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DNA repair protein complementing XP-C cells; xeroderma pigmentosum group C-complementing protein; p125 (XPC, XPCC)

Function: - required for nucleotide excision repair - may play a part in DNA damage recognition &/or in altering chromatin structure to allow access by damage-processing enzymes - subunit of a heterodimeric protein that binds strongly to single-stranded DNA [2] - phosphorylated upon DNA damage, probably by ATM or ATR - interacts with CETN2 Structure: belongs to the XPC family Compartment: nucleus (probable) Pathology: - defects in XPC are a cause of xeroderma pigmentosum complementation group C Comparative biology: - RAD4 protein in yeast

Related

XP C-correcting protein gene or RAD4

General

protein subunit Xeroderma pigmentosum group complementing protein

Properties

SIZE: entity length = 940 aa MW = 106 kD COMPARTMENT: cell nucleus MOTIF: glutamate-rich region {30-177} MOTIF: glutamate residue (SEVERAL) glutamate-rich region {30-34} MOTIF: glutamate residue (SEVERAL) Ser phosphorylation site {S94} glutamate-rich region {124-130} MOTIF: glutamate residue (SEVERAL) Thr phosphorylation site {T169} Ser phosphorylation site {S347} Ser phosphorylation site {S351} lysine-rich region {359-395} MOTIF: lysine residue (SEVERAL) peptide motif {390-395} lysine-rich region {408-431} MOTIF: lysine residue (SEVERAL) acidic region {432-461} MOTIF: acidic residue (SEVERAL) lysine-rich region {466-493} MOTIF: lysine residue (SEVERAL) serine-rich region {501-507} MOTIF: serine residue (SEVERAL) Ser phosphorylation site {S883} Ser phosphorylation site {S884} Ser phosphorylation site {S891} Ser phosphorylation site {S892}

Database Correlations

OMIM 278720 UniProt Q01831 PFAM correlations Kegg hsa:7508

References

  1. Bootsma D & Hoeijmakers JH DNA repair. Engagement with transcription. Nature 363:114 1993 PMID: 8483493
  2. Lehmann AR. Nucleotide excision repair and the link with transcription. Trends Biochem Sci. 1995 Oct;20(10):402-5. Review. PMID: 8533152
  3. UniProt :accession Q01831
  4. Allelic variations of the XP genes http://www.xpmutations.org/
  5. Atlas of Genetics & Cytogenetics in Oncology & Haematology http://atlasgeneticsoncology.org/genes/XPCID122.html
  6. GeneReviews https://www.genecards.org/cgi-bin/carddisp.pl?gene=XPC
  7. NIEHS-SNPs http://egp.gs.washington.edu/data/xpc/

Component-of

XPC/hHR23B heterodimer