000006	80.22	gold quality
heart	UBERON:0000948	80.14	gold quality
body of pancreas	UBERON:0001150	80.05	gold quality
right atrium auricular region	UBERON:0006631	79.51	gold quality
epithelial cell of pancreas	CL:0000083	79.35	gold quality
pancreas	UBERON:0001264	79.32	gold quality

Single-cell (SCXA)

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

Experiment	Marker?	Max mean expression
E-ANND-3	yes	3.77

Regulation

Is transcription factor: no

miRNA regulators (miRDB)

25 targeting SETD9, top 30 by miRDB confidence (max_score; target_count = how many genes the miRNA targets in total — lower means more specific):

miRNA	Max score	Avg score	miRNA target_count
HSA-MIR-371B-5P	99.99	75.34	4759
HSA-MIR-4803	99.98	71.99	3117
HSA-MIR-373-5P	99.98	75.36	4753
HSA-MIR-616-5P	99.98	75.58	4775
HSA-MIR-3065-5P	99.97	71.56	3281
HSA-MIR-3686	99.90	70.53	2432
HSA-MIR-8062	99.88	68.43	995
HSA-MIR-3919	99.87	69.45	2489
HSA-MIR-7154-5P	99.69	70.52	1900
HSA-MIR-561-3P	99.64	70.90	3647
HSA-MIR-7159-5P	99.53	72.12	2472
HSA-MIR-203A-3P	99.49	70.56	2806
HSA-MIR-208A-5P	99.42	70.83	1913
HSA-MIR-208B-5P	99.42	70.83	1952
HSA-MIR-889-3P	99.40	69.76	2103
HSA-MIR-148A-5P	99.30	68.27	1141
HSA-MIR-329-5P	99.27	68.11	1597
HSA-MIR-223-5P	99.24	68.82	1206
HSA-MIR-8077	99.17	66.67	862
HSA-MIR-4650-3P	99.01	68.39	1062
HSA-MIR-4778-5P	97.96	68.06	1634
HSA-MIR-4678	97.59	68.31	902
HSA-MIR-3184-3P	96.96	66.91	845
HSA-MIR-331-5P	96.59	67.94	705
HSA-MIR-6800-5P	94.59	64.80	525

Literature-anchored findings (GeneRIF, showing 1)

• SNP variants at the MAP3K1/SETD9 gene boundary associate with somatic PIK3CA variants in breast cancers. (PMID:28029147)

Cross-species orthologs

1 orthologs

Organism	Symbol	Gene ID
danio_rerio	setd9	ENSDARG00000074456

Protein

Protein identifiers

SET domain-containing protein 9 — Q8NE22 (reviewed: Q8NE22)

All UniProt accessions (3): Q8NE22, C9J935, F8WBJ9

UniProt curated annotations — full annotation on UniProt →

Similarity. Belongs to the class V-like SAM-binding methyltransferase superfamily.

Isoforms (2)

UniProt ID	Names	Canonical?
Q8NE22-1	1	yes
Q8NE22-2	2

RefSeq proteins (5): NP_001165461, NP_001309947, NP_001309948, NP_001309951, NP_714917* (*=MANE)

Domains & families (InterPro)

ID	Name	Type
IPR001214	SET_dom	Domain
IPR040415	SETD9	Family
IPR046341	SET_dom_sf	Homologous_superfamily

UniProt features (8 total): splice variant 2, sequence variant 2, chain 1, domain 1, binding site 1, sequence conflict 1

Structure

Experimental structures (PDB)

0 structures.

Predicted structure (AlphaFold)

Model	pLDDT	Fraction very-high
AF-Q8NE22-F1	87.23	0.63

Functional residue map

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

Ligand- & substrate-binding residues (1): 294

Function

Pathways and Gene Ontology

Reactome pathways

6 pathways

ID	Pathway
R-HSA-6804760	Regulation of TP53 Activity through Methylation
R-HSA-212436	Generic Transcription Pathway
R-HSA-3700989	Transcriptional Regulation by TP53
R-HSA-5633007	Regulation of TP53 Activity
R-HSA-73857	RNA Polymerase II Transcription
R-HSA-74160	Gene expression (Transcription)

MSigDB gene sets: 93 (showing top): RODRIGUES_THYROID_CARCINOMA_POORLY_DIFFERENTIATED_UP, GOBP_SIGNAL_TRANSDUCTION_BY_P53_CLASS_MEDIATOR, GOBP_REGULATION_OF_SIGNAL_TRANSDUCTION_BY_P53_CLASS_MEDIATOR, JAATINEN_HEMATOPOIETIC_STEM_CELL_UP, GOBP_METHYLATION, chr5q11, GOMF_N_METHYLTRANSFERASE_ACTIVITY, GOMF_S_ADENOSYLMETHIONINE_DEPENDENT_METHYLTRANSFERASE_ACTIVITY, GOMF_TRANSFERASE_ACTIVITY_TRANSFERRING_ONE_CARBON_GROUPS, GOMF_LYSINE_N_METHYLTRANSFERASE_ACTIVITY, SENGUPTA_NASOPHARYNGEAL_CARCINOMA_WITH_LMP1_UP, MARTENS_TRETINOIN_RESPONSE_DN, REACTOME_TRANSCRIPTIONAL_REGULATION_BY_TP53, REACTOME_REGULATION_OF_TP53_ACTIVITY, REACTOME_REGULATION_OF_TP53_ACTIVITY_THROUGH_METHYLATION

GO Biological Process (2): methylation (GO:0032259), regulation of signal transduction by p53 class mediator (GO:1901796)

GO Molecular Function (4): lysine N-methyltransferase activity (GO:0016278), protein binding (GO:0005515), methyltransferase activity (GO:0008168), transferase activity (GO:0016740)

GO Cellular Component (2): nucleoplasm (GO:0005654), mitochondrion (GO:0005739)

Reactome top-level categories

Rollup of top-5 pathways:

Category	Pathways
Regulation of TP53 Activity	1
RNA Polymerase II Transcription	1
Generic Transcription Pathway	1
Transcriptional Regulation by TP53	1
Gene expression (Transcription)	1

GO top-level categories

Rollup of top GO terms by namespace:

Category	Terms
metabolic process	1
signal transduction by p53 class mediator	1
regulation of intracellular signal transduction	1
N-methyltransferase activity	1
S-adenosylmethionine-dependent methyltransferase activity	1
binding	1
transferase activity, transferring one-carbon groups	1
catalytic activity	1
nuclear lumen	1
cellular anatomical structure	1
cytoplasm	1
intracellular membrane-bounded organelle	1

Protein interactions and networks

STRING

298 interactions, top by confidence (×1000):

Protein A	Protein B	Partner UniProt	Score
SETD9	MIER3	Q7Z3K6	604
SETD9	SETD7	Q8WTS6	461
SETD9	TOX3	O15405	447
SETD9	SETD3	Q86TU7	433
SETD9	SMIM19	Q96E16	420
SETD9	MAP3K1	Q13233	419
SETD9	SETD4	Q9NVD3	411
SETD9	TMEM53	Q6P2H8	368
SETD9	JMJD8	Q96S16	366
SETD9	LSP1	P33241	358
SETD9	TMEM267	Q0VDI3	348
SETD9	TCP10L	Q8TDR4	336
SETD9	SETD5	Q9C0A6	332
SETD9	NSD1	Q96L73	326
SETD9	ASB17	Q8WXJ9	324

IntAct

11 interactions, top by confidence:

A	B	Type	Score
SETD9	USO1	psi-mi:“MI:0915”(physical association)	0.500
SETD9	USO1	psi-mi:“MI:0914”(association)	0.500
SETD9	USO1	psi-mi:“MI:0915”(physical association)	0.400
SETD9	PLCG2	psi-mi:“MI:0915”(physical association)	0.370
SETD9	CRK	psi-mi:“MI:0915”(physical association)	0.370
ATAD3A	TMEM223	psi-mi:“MI:0914”(association)	0.350
S100A2	PLEKHG3	psi-mi:“MI:0914”(association)	0.350
SLC30A7	ESYT2	psi-mi:“MI:0914”(association)	0.350
CEBPA	HAX1	psi-mi:“MI:0914”(association)	0.350
STAT3	HAX1	psi-mi:“MI:0914”(association)	0.350

BioGRID (10): USO1 (Affinity Capture-MS), SETD9 (Two-hybrid), SETD9 (Two-hybrid), USO1 (Affinity Capture-MS), SETD9 (Affinity Capture-MS), USO1 (Affinity Capture-MS), HSPD1 (Affinity Capture-MS), SETD9 (Affinity Capture-MS), SETD9 (Affinity Capture-RNA), SETD9 (Affinity Capture-RNA)

ESM2 similar proteins: A2AQW0, A4IIA7, A8MYZ0, A9JRL3, E1C3P4, F1RET2, F4I933, O35099, P29995, Q0P410, Q149N8, Q1RMU2, Q28DH9, Q29RL0, Q3B7T1, Q3TYX3, Q3UD82, Q5I0G3, Q5NDE6, Q5NDE7, Q5NDE8, Q5PP37, Q5R9R1, Q5RBW0, Q5RED8, Q66H62, Q66J91, Q66JT5, Q6AXS8, Q6DD21, Q6DEY8, Q6GPQ4, Q6GQV7, Q6NXY1, Q6ZN16, Q7TPQ3, Q80TQ2, Q8C0W1, Q8N3A8, Q8NE22

Diamond homologs: Q5RBW0, Q8NE22

SIGNOR signaling

0 interactions.

Disease & clinical

Clinical variants and AI predictions

ClinVar

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

Classification	Count (floor)
Pathogenic	0
Likely pathogenic	0
Uncertain significance	33
Likely benign	3
Benign	0

Top pathogenic / likely-pathogenic (0)

SpliceAI

1307 predictions. Top by Δscore:

Variant	Effect	Δscore
5:56913007:ATAG:A	acceptor_loss	1.0000
5:56913008:TAGGT:T	acceptor_loss	1.0000
5:56913132:CAGGT:C	donor_loss	1.0000
5:56913134:GGTA:G	donor_loss	1.0000
5:56913135:G:GG	donor_gain	1.0000
5:56913136:T:G	donor_loss	1.0000
5:56914859:A:AG	acceptor_gain	1.0000
5:56914860:G:GG	acceptor_gain	1.0000
5:56914860:GACA:G	acceptor_gain	1.0000
5:56923581:CAAAG:C	acceptor_gain	1.0000
5:56923582:AAAG:A	acceptor_gain	1.0000
5:56923583:AAG:A	acceptor_gain	1.0000
5:56923584:AG:A	acceptor_gain	1.0000
5:56923586:C:CC	acceptor_gain	1.0000
5:56923586:CTA:C	acceptor_loss	1.0000
5:56923587:T:C	acceptor_loss	1.0000
5:56923685:TTTTA:T	donor_loss	1.0000
5:56923686:TTTAC:T	donor_loss	1.0000
5:56923687:TTAC:T	donor_loss	1.0000
5:56923688:TACCT:T	donor_loss	1.0000
5:56923689:ACC:A	donor_loss	1.0000
5:56923690:CCT:C	donor_loss	1.0000
5:56923832:CC:C	acceptor_gain	1.0000
5:56923833:CC:C	acceptor_gain	1.0000
5:56923913:A:AC	donor_gain	1.0000
5:56923914:C:CC	donor_gain	1.0000
5:56923914:CGTAA:C	donor_gain	1.0000
5:56911242:A:T	donor_gain	0.9900
5:56911388:GCAAA:G	donor_gain	0.9900
5:56911498:G:GT	donor_gain	0.9900

AlphaMissense

1941 scored. Top likely-pathogenic:

Variant	Protein change	am_pathogenicity
5:56913972:T:A	V230D	0.995
5:56914876:T:A	V241D	0.994
5:56913926:T:A	W215R	0.991
5:56913926:T:C	W215R	0.991
5:56916829:T:A	V276D	0.990
5:56916871:T:A	L290H	0.990
5:56911489:T:A	V140D	0.989
5:56911530:T:G	Y154D	0.989
5:56911434:T:C	F122L	0.988
5:56911436:C:A	F122L	0.988
5:56911436:C:G	F122L	0.988
5:56913011:G:A	G156D	0.988
5:56913889:G:C	R202S	0.988
5:56913889:G:T	R202S	0.988
5:56914874:T:A	N240K	0.988
5:56914874:T:G	N240K	0.988
5:56913100:G:T	G186W	0.987
5:56913979:T:A	N232K	0.986
5:56913979:T:G	N232K	0.986
5:56911483:T:A	V138D	0.985
5:56911536:G:C	G156R	0.984
5:56913101:G:A	G186E	0.984
5:56913963:G:A	G227E	0.984
5:56911455:A:C	S129R	0.983
5:56911457:C:A	S129R	0.983
5:56911457:C:G	S129R	0.983
5:56916838:T:A	V279D	0.983
5:56916871:T:C	L290P	0.983
5:56916835:T:C	L278P	0.982
5:56909712:T:A	W23R	0.981

dbSNP variants (sampled 300 via entrez): RS1000029254 (5:56924297 G>A,C), RS1000045817 (5:56907534 T>G), RS1000505377 (5:56907566 C>T), RS1000682167 (5:56913405 A>G), RS1000704966 (5:56927202 G>A), RS1000780506 (5:56920447 AG>A), RS1000885926 (5:56928026 T>C), RS1001515288 (5:56921419 C>T), RS1001544223 (5:56916133 C>T), RS1001637710 (5:56908857 T>A,G), RS1001653155 (5:56916408 A>G), RS1001668899 (5:56909225 G>C,T), RS1001679006 (5:56917314 G>C), RS1001724623 (5:56910169 C>A), RS1001843471 (5:56917463 A>G)

Disease associations

OMIM: gene `` | disease phenotypes:

GenCC curated gene-disease

Mondo (1): ependymoma (MONDO:0016698)

Orphanet (1): Ependymoma (Orphanet:251636)

HPO phenotypes

0 total (0 of 0 shown, HPO-id order):

GWAS associations

8 associations (top):

Study	Trait	p-value
GCST000809_5	Triglycerides	3.000000e-06
GCST005196_83	Coronary artery disease	2.000000e-08
GCST005956_55	Waist-to-hip ratio adjusted for BMI	9.000000e-06
GCST005957_9	Waist-to-hip ratio adjusted for BMI (age <50)	2.000000e-07
GCST005962_24	Waist-to-hip ratio adjusted for BMI x sex x age interaction (4df test)	3.000000e-08
GCST010701_111	Cortical surface area (MOSTest)	8.000000e-12
GCST010702_47	Subcortical volume (MOSTest)	6.000000e-10
GCST010703_329	Brain morphology (MOSTest)	2.000000e-10

EFO canonical traits (5, from GWAS)

EFO ID	Trait name
EFO:0004530	triglyceride measurement
EFO:0007788	BMI-adjusted waist-hip ratio
EFO:0008007	age at assessment
EFO:0008343	sex interaction measurement
EFO:0004346	neuroimaging measurement

MeSH disease descriptors (1)

Descriptor	Name	Tree numbers
D004806	Ependymoma	C04.557.465.625.600.380.290; C04.557.470.670.380.290; C04.557.580.625.600.380.290

Drugs & pharmacology

Drug and pharmacology data

Is drug target: no

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

CTD chemical–gene interactions

25 total (human), top 25 by PubMed support.

Chemical	Actions (top 5)	PubMed papers
Valproic Acid	affects cotreatment, increases expression, affects expression	4
Cyclosporine	decreases expression, increases expression, increases methylation	3
dicrotophos	decreases expression	1
triphenyl phosphate	affects expression	1
bisphenol A	affects cotreatment, increases methylation	1
decabromobiphenyl ether	affects expression	1
beta-lapachone	decreases expression	1
di-n-butylphosphoric acid	affects expression	1
4-(5-benzo(1,3)dioxol-5-yl-4-pyridin-2-yl-1H-imidazol-2-yl)benzamide	affects cotreatment, increases expression	1
ICG 001	decreases expression	1
dorsomorphin	affects cotreatment, increases expression	1
4-(4-((5-(4,5-dimethyl-2-nitrophenyl)-2-furanyl)methylene)-4,5-dihydro-3-methyl-5-oxo-1H-pyrazol-1-yl)benzoic acid	decreases expression	1
Fulvestrant	affects cotreatment, increases methylation	1
Vorinostat	increases expression	1
Benzo(a)pyrene	decreases methylation	1
Tetrachlorodibenzodioxin	decreases expression	1
Thimerosal	increases expression	1
Tobacco Smoke Pollution	increases expression	1
Tretinoin	decreases expression	1
Tunicamycin	decreases expression	1
Aflatoxin B1	increases methylation	1
Okadaic Acid	decreases expression	1
Copper Sulfate	decreases expression	1
Acrylamide	increases expression	1
S-Nitrosoglutathione	decreases expression	1

Cellosaurus cell lines

2 cell lines: 2 cancer cell line

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

Cellosaurus	Name	Category	Sex
CVCL_TK71	HAP1 SETD9 (-) 1	Cancer cell line	Male
CVCL_TK72	HAP1 SETD9 (-) 2	Cancer cell line	Male

Clinical trials (associated diseases)

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

Trial	Phase	Status	Title
NCT00517959	PHASE3	UNKNOWN	SCRT Versus Conventional RT in Children and Young Adults With Low Grade and Benign Brain Tumors
NCT01096368	PHASE3	COMPLETED	Maintenance Chemotherapy or Observation Following Induction Chemotherapy and Radiation Therapy in Treating Patients With Newly Diagnosed Ependymoma
NCT00003479	PHASE2	TERMINATED	Antineoplaston Therapy in Treating Patients With Ependymoma
NCT00520936	PHASE2	COMPLETED	A Study of Pemetrexed in Children With Recurrent Cancer
NCT00840047	PHASE2	ACTIVE_NOT_RECRUITING	Methionine PET/CT Studies In Patients With Cancer
NCT01088035	PHASE2	TERMINATED	Carboplatin as a Radiosensitizer in Treating Childhood Ependymoma
NCT01247922	PHASE2	TERMINATED	Single-agent Erlotinib in Patients Previously Treated With Oral Etoposide in Protocol OSI-774-205
NCT01288235	PHASE2	COMPLETED	Proton Radiotherapy for Pediatric Brain Tumors Requiring Partial Brain Irradiation
NCT01295944	PHASE2	COMPLETED	Carboplatin and Bevacizumab for Recurrent Ependymoma
NCT01356290	PHASE2	RECRUITING	Antiangiogenic Therapy for Children With Recurrent Medulloblastoma, Ependymoma, ATRT and Rare CNS Tumors
NCT01836549	PHASE2	TERMINATED	Imetelstat Sodium in Treating Younger Patients With Recurrent or Refractory Brain Tumors
NCT02125786	PHASE2	ACTIVE_NOT_RECRUITING	A Trial of Surgery and Fractionated Re-Irradiation for Recurrent Ependymoma
NCT02689336	PHASE2	WITHDRAWN	Erlotinib in Combination With Temozolomide in Treating Relapsed/Recurrent/Refractory Pediatric Solid Tumors
NCT03095248	PHASE2	TERMINATED	Trial of Selumetinib in Patients With Neurofibromatosis Type II Related Tumors
NCT03155620	PHASE2	ACTIVE_NOT_RECRUITING	Targeted Therapy Directed by Genetic Testing in Treating Pediatric Patients With Relapsed or Refractory Advanced Solid Tumors, Non-Hodgkin Lymphomas, or Histiocytic Disorders (The Pediatric MATCH Screening Trial)
NCT03173950	PHASE2	COMPLETED	Immune Checkpoint Inhibitor Nivolumab in People With Recurrent Select Rare CNS Cancers
NCT03194906	PHASE2	COMPLETED	Memantine for Prevention of Cognitive Late Effects in Pediatric Patients Receiving Cranial Radiation Therapy for Localized Brain Tumors
NCT03210714	PHASE2	ACTIVE_NOT_RECRUITING	Erdafitinib in Treating Patients With Relapsed or Refractory Advanced Solid Tumors, Non-Hodgkin Lymphoma, or Histiocytic Disorders With FGFR Mutations (A Pediatric MATCH Treatment Trial)
NCT03213652	PHASE2	ACTIVE_NOT_RECRUITING	Ensartinib in Treating Patients With Relapsed or Refractory Advanced Solid Tumors, Non-Hodgkin Lymphoma, or Histiocytic Disorders With ALK or ROS1 Genomic Alterations (A Pediatric MATCH Treatment Trial)
NCT03213665	PHASE2	COMPLETED	Tazemetostat in Treating Patients With Relapsed or Refractory Advanced Solid Tumors, Non-Hodgkin Lymphoma, or Histiocytic Disorders With EZH2, SMARCB1, or SMARCA4 Gene Mutations (A Pediatric MATCH Treatment Trial)
NCT03213678	PHASE2	COMPLETED	Samotolisib in Treating Patients With Relapsed or Refractory Advanced Solid Tumors, Non-Hodgkin Lymphoma, or Histiocytic Disorders With TSC or PI3K/MTOR Mutations (A Pediatric MATCH Treatment Trial)
NCT03213704	PHASE2	ACTIVE_NOT_RECRUITING	Larotrectinib in Treating Patients With Relapsed or Refractory Advanced Solid Tumors, Non-Hodgkin Lymphoma, or Histiocytic Disorders With NTRK Fusions (A Pediatric MATCH Treatment Trial)
NCT03220035	PHASE2	COMPLETED	Vemurafenib in Treating Patients With Relapsed or Refractory Advanced Solid Tumors, Non-Hodgkin Lymphoma, or Histiocytic Disorders With BRAF V600 Mutations (A Pediatric MATCH Treatment Trial)
NCT03233204	PHASE2	COMPLETED	Olaparib in Treating Patients With Relapsed or Refractory Advanced Solid Tumors, Non-Hodgkin Lymphoma, or Histiocytic Disorders With Defects in DNA Damage Repair Genes (A Pediatric MATCH Treatment Trial)
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NCT03698994	PHASE2	ACTIVE_NOT_RECRUITING	Ulixertinib in Treating Patients With Advanced Solid Tumors, Non-Hodgkin Lymphoma, or Histiocytic Disorders With MAPK Pathway Mutations (A Pediatric MATCH Treatment Trial)
NCT03727841	PHASE2	TERMINATED	Marizomib for Recurrent Low-Grade and Anaplastic Supratentorial, Infratentorial, and Spinal Cord Ependymoma
NCT04049669	PHASE2	ACTIVE_NOT_RECRUITING	Pediatric Trial of Indoximod With Chemotherapy and Radiation for Relapsed Brain Tumors or Newly Diagnosed DIPG
NCT04195555	PHASE2	ACTIVE_NOT_RECRUITING	Ivosidenib in Treating Patients With Advanced Solid Tumors, Lymphoma, or Histiocytic Disorders With IDH1 Mutations (A Pediatric MATCH Treatment Trial)
NCT04284774	PHASE2	ACTIVE_NOT_RECRUITING	Tipifarnib for the Treatment of Advanced Solid Tumors, Lymphoma, or Histiocytic Disorders With HRAS Gene Alterations, a Pediatric MATCH Treatment Trial
NCT04320888	PHASE2	ACTIVE_NOT_RECRUITING	Selpercatinib for the Treatment of Advanced Solid Tumors, Lymphomas, or Histiocytic Disorders With Activating RET Gene Alterations, a Pediatric MATCH Treatment Trial
NCT04374305	PHASE2	RECRUITING	Innovative Trial for Understanding the Impact of Targeted Therapies in NF2-Related Schwannomatosis (INTUITT-NF2)
NCT04743661	PHASE2	ACTIVE_NOT_RECRUITING	131I-Omburtamab, in Recurrent Medulloblastoma and Ependymoma
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NCT07424092	PHASE2	RECRUITING	Intratumoral DNX-2401 for High Grade Pediatric Brain Tumors
NCT00634231	PHASE1	COMPLETED	A Phase I Study of AdV-tk + Prodrug Therapy in Combination With Radiation Therapy for Pediatric Brain Tumors
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NCT01498783	PHASE1	COMPLETED	Phase I Study of 5-Fluorouracil in Children and Young Adults With Recurrent Ependymoma