xeroderma pigmentosum complementation group A

(redirected from XP-A)

xeroderma pigmentosum complementation group A

A rare autosomal recessive disorder (OMIM:278700) characterised by UV light sensitivity, a markedly increased risk of UV light-induced malignancy and, in some cases, neurologic disease.

Molecular pathology
Caused by defects of XPA, which encodes a protein of the nucleotide excision repair (NER) complex responsible for repair of UV light- and chemical-carcinogen-induced DNA adducts.
References in periodicals archive ?
In people with XP-A, for example, DNA repair is almost completely knocked out, causing brain deterioration and many skin tumors.