SSX4

Chr X

SSX family member 4

Also known as: CT5.4

The SSX4 protein functions as a transcriptional repressor and modulator of transcription. Mutations in SSX4 cause synovial sarcoma through characteristic t(X;18) translocations that fuse SSX4 with the synovial sarcoma translocation gene on chromosome 18, following X-linked inheritance patterns.

OMIMResearchSummary from RefSeq, UniProt
LOEUF 1.30
Clinical SummarySSX4
Population Constraint (gnomAD)
Moderately constrained gene (pLI 0.52) — some intolerance to loss-of-function variants.
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ClinVar Variants
85 unique Pathogenic / Likely Pathogenic· 14 VUS of 100 total submissions
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Clinical Trials
1 active or recruiting trial — potential therapeutic options may be available

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Moderate LoF intolerance
LoF Constraint
1.30LOEUF
pLI 0.520
Z-score 1.35
OE 0.00 (0.001.30)
Moderately constrained

Highly tolerant — LoF variants common in population

Missense Constraint
-0.42Z-score
OE missense 1.27 (0.921.75)
24 obs / 18.9 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios
LoF OE0.00 (0.001.30)
00.351.4
Missense OE1.27 (0.921.75)
00.61.4
Synonymous OE0.68
01.21.6
LoF obs/exp: 0 / 2.1Missense obs/exp: 24 / 18.9Syn Z: 0.68

ClinVar Variant Classifications

100 submitted variants in ClinVar

Classification Summary

Pathogenic82
Likely Pathogenic3
VUS14
Likely Benign1
82
Pathogenic
3
Likely Pathogenic
14
VUS
1
Likely Benign

Curated Variants Distribution

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

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
82
Likely Pathogenic
3
VUS
14
Likely Benign
1
Benign
0
Total100

Counts from ClinVar esearch · Updated hourly

View in ClinVar →

Protein Context — Lollipop Plot

SSX4 · protein map & ClinVar variants

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

3D Protein StructureAlphaFold
Clinical Literature
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