RBMY1J

Chr Y

RNA binding motif protein Y-linked family 1 member J

The protein functions as an RNA-binding splicing factor required for sperm development during spermatogenesis. Mutations in this Y-linked gene cause male infertility due to azoospermia (absence of sperm), with inheritance following a patrilineal pattern from father to son. The gene is located in the AZFb azoospermia factor region and is part of a gene cluster with multiple paralogs on the Y chromosome.

Summary from RefSeq, UniProt
Research Assistant →
0
Active trials
0
Pubs (1 yr)
71
P/LP submissions
P/LP missense
1.89
LOEUF
DN
Mechanism· predicted
Clinical SummaryRBMY1J
Population Constraint (gnomAD)
Constrained for loss-of-function variants (OE-LoF 0.00) despite low pLI — interpret in context.
📋
ClinVar Variants
71 unique Pathogenic / Likely Pathogenic· 11 VUS of 93 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
1.89LOEUF
pLI 0.287
Z-score 0.23
OE 0.00 (0.001.89)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
0.31Z-score
OE missense 0.00 (0.001.78)
0 obs / 0.8 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.00 (0.001.89)
00.351.4
Missense OE0.00 (0.001.78)
00.61.4
Synonymous OE0.00
01.21.6
LoF obs/exp: 0 / 0.1Missense obs/exp: 0 / 0.8Syn Z: 0.39
DN
0.78top 25%
GOF
0.4776th %ile
LOF
0.3841th %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

93 submitted variants in ClinVar

Classification Summary

Pathogenic68
Likely Pathogenic3
VUS11
Likely Benign8
Benign3
68
Pathogenic
3
Likely Pathogenic
11
VUS
8
Likely Benign
3
Benign

Curated Variants Distribution

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

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
68
Likely Pathogenic
3
VUS
11
Likely Benign
8
Benign
3
Total93

Counts from ClinVar esearch · Updated hourly

View in ClinVar →

Protein Context — Lollipop Plot

RBMY1J · 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
Open Research Assistant →
Key Publications
Landmark & review papers · by relevance
PubMed
Top 1 results · since 2015Search PubMed ↗
Recent Gene-Specific Literature
Gene in title · MEDLINE · newest first
Europe PMC

No open access results found