DAZ3

Chr Y

deleted in azoospermia 3

Also known as: pDP1679

DAZ3 encodes an RNA-binding protein that regulates mRNA translation and plays an essential role in spermatogenesis, with expression restricted to premeiotic germ cells. Mutations cause azoospermia (absence of sperm) as part of the Y-chromosomal azoospermia factor, leading to male infertility. This gene is located on the Y chromosome and follows Y-linked inheritance, passing from father to son.

Summary from RefSeq, UniProt
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Primary Disease Associations & Inheritance

UniProtSpermatogenic failure Y-linked 2
0
Active trials
0
Pubs (1 yr)
77
P/LP submissions
P/LP missense
LOEUF
Multiple*
Mechanism· predicted
Clinical SummaryDAZ3
📋
ClinVar Variants
77 unique Pathogenic / Likely Pathogenic· 19 VUS of 106 total submissions

Population Genetics & Constraint

Constraint data not available from gnomAD.

DN
0.76top 25%
GOF
0.79top 25%
LOF
0.3549th %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

106 submitted variants in ClinVar

Classification Summary

Pathogenic74
Likely Pathogenic3
VUS19
Likely Benign7
Benign3
74
Pathogenic
3
Likely Pathogenic
19
VUS
7
Likely Benign
3
Benign

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories· variant type breakdown unavailable

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
74
Likely Pathogenic
3
VUS
19
Likely Benign
7
Benign
3
Total106

Counts from ClinVar esearch · Updated hourly

View in ClinVar →

Protein Context — Lollipop Plot

DAZ3 · protein map & ClinVar variants

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

Clinical Trials

Active and recruiting trials from ClinicalTrials.gov

No active trials found for this gene.

Search ClinicalTrials.gov →
Clinical Literature
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Recent Gene-Specific Literature
Gene in title · MEDLINE · newest first
Europe PMC