XPC

Chr 3AR

XPC complex subunit, DNA damage recognition and repair factor

Also known as: RAD4, XP3, XPCC, p125

The protein encoded by this gene is a key component of the XPC complex, which plays an important role in the early steps of global genome nucleotide excision repair (NER). The encoded protein is important for damage sensing and DNA binding, and shows a preference for single-stranded DNA. Mutations in this gene or some other NER components can result in Xeroderma pigmentosum, a rare autosomal recessive disorder characterized by increased sensitivity to sunlight with the development of carcinomas at an early age. Alternatively spliced transcript variants have been found for this gene. [provided by RefSeq, Aug 2017]

OMIMResearchGenerating clinical summary…
LOFmechanismARLOEUF 0.782 OMIM phenotypes
Clinical SummaryXPC
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Gene-Disease Validity (ClinGen)
xeroderma pigmentosum group C · ARDefinitive

Definitive — sufficient evidence for diagnostic panels

Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
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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

Tolerant — LoF & missense variants common in population
LoF Constraint?
0.78LOEUF
pLI 0.000
Z-score 2.70
OE 0.56 (0.400.78)
Tolerant

Typical tolerance to LoF variation

Missense Constraint?
0.69Z-score
OE missense 0.91 (0.850.99)
483 obs / 527.9 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios?
LoF OE?0.56 (0.400.78)
00.351.4
Missense OE?0.91 (0.850.99)
00.61.4
Synonymous OE?0.99
01.21.6
LoF obs/exp: 24 / 43.2Missense obs/exp: 483 / 527.9Syn Z: 0.13

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

XPC · protein map & ClinVar variants

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