GRAP

Chr 17AR

GRB2 related adaptor protein

Also known as: DFNB114

This gene encodes a cytoplasmic signaling protein that couples signals from receptor and cytoplasmic tyrosine kinases to the Ras signaling pathway through its SH2 and SH3 domains. Mutations cause autosomal recessive deafness-114, affecting inner ear function and hearing. The gene shows tolerance to loss-of-function variants (pLI = 0.11), consistent with the recessive inheritance pattern observed clinically.

OMIMResearchSummary from RefSeq, OMIM, UniProt
MultiplemechanismARLOEUF 1.221 OMIM phenotype
Clinical SummaryGRAP
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Gene-Disease Validity (ClinGen)
nonsyndromic genetic hearing loss · ARLimited

Limited evidence — not for standalone diagnostic reporting

Population Constraint (gnomAD)
Low constraint (pLI 0.11) — loss-of-function variants are relatively tolerated in the population.

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
1.22LOEUF
pLI 0.111
Z-score 1.28
OE 0.39 (0.161.22)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
1.14Z-score
OE missense 0.64 (0.500.81)
49 obs / 77.1 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.39 (0.161.22)
00.351.4
Missense OE0.64 (0.500.81)
00.61.4
Synonymous OE0.89
01.21.6
LoF obs/exp: 2 / 5.1Missense obs/exp: 49 / 77.1Syn Z: 0.50
DN
0.6939th %ile
GOF
0.72top 25%
LOF
0.2872th %ile

This gene has evidence for multiple mechanisms of pathogenicity (gain-of-function and dominant-negative). Both the Badonyi & Marsh prediction and the broader genomic evidence point to gain-of-function as the predominant mechanism. Different variants in this gene may act through different mechanisms — interpret in context of the specific variant.

GOFprediction above median
DNprediction 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.

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

GRAP · 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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Recent Gene-Specific Literature
Gene in title · MEDLINE · newest first
Europe PMC