Gene Information

BRIP1

BRCA1 interacting DNA helicase 1 [Source:HGNC Symbol;Acc:HGNC:20473]

protein_codingChromosome 17ENSG00000136492

Overview

BRIP1 variants are associated with 2 diseases including Fanconi anemia and hereditary breast ovarian cancer syndrome, with 9 documented clinical phenotypes.


Associated Diseases

The BRIP1 gene is associated with 2 medical conditions, including:

Fanconi anemia

MONDO:0019391

hereditary breast ovarian cancer syndrome

MONDO:0003582


Clinical Phenotypes

Research has identified 9 phenotypic features associated with BRIP1 variants:

Autosomal recessive inheritance

HP:0000007

Intrauterine growth retardation

HP:0001511

Chromosomal breakage induced by crosslinking agents

HP:0003221

Bone marrow hypocellularity

HP:0005528

Microphthalmia

HP:0000568

Global developmental delay

HP:0001263

Postnatal growth retardation

HP:0008897

Short thumb

HP:0009778

Multiple cafe-au-lait spots

HP:0007565


ClinVar Variants

ClinVar contains 10 variants associated with BRIP1. Below are some notable variants:

NM_032043.3(BRIP1):c.2485del (p.Gly830fs)

Pathogenic

VCV004294276

Review: criteria provided, single submitter

NM_032043.3(BRIP1):c.1511_1515dup (p.Pro506fs)

Pathogenic

VCV004294256

Review: criteria provided, single submitter

NM_032043.3(BRIP1):c.12del (p.Met4fs)

Pathogenic

VCV004294253

Review: criteria provided, single submitter

NM_032043.3(BRIP1):c.2257+2_2257+8del

Likely pathogenic

VCV004294231

Review: criteria provided, single submitter

NM_032043.3(BRIP1):c.2492_2492+3del

Likely pathogenic

VCV004294230

Review: criteria provided, single submitter


Recent Publications

There are 387 publications about BRIP1 indexed in PubMed. Below are some recent papers:

Salpingectomy in individuals at high risk for tubo-ovarian cancer - consensus and precaution.

Wilke RN, Pennington KP, Gootzen TA

PMID: 41183726

2025 Nov 1

Am J Obstet Gynecol

Recontact and follow-up for individuals with germline pathogenic variants in hereditary breast and ovarian cancer susceptibility genes: a UK Cancer Genetics Group consensus meeting.

Christopher J, Edgerley K, McIldowie B

PMID: 41159931

2025 Oct 1

J Med Genet

Frequency and clinical features of germline pathogenic variants in sarcoma: a case-control study.

Rodriguez-Hernandez A, Horiguchi M, Horton C

PMID: 41092038

2025 Oct 13

J Natl Cancer Inst

Integrated computational and experimental approach to identify Nrf2-regulated molecular targets in cerebral ischemia.

Lewczuk A, Boratyńska-Jasińska A, Charzewski Ł

PMID: 41091415

2025 Oct 15

Pharmacol Rep

Management of individuals with heterozygous germline pathogenic variants in RAD51C, RAD51D, and BRIP1: A clinical practice resource of the American College of Medical Genetics and Genomics (ACMG).

Ngeow J, Chiang J, Astiazaran-Symonds E

PMID: 41070818

2025 Nov

Genet Med

View all 387 publications on PubMed →

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Trusted Data Sources

EnsemblMonarch InitiativeClinVarGeneReviewsPubMed

Last updated: 11/20/2025

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