NTRK3

Chr 15

neurotrophic receptor tyrosine kinase 3

Also known as: GP145-TrkC, TRKC, gp145(trkC)

This gene encodes a member of the neurotrophic tyrosine receptor kinase (NTRK) family. This kinase is a membrane-bound receptor that, upon neurotrophin binding, phosphorylates itself and members of the MAPK pathway. Signalling through this kinase leads to cell differentiation and may play a role in the development of proprioceptive neurons that sense body position. Mutations in this gene have been associated with medulloblastomas, secretory breast carcinomas and other cancers. Several transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Jul 2011]

OMIMResearchGenerating clinical summary…
MultiplemechanismLOEUF 0.26
Clinical SummaryNTRK3
🧬
Gene-Disease Validity (ClinGen)
congenital heart disease · ADDisputed

Disputed — evidence questions this relationship

Population Constraint (gnomAD)
Highly constrained gene — heterozygous loss-of-function variants are very rare in the population (pLI 1.00). One damaged copy is likely sufficient to cause disease.
📋
ClinVar Variants
1 unique Pathogenic / Likely Pathogenic· 58 VUS of 138 total submissions
💊
Clinical Trials
11 active or recruiting trials — potential therapeutic options may be available

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

LoF intolerant — likely haploinsufficient
LoF Constraint?
0.26LOEUF
pLI 0.998
Z-score 5.18
OE 0.12 (0.060.26)
Highly constrained

Highly LoF-intolerant (top ~10% of genes)

Missense Constraint?
1.83Z-score
OE missense 0.77 (0.700.83)
375 obs / 489.2 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios?
LoF OE?0.12 (0.060.26)
00.351.4
Missense OE?0.77 (0.700.83)
00.61.4
Synonymous OE?1.20
01.21.6
LoF obs/exp: 5 / 40.7Missense obs/exp: 375 / 489.2Syn Z: -2.15

This gene — mechanism propensity

DN
0.5674th %ile
GOF
0.72top 25%
LOF
0.57top 25%

This gene has evidence for multiple mechanisms of pathogenicity (loss-of-function and gain-of-function). The Badonyi & Marsh model scores gain-of-function highest among its predictions, but genomic evidence (constraint, ClinVar variant spectrum, and literature) most strongly supports loss-of-function (haploinsufficiency). Different variants in this gene may act through different mechanisms — interpret in context of the specific variant.

LOFLOEUF 0.26
GOFprediction above median

Note: In-silico variant effect predictors (SIFT, PolyPhen, REVEL, CADD) may underestimate pathogenicity of missense variants in genes with GOF or DN mechanisms. Consider functional evidence and clinical context.

Predictions from Badonyi M, Marsh JA. PLoS ONE. 2024;19(8):e0307312. Mechanism ranking also informed by gnomAD constraint, ClinVar, and ClinGen data.

ClinVar Variant Classifications

138 submitted variants in ClinVar

Classification Summary

Pathogenic1
VUS58
Likely Benign22
Benign46
Conflicting1
1
Pathogenic
58
VUS
22
Likely Benign
46
Benign
1
Conflicting

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
0
0
1
0
1
Likely Pathogenic
0
0
0
0
0
VUS
2
56
0
0
58
Likely Benign
0
1
4
17
22
Benign
0
1
37
8
46
Conflicting
1
Total2584225128

LoF = frameshift, stop gained/lost, canonical splice · Counts from ClinVar esearch · Updated hourly

View in ClinVar →

33 pathogenic / likely-pathogenic (of 39) ClinVar copy-number / structural variants overlap NTRK3 — these span large chromosomal regions, not the gene specifically, and are excluded from the counts above. Explore in CNV tools →

Protein Context — Lollipop Plot

NTRK3 · protein map & ClinVar variants

Showing all ClinVar variants across the protein. Search a specific variant to highlight its position.

Clinical Trials

Active and recruiting trials from ClinicalTrials.gov

Solid Tumors Harboring NTRK Fusion

A Study to Test the Safety and Efficacy of the Drug Larotrectinib for the Treatment of Tumors With NTRK-fusion in Children

ACTIVE NOT RECRUITING
NCT02637687Phase PHASE1, PHASE2BayerStarted 2015-12-16
Larotrectinib (Vitrakvi, BAY2757556)
Oncogene-addicted Non Small Cell Lung CancerEGFR MutationALK Fusion-positive Solid or CNS Tumors

Integrated Genomics in Oncogene-driven NSCLC With Acquired Resistance

ENROLLING BY INVITATION
NCT07122882Chang Gung Memorial HospitalStarted 2025-09-01
Soft Tissue SarcomaAdvanced CancerMetastatic Cancer

RNASARC - Molecular Screening Program of Soft Tissue Sarcomas With Complex Genomic Profile to Detect NTRK1/2/3, ROS1 or ALK Gene Fusions.

ACTIVE NOT RECRUITING
NCT03375437Phase NACentre Leon BerardStarted 2018-02-15
Blood and tumor samples
Locally Advanced Solid TumorsMetastatic Solid Tumors

A Study of Repotrectinib (TPX-0005) in Patients With Advanced Solid Tumors Harboring ALK, ROS1, or NTRK1-3 Rearrangements

RECRUITING
NCT03093116Phase PHASE1, PHASE2Turning Point Therapeutics, Inc.Started 2017-03-07
Oral repotrectinib (TPX-0005)
Lymphoma, Non-HodgkinMultiple MyelomaAdvanced Solid Tumors

Canadian Profiling and Targeted Agent Utilization Trial (CAPTUR)

RECRUITING
NCT03297606Phase PHASE2Canadian Cancer Trials GroupStarted 2018-03-23
OlaparibDasatinibNivolumab plus Ipilimumab
Breast CancerCholangiocarcinomaColorectal Cancer

Basket Study of Entrectinib (RXDX-101) for the Treatment of Patients With Solid Tumors Harboring NTRK 1/2/3 (Trk A/B/C), ROS1, or ALK Gene Rearrangements (Fusions)

ACTIVE NOT RECRUITING
NCT02568267Phase PHASE2Hoffmann-La RocheStarted 2015-11-19
Entrectinib
Advanced LymphomaAdvanced Malignant Solid NeoplasmBladder Carcinoma

Targeted Therapy Directed by Genetic Testing in Treating Patients With Advanced Refractory Solid Tumors, Lymphomas, or Multiple Myeloma (The MATCH Screening Trial)

ACTIVE NOT RECRUITING
NCT02465060Phase PHASE2National Cancer Institute (NCI)Started 2015-08-17
AdavosertibAfatinibAfatinib Dimaleate
Solid TumorsCNS Tumors

Study Of Entrectinib (Rxdx-101) in Children and Adolescents With Locally Advanced Or Metastatic Solid Or Primary CNS Tumors And/Or Who Have No Satisfactory Treatment Options

ACTIVE NOT RECRUITING
NCT02650401Phase PHASE1, PHASE2Hoffmann-La RocheStarted 2016-05-03
Entrectinib
Advanced Malignant Solid NeoplasmAnn Arbor Stage III Non-Hodgkin LymphomaAnn Arbor Stage IV Non-Hodgkin Lymphoma

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)

ACTIVE NOT RECRUITING
NCT03155620Phase PHASE2National Cancer Institute (NCI)Started 2017-07-31
Biopsy ProcedureBiospecimen CollectionBone Marrow Aspiration and Biopsy
Microwave AblationContrast-enhanced UltrasoundGenetic and Molecular Diagnostics

Efficacy and Prognosis of Microwave Ablation Treatment for Papillary Thyroid Microcarcinoma Assessed by Contrast-Enhanced Ultrasound Combined With Genetic and Molecular Diagnostics: A Prospective Observational Study

RECRUITING
NCT07054229Sun Yat-Sen Memorial Hospital of Sun Yat-Sen UniversityStarted 2024-01-01
Contrast enhanced Ultrasound88-gene panel for thyroid cancer
Differentiated Thyroid CarcinomaMedullary Thyroid Carcinoma

Spanish Study for Molecular Characterization of Thyroid Carcinoma

ACTIVE NOT RECRUITING
NCT04970134Grupo Español de Tratamiento de Tumores de Cabeza y CuelloStarted 2021-06-14
Immunohistochemistry (IHC)Fluorescence In-Situ Hybridization (FISH)Oncomine Focus Assay Platform with 52 genes