DDX3Y

Chr Y

DEAD-box helicase 3 Y-linked

Also known as: DBY

The protein encoded by this gene is a member of the DEAD-box RNA helicase family, characterized by nine conserved motifs, included the conserved Asp-Glu-Ala-Asp (DEAD) motif. These motifs are thought to be involved in ATP binding, hydrolysis, RNA binding, and in the formation of intramolecular interactions. This protein shares high similarity to DDX3X, on the X chromosome, but a deletion of this gene is not complemented by DDX3X. Mutations in this gene result in male infertility, a reduction in germ cell numbers, and can result in Sertoli-cell only sydrome. Pseudogenes sharing similarity to both this gene and the DDX3X paralog are found on chromosome 4 and the X chromosome. Alternative splicing results in multiple transcript variants encoding different isoforms. [provided by RefSeq, Oct 2014]

0
Active trials
15
Pubs (1 yr)
67
P/LP submissions
P/LP missense
0.29
LOEUF· LoF intol.
Mechanism
Clinical SummaryDDX3Y
Population Constraint (gnomAD)
Highly constrained gene — heterozygous loss-of-function variants are very rare in the population (pLI 0.96). One damaged copy is likely sufficient to cause disease.
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ClinVar Variants
67 unique Pathogenic / Likely Pathogenic· 6 VUS of 120 total submissions
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GeneReview available — DDX3Y
Authoritative clinical overview · Recommended first read
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Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

LoF intolerant — likely haploinsufficient
LoF Constraint
0.29LOEUF
pLI 0.965
Z-score 2.99
OE 0.00 (0.000.29)
Highly constrained

Highly LoF-intolerant (top ~10% of genes)

Missense Constraint
2.10Z-score
OE missense 0.45 (0.360.57)
53 obs / 117.0 exp
Mild constraint

Moderately missense-constrained (top ~2.5%)

Observed / Expected Ratios
LoF OE0.00 (0.000.29)
00.351.4
Missense OE0.45 (0.360.57)
00.61.4
Synonymous OE0.79
01.21.6
LoF obs/exp: 0 / 10.4Missense obs/exp: 53 / 117.0Syn Z: 0.96

ClinVar Variant Classifications

120 submitted variants in ClinVar

Classification Summary

Pathogenic60
Likely Pathogenic7
VUS6
Likely Benign3
60
Pathogenic
7
Likely Pathogenic
6
VUS
3
Likely Benign

Curated Variants Distribution

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

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
60
Likely Pathogenic
7
VUS
6
Likely Benign
3
Benign
0
Total76

Counts from ClinVar esearch · Updated hourly

View in ClinVar →

Protein Context — Lollipop Plot

DDX3Y · 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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