DAZ1

Chr Y

deleted in azoospermia 1

Also known as: DAZ, SPGY

DAZ1 encodes an RNA-binding protein essential for spermatogenesis that regulates mRNA translation in premeiotic germ cells and promotes progression to meiosis. Mutations cause male infertility due to azoospermia (absence of sperm), and the gene follows Y-linked inheritance since it is located on the Y chromosome. This gene is expressed specifically in spermatogonia and is part of the azoospermia factor (AZF) region critical for male fertility.

Summary from RefSeq, UniProt
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Primary Disease Associations & Inheritance

UniProtSpermatogenic failure Y-linked 2
0
Active trials
1
Pubs (1 yr)
77
P/LP submissions
P/LP missense
LOEUF
Multiple*
Mechanism· predicted
Clinical SummaryDAZ1
📋
ClinVar Variants
77 unique Pathogenic / Likely Pathogenic· 13 VUS of 104 total submissions

Population Genetics & Constraint

Constraint data not available from gnomAD.

DN
0.6937th %ile
GOF
0.96top 5%
LOF
0.4135th %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

104 submitted variants in ClinVar

Classification Summary

Pathogenic75
Likely Pathogenic2
VUS13
Likely Benign11
Benign3
75
Pathogenic
2
Likely Pathogenic
13
VUS
11
Likely Benign
3
Benign

Curated Variants Distribution

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

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
75
Likely Pathogenic
2
VUS
13
Likely Benign
11
Benign
3
Total104

Counts from ClinVar esearch · Updated hourly

View in ClinVar →

Protein Context — Lollipop Plot

DAZ1 · 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.

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Clinical Literature
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