PROSITE documentation PDOC00611
XPA protein signatures


Xeroderma pigmentosum (XP) [1] is a human autosomal recessive disease, characterized by a high incidence of sunlight-induced skin cancer. People's skin cells with this condition are hypersensitive to ultraviolet light, due to defects in the incision step of DNA excision repair. There are a minimum of seven genetic complementation groups involved in this pathway: XP-A to XP-G. XP-A is the most severe form of the disease and is due to defects in a 30 Kd nuclear protein called XPA (or XPAC) [2].

The sequence of the XPA protein is conserved from higher eukaryotes [3] to yeast (gene RAD14) [4]. XPA is a hydrophilic protein of 247 to 296 amino-acid residues which has a C4-type zinc finger motif in its central section.

We have developed two signature patterns for XPA proteins. The first corresponds to the zinc finger region, the second to a highly conserved region located some 12 residues after the zinc finger region.

Last update:

May 2004 / Text revised.


Technical section

PROSITE methods (with tools and information) covered by this documentation:

XPA_1, PS00752; XPA protein signature 1  (PATTERN)

XPA_2, PS00753; XPA protein signature 2  (PATTERN)


1AuthorsTanaka K. Wood R.D.
TitleXeroderma pigmentosum and nucleotide excision repair of DNA.
SourceTrends Biochem. Sci. 19:83-86(1994).
PubMed ID8160271

2AuthorsMiura N. Miyamoto I. Asahina H. Satokata I. Tanaka K. Okada Y.
TitleIdentification and characterization of xpac protein, the gene product of the human XPAC (xeroderma pigmentosum group A complementing) gene.
SourceJ. Biol. Chem. 266:19786-19789(1991).
PubMed ID1918083

3AuthorsShimamoto T. Kohno K. Tanaka K. Okada Y.
TitleMolecular cloning of human XPAC gene homologs from chicken, Xenopus laevis and Drosophila melanogaster.
SourceBiochem. Biophys. Res. Commun. 181:1231-1237(1991).
PubMed ID1764072

4AuthorsBankmann M. Prakash L. Prakash S.
TitleYeast RAD14 and human xeroderma pigmentosum group A DNA-repair genes encode homologous proteins.
SourceNature 355:555-558(1992).
PubMed ID1741034

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