# NOTCH3

Source: https://onco.cc/targets/notch3/  
OnCo record `notch3` (Target). Data CC BY-NC 4.0, attribute "Data from OnCo (onco.cc)"; commercial use needs a licence.

## TL;DR

NOTCH3 (Neurogenic locus notch homolog protein 3) is a protein that switches other genes on and off. The public catalogues list it as a drug target and a biomarker, and clinical evidence ties its variants to diagnosis, prognosis or drug response. Tied to Pancreatic ductal adenocarcinoma.

## Summary

Functions as a receptor for membrane-bound ligands Jagged1, Jagged2 and Delta1 to regulate cell-fate determination. Upon ligand activation through the released notch intracellular domain (NICD), it forms a transcriptional activator complex with RBPJ/RBPSUH and activates genes of the enhancer of split locus. Affects the implementation of differentiation, proliferation and apoptotic programs.

CIViC holds 2 clinical evidence items and 0 assertions across 1 variant, naming Tarextumab.

## Fields

- Kind: Target
- Last checked: 2026-09-23
- Also known as: notch receptor 3; Neurogenic locus notch homolog protein 3; CASIL; CADASIL
- Tags: cancer-genes-wave
- Symbol: NOTCH3
- Class: transcription
- Biology: Functions as a receptor for membrane-bound ligands Jagged1, Jagged2 and Delta1 to regulate cell-fate determination. Upon ligand activation through the released notch intracellular domain (NICD), it forms a transcriptional activator complex with RBPJ/RBPSUH and activates genes of the enhancer of split locus. Affects the implementation of differentiation, proliferation and apoptotic programs. Location: Cell membrane; Nucleus (UniProt). Locus 19p13.12 (HGNC).
- Where found: Pancreatic ductal adenocarcinoma: CIViC evidence names this disease

## Notes

- Written by scripts/fetch-cancer-genes.ts from CIViC, Open Targets, IntOGen, HGNC and UniProt; the function text is UniProt's, condensed and in UK spelling. Roles: CIViC lists 1 therapies; CIViC holds 2 clinical evidence items on its variants. Evidence tier "clinical-evidence" is the strongest of those signals.
- Prevalence not recorded: none of the sources gives a positivity rate.
- Diseases the sources name that have no OnCo cancer page yet, so they are not linked: Rectum Cancer.

## Sources

- HGNC HGNC:7883: https://www.genenames.org/data/gene-symbol-report/#!/hgnc_id/HGNC:7883
- UniProt Q9UM47: https://www.uniprot.org/uniprotkb/Q9UM47/entry
- NCBI Gene 4854: https://www.ncbi.nlm.nih.gov/gene/4854
- Ensembl ENSG00000074181: https://www.ensembl.org/Homo_sapiens/Gene/Summary?g=ENSG00000074181

## Connected records

- collections: [CIViC](https://onco.cc/collections/civic/)
- cancers: [Pancreatic ductal adenocarcinoma](https://onco.cc/cancers/pancreatic/)

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JSON: https://onco.cc/api/v1/entities/notch3.json