PROSITE documentation PDOC00611XPA 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.
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PROSITE methods (with tools and information) covered by this documentation:
| 1 | Authors | Tanaka K. Wood R.D. |
| Title | Xeroderma pigmentosum and nucleotide excision repair of DNA. | |
| Source | Trends Biochem. Sci. 19:83-86(1994). | |
| PubMed ID | 8160271 |
| 2 | Authors | Miura N. Miyamoto I. Asahina H. Satokata I. Tanaka K. Okada Y. |
| Title | Identification and characterization of xpac protein, the gene product of the human XPAC (xeroderma pigmentosum group A complementing) gene. | |
| Source | J. Biol. Chem. 266:19786-19789(1991). | |
| PubMed ID | 1918083 |
| 3 | Authors | Shimamoto T. Kohno K. Tanaka K. Okada Y. |
| Title | Molecular cloning of human XPAC gene homologs from chicken, Xenopus laevis and Drosophila melanogaster. | |
| Source | Biochem. Biophys. Res. Commun. 181:1231-1237(1991). | |
| PubMed ID | 1764072 |
| 4 | Authors | Bankmann M. Prakash L. Prakash S. |
| Title | Yeast RAD14 and human xeroderma pigmentosum group A DNA-repair genes encode homologous proteins. | |
| Source | Nature 355:555-558(1992). | |
| PubMed ID | 1741034 | |
| DOI | 10.1038/355555a0 |
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