UVSSA

Chr 4AR

UV stimulated scaffold protein A

Also known as: KIAA1530, UVSS3

This protein serves as a key adapter in transcription-coupled nucleotide excision repair, facilitating removal of DNA lesions that block RNA polymerase II during transcription by promoting polymerase ubiquitination and recruiting repair machinery including the TFIIH complex. Mutations cause UV-sensitive syndrome 3, an autosomal recessive disorder characterized by abnormal sensitivity to ultraviolet radiation. The gene shows minimal constraint against loss-of-function variants (LOEUF 1.48), consistent with its recessive inheritance pattern.

Summary from RefSeq, OMIM, UniProt
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Primary Disease Associations & Inheritance

UV-sensitive syndrome 3MIM #614640
AR
0
Active trials
6
Pubs (1 yr)
0
P/LP submissions
P/LP missense
1.48
LOEUF
LOF
Mechanism· G2P
Clinical SummaryUVSSA
Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
1.48LOEUF
pLI 0.000
Z-score -0.61
OE 1.12 (0.851.48)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
-2.03Z-score
OE missense 1.26 (1.181.35)
593 obs / 469.0 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios
LoF OE1.12 (0.851.48)
00.351.4
Missense OE1.26 (1.181.35)
00.61.4
Synonymous OE1.14
01.21.6
LoF obs/exp: 35 / 31.3Missense obs/exp: 593 / 469.0Syn Z: -1.51

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

UVSSA · protein map & ClinVar variants

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

3D Protein StructureAlphaFold

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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