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DNA repair protein complementing XP-A cells; xeroderma pigmentosum group A-complementing protein (XPA, XPAC)

Function: - role in DNA excision repair - initiates repair by binding to damaged sites with various affinities, depending on the photoproduct & the transcriptional state of the region - required for UV-induced CHK1 phosphorylation & the recruitment of CEP164 to cyclobutane pyrimidine dimmers (CPD), sites of DNA damage after UV irradiation - phosphorylated upon DNA damage, probably by ATM or ATR - interacts with XAB1 & RPA1 - interacts (via N-terminus) with CEP164 upon UV irradiation Structure: belongs to the XPA family Compartment: nucleus Expression: - expressed in various cell lines & in skin fibroblasts Pathology: - defects in XPA are a cause of xeroderma pigmentosum complementation group A Comparative biology: - RAD14 protein in yeast

Related

XP A-correcting protein gene or RAD14

General

Xeroderma pigmentosum group complementing protein

Properties

SIZE: MW = 31 kD entity length = 273 aa COMPARTMENT: cell nucleus MOTIF: CEP164/UV resistance {4-97} MOTIF: binding site SITE: 4-29 FOR-BINDING-OF: replication protein A 32 kD subunit nuclear translocation signal {26-47} binding site SITE: 72-84 FOR-BINDING-OF: DNA excision repair protein ERCC1 MOTIF: glutamate-rich region {78-84} MOTIF: glutamate residue (SEVERAL) binding site SITE: 98-187 FOR-BINDING-OF: replication protein A 70 kD DNA-binding subunit MOTIF: Zinc finger NAME: Zinc finger SITE: 105-129 EFFECTOR-BOUND: Zn+2 Ser phosphorylation site {S196} binding site SITE: 226-273 FOR-BINDING-OF: transcription factor IIH

References

  1. UniProt :accession P23025
  2. Allelic variations of the XP genes http://www.xpmutations.org/
  3. Atlas of Genetics & Cytogenetics in Oncology & Haematology http://atlasgeneticsoncology.org/genes/XPAID104.html
  4. GeneReviews https://www.genecards.org/cgi-bin/carddisp.pl?gene=XPA
  5. NIEHS-SNPs http://egp.gs.washington.edu/data/xpa/
  6. Bootsma D & Hoeijmakers JH DNA repair. Engagement with transcription. Nature 363:114 1993 PMID: 8483493
  7. He Z, Henricksen LA, Wold MS, Ingles CJ. RPA involvement in the damage-recognition and incision steps of nucleotide excision repair. Nature. 1995 Apr 6;374(6522):566-9. PMID: 7700386 (RFA binding)
  8. de Laat WL et al, Molecular mechonisms of nucleotide excision repair. Genes & Dev 13:768-85, 1999 PMID: 10197977

Databases & Figures

OMIM correlations MORBIDMAP 611153 UniProt P23025 PFAM correlations Entrez Gene 7507 Kegg hsa:7507 Model for nucleotide excision repair (v2)