BRAT1

Chr 7AR

BRCA1 associated ATM activator 1

Also known as: BAAT1, C7orf27, NEDCAS, RMFSL

This protein interacts with BRCA1 and ATM proteins in DNA damage response pathways and is involved in cell cycle checkpoint signaling. Biallelic mutations cause autosomal recessive neurodevelopmental disorders ranging from lethal neonatal rigidity with multifocal seizures to milder presentations with cerebellar atrophy and variable seizures. The pathogenic mechanism involves loss of function, as the gene shows tolerance to heterozygous loss-of-function variants but requires biallelic mutations for disease manifestation.

GeneReviewsOMIMResearchSummary from RefSeq, OMIM, UniProt
LOFmechanismARLOEUF 0.992 OMIM phenotypes
Clinical SummaryBRAT1
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Gene-Disease Validity (ClinGen)
neonatal-onset encephalopathy with rigidity and seizures · ARDefinitive

Definitive — sufficient evidence for diagnostic panels

2 total gene-disease associations curated

Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
📖
GeneReview available — BRAT1
Authoritative clinical overview · Recommended first read
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Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
0.99LOEUF
pLI 0.000
Z-score 1.58
OE 0.71 (0.510.99)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
-0.59Z-score
OE missense 1.08 (1.001.16)
532 obs / 494.9 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios
LoF OE0.71 (0.510.99)
00.351.4
Missense OE1.08 (1.001.16)
00.61.4
Synonymous OE1.14
01.21.6
LoF obs/exp: 24 / 33.9Missense obs/exp: 532 / 494.9Syn Z: -1.67

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

BRAT1 · protein map & ClinVar variants

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

3D Protein StructureAlphaFold

Clinical Trials

Active and recruiting trials from ClinicalTrials.gov

No active trials found for this gene.

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Clinical Literature
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