Gene Symbol: Xpa
Description: XPA, DNA damage recognition and repair factor
Alias: DNA repair protein complementing XP-A cells, xeroderma pigmentosum, complementation group A
Species: rat
Products:     Xpa

Top Publications

  1. Tanaka K, Miura N, Satokata I, Miyamoto I, Yoshida M, Satoh Y, et al. Analysis of a human DNA excision repair gene involved in group A xeroderma pigmentosum and containing a zinc-finger domain. Nature. 1990;348:73-6 pubmed
    ..The human and mouse XPAC genes are located on chromosome 9q34.1 and chromosome 4C2, respectively. Human XPAC cDNA encodes a protein of 273 amino acids with a zinc-finger motif. ..
  2. Park C, Sancar A. Formation of a ternary complex by human XPA, ERCC1, and ERCC4(XPF) excision repair proteins. Proc Natl Acad Sci U S A. 1994;91:5017-21 pubmed
    The xeroderma pigmentosum complementation group A (XP-A) protein, XPA, has recently been expressed in Escherichia coli in a soluble and fully functional form. An affinity column was prepared by linking the XPA protein to a solid support...
  3. Wijnhoven S, Kool H, Mullenders L, Slater R, van Zeeland A, Vrieling H. DMBA-induced toxic and mutagenic responses vary dramatically between NER-deficient Xpa, Xpc and Csb mice. Carcinogenesis. 2001;22:1099-106 pubmed
    ..Aprt gene in splenic T-lymphocytes after 7,12-dimethyl-1,2-benz[a]anthracene (DMBA) exposure in total NER-deficient Xpa mice, global genome repair (GGR)-deficient Xpc mice and transcription coupled repair (TCR)-deficient Csb mice...
  4. Guthrie O, Carrero Martinez F. Real-time quantification of Xeroderma pigmentosum mRNA from the mammalian cochlea. Ear Hear. 2010;31:714-21 pubmed publisher
    Human mutations in the DNA repair genes, Xeroderma pigmentosum (XP)-C and XPA result in hearing loss, which has fueled the hypothesis that there is a significant demand for these genes in protecting cochlear genetic material...
  5. Andressoo J, Weeda G, de Wit J, Mitchell J, Beems R, van Steeg H, et al. An Xpb mouse model for combined xeroderma pigmentosum and cockayne syndrome reveals progeroid features upon further attenuation of DNA repair. Mol Cell Biol. 2009;29:1276-90 pubmed publisher
    ..to show overt CS features unless the DNA repair capacity is further challenged by crossings to the NER-deficient Xpa background...
  6. Miyamoto I, Miura N, Niwa H, Miyazaki J, Tanaka K. Mutational analysis of the structure and function of the xeroderma pigmentosum group A complementing protein. Identification of essential domains for nuclear localization and DNA excision repair. J Biol Chem. 1992;267:12182-7 pubmed
    ..These results suggest that all 4 cysteines forming a zinc-finger structure and also the glutamic acid cluster are important for DNA repair function. ..
  7. Bol S, van Steeg H, Jansen J, Van Oostrom C, de Vries A, de Groot A, et al. Elevated frequencies of benzo(a)pyrene-induced Hprt mutations in internal tissue of XPA-deficient mice. Cancer Res. 1998;58:2850-6 pubmed
    ..Recently, mice have been generated via gene targeting that are deficient in the expression of the XPA gene [A. de Vries et al., Nature (Lond.), 377: 169-173, 1995]...
  8. van Oosten M, Rebel H, Friedberg E, van Steeg H, van der Horst G, van Kranen H, et al. Differential role of transcription-coupled repair in UVB-induced G2 arrest and apoptosis in mouse epidermis. Proc Natl Acad Sci U S A. 2000;97:11268-73 pubmed
    ..epidermis of UVB-exposed CS group B (Csb(-/-)) mice (no TCR), XP group C (Xpc(-/-)) mice (no GGR), and XP group A (Xpa(-/-)) mice (no TCR and no GGR)...
  9. Guthrie O. Localization and distribution of neurons that co-express xeroderma pigmentosum-A and epidermal growth factor receptor within Rosenthal's canal. Acta Histochem. 2015;117:688-95 pubmed publisher
    Xeroderma pigmentosum-A (XPA) is a C4-type zinc-finger scaffolding protein that regulates the removal of bulky-helix distorting DNA damage products from the genome...

More Information


  1. Ng J, Vermeulen W, van der Horst G, Bergink S, Sugasawa K, Vrieling H, et al. A novel regulation mechanism of DNA repair by damage-induced and RAD23-dependent stabilization of xeroderma pigmentosum group C protein. Genes Dev. 2003;17:1630-45 pubmed
    ..These findings resolve the primary function of RAD23 in repair and reveal a novel DNA-damage-dependent regulation mechanism of DNA repair in eukaryotes, which may be part of a more global damage-response circuitry. ..
  2. Garssen J, van Steeg H, De Gruijl F, de Boer J, van der Horst G, van Kranen H, et al. Transcription-coupled and global genome repair differentially influence UV-B-induced acute skin effects and systemic immunosuppression. J Immunol. 2000;164:6199-205 pubmed
    ..in NER were compared: CSB and XPC mice lacking transcription-coupled or global genome NER, respectively, as well as XPA and TTD/XPD mice carrying complete or partial defects in both NER subpathways, respectively...
  3. Saijo M, Takedachi A, Tanaka K. Nucleotide excision repair by mutant xeroderma pigmentosum group A (XPA) proteins with deficiency in interaction with RPA. J Biol Chem. 2011;286:5476-83 pubmed publisher
    The xeroderma pigmentosum group A protein (XPA) is a core component of nucleotide excision repair (NER)...
  4. Sakaguchi K, Ishibashi T, Uchiyama Y, Iwabata K. The multi-replication protein A (RPA) system--a new perspective. FEBS J. 2009;276:943-63 pubmed publisher
    ..Convergent evolution seems to be ubiquitous in these processes. Using recent findings, we review the composition and biological functions of RPA complexes. ..
  5. Guthrie O. Noise Stress Induces an Epidermal Growth Factor Receptor/Xeroderma Pigmentosum-A Response in the Auditory Nerve. J Histochem Cytochem. 2017;65:173-184 pubmed publisher
    ..by mobilizing one or more epidermal growth factor receptor (EGFR) cascades that employ xeroderma pigmentosum-A (XPA) to repair damaged genes...
  6. Kamileri I, Karakasilioti I, Garinis G. Nucleotide excision repair: new tricks with old bricks. Trends Genet. 2012;28:566-73 pubmed publisher
    ..Together, these findings add new pieces to the puzzle for understanding NER and the relevance of NER defects in development and disease. ..
  7. Guthrie O. Preincision complex-I from the excision nuclease reaction among cochlear spiral limbus and outer hair cells. J Mol Histol. 2008;39:617-25 pubmed publisher
    ..PIC-1 is maintained by xeroderma pigmentosum A and C (XPA and XPC) through protein-protein interactions and DNA binding...
  8. Yang S, Zhang Y, Zhang L, Huang Y, Sun F. Immunohistochemical analysis of nucleotide excision repair factors XPA and XPB in adult rat brain. Anat Rec (Hoboken). 2008;291:775-80 pubmed publisher
    To study the regional and cellular distribution of xeroderma pigmentosum group A and B (XPA and XPB) proteins, two nucleotide excision repair (NER) factors, in the mammalian brain we used immunohistochemistry and triple fluorescent ..
  9. Tripsianes K, Folkers G, Zheng C, Das D, Grinstead J, Kaptein R, et al. Analysis of the XPA and ssDNA-binding surfaces on the central domain of human ERCC1 reveals evidence for subfunctionalization. Nucleic Acids Res. 2007;35:5789-98 pubmed
    ..reveals a completely distinct function for the ERCC1 central domain by performing interactions with both XPA and single-stranded DNA...
  10. de Waard H, de Wit J, Gorgels T, van den Aardweg G, Andressoo J, Vermeij M, et al. Cell type-specific hypersensitivity to oxidative damage in CSB and XPA mice. DNA Repair (Amst). 2003;2:13-25 pubmed
    ..of genetic background and directly related to the CSB defect and is not observed in totally NER-deficient XPA MEFs...
  11. Guthrie O, Xu H. Noise exposure potentiates the subcellular distribution of nucleotide excision repair proteins within spiral ganglion neurons. Hear Res. 2012;294:21-30 pubmed publisher
    ..cascades that employs Cockayne syndrome-A (CSA), the xeroderma pigmentosum-C (XPC) and the xeroderma pigmentosum-A (XPA) proteins. Noise exposure induces stress within the spiral ganglia...
  12. Lalonde R, Strazielle C. Behavioral effects of neonatal lesions on the cerebellar system. Int J Dev Neurosci. 2015;43:58-65 pubmed publisher
    ..Deficits are also evident in murine null mutants such as Zic1, Fgfr1/FgFr2, and Xpa/Ercc8...
  13. He Z, Henricksen L, Wold M, Ingles C. RPA involvement in the damage-recognition and incision steps of nucleotide excision repair. Nature. 1995;374:566-9 pubmed
    ..specifically and directly to two excision repair proteins, the xeroderma pigmentosum damage-recognition protein XPA (refs 8, 9) and the endonuclease XPG (refs 10-13)...
  14. de Boer J, Andressoo J, de Wit J, Huijmans J, Beems R, van Steeg H, et al. Premature aging in mice deficient in DNA repair and transcription. Science. 2002;296:1276-9 pubmed
    ..TTD mice carrying an additional mutation in XPA, which enhances the DNA repair defect, showed a greatly accelerated aging phenotype, which correlated with an ..