XPA

This gene encodes a zinc finger protein plays a central role in nucleotide excision repair (NER), a specialized type of DNA repair. NER is responsible for repair of UV radiation-induced photoproducts and DNA adducts induced by chemical carcinogens and chemotherapeutic drugs. The encoded protein interacts with DNA and several NER proteins, acting as a scaffold to assemble the NER incision complex at sites of DNA damage. Mutations in this gene cause Xeroderma pigmentosum complementation group A (XP-A), an autosomal recessive skin disorder featuring hypersensitivity to sunlight and increased risk for skin cancer. [provided by RefSeq, Aug 2017]

XPA, DNA Damage Recognition And Repair Factor

Involved in DNA excision repair. Initiates repair by binding to damaged sites with various affinities, depending on the photoproduct and the transcriptional state of the region. Required for UV-induced CHEK1 phosphorylation and the recruitment of CEP164 to cyclobutane pyrimidine dimmers (CPD), sites of DNA damage after UV irradiation.

Biological Process base-excision repair Source:GO_Central1 Publication
Biological Process DNA repair Source:CAFA1 Publication
Biological Process nucleotide-excision repair involved in interstrand cross-link repair Source:GO_Central1 Publication
Biological Process nucleotide-excision repair, DNA damage recognition Source:GO_Central1 Publication
Biological Process nucleotide-excision repair, DNA incision Source:CACAO1 Publication
Biological Process protein localization to nucleus Source:CAFA1 Publication
Biological Process UV protection Source:CAFA1 Publication
Biological Process UV-damage excision repair Source:GO_Central1 Publication

Nucleus

Xeroderma pigmentosum complementation group A (XP-A):
An autosomal recessive pigmentary skin disorder characterized by solar hypersensitivity of the skin, high predisposition for developing cancers on areas exposed to sunlight and, in some cases, neurological abnormalities. The skin develops marked freckling and other pigmentation abnormalities. XP-A patients show the most severe skin symptoms and progressive neurological disorders.

ATR-dependent phosphorylation of XPA at Ser-196 is important for cell survival in response to UV damage.
Ubiquitinated by HERC2 leading to degradation by the proteasome.