DAP3

Chr 1AR

death associated protein 3

Also known as: DAP-3, MRP-S29, MRPS29, PRLTS7, S29mt, bMRP-10, mS29

The protein functions as a component of the mitochondrial small ribosomal subunit and translates mitochondrial mRNAs, displaying GTPase activity and participating in interferon-gamma-induced cell death pathways. Mutations cause Perrault syndrome 7, an autosomal recessive disorder characterized by sensorineural hearing loss and ovarian dysgenesis in females. The gene is highly constrained against loss-of-function variants (pLI ~0, LOEUF 1.105), indicating that heterozygous loss-of-function is well-tolerated but homozygous loss causes disease.

GeneReviewsOMIMResearchSummary from RefSeq, OMIM, UniProt
DNmechanismARLOEUF 1.101 OMIM phenotype
Clinical SummaryDAP3
Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
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GeneReview available — DAP3
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Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
1.10LOEUF
pLI 0.000
Z-score 1.17
OE 0.74 (0.501.10)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
0.31Z-score
OE missense 0.94 (0.841.06)
206 obs / 218.9 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.74 (0.501.10)
00.351.4
Missense OE0.94 (0.841.06)
00.61.4
Synonymous OE0.98
01.21.6
LoF obs/exp: 17 / 23.1Missense obs/exp: 206 / 218.9Syn Z: 0.13
DN
0.6260th %ile
GOF
0.3392th %ile
LOF
0.3551th %ile

The highest-scoring mechanism for this gene is dominant-negative.

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

DAP3 · 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