Xeroderma Pigmentosum, Complementation Group C (XPC)

XP-C; XP3; XPCC; RAD4; Xeroderma Pigmentosum Group C Protein; DNA repair protein complementing XP-C cells

SPN	W333
HGNC	12816
MGI	103557
PubMed	1649389
RGD	1305760
UniProt	Q01831
Wiki	XPC_(gene

Xeroderma pigmentosum, complementation group C, also known as XPC, is a component of the nucleotide excision repair (NER) pathway. There are multiple components involved in the NER pathway, including Xeroderma pigmentosum (XP) A-G and V, Cockayne syndrome (CS) A and B, and trichothiodystrophy (TTD) group A, etc.

This component, XPC, plays an important role in the early steps of global genome NER, especially in damage recognition, open complex formation, and repair protein complex formation.Mutations in this gene or some other NER components result in Xeroderma pigmentosum, a rare autosomal recessive disorder characterized by increased sensitivity to sunlight with the development of carcinomas at an early age.

Reference

"Purification and cloning of a nucleotide excision repair complex involving the Xeroderma pigmentosum group C protein and a human homologue of yeast RAD23."
EMBO J. 13:1831-1843(1994) [PubMed] [Europe PMC] [Abstract]

"The human XPC DNA repair gene: arrangement, splice site information content and influence of a single nucleotide polymorphism in a splice acceptor site on alternative splicing and function."
Nucleic Acids Res. 30:3624-3631(2002) [PubMed] [Europe PMC] [Abstract]

"Protein interaction network of alternatively spliced isoforms from brain links genetic risk factors for autism."
Nat. Commun. 5:3650-3650(2014) [PubMed] [Europe PMC] [Abstract]

"Complete sequencing and characterization of 21,243 full-length human cDNAs."
Nat. Genet. 36:40-45(2004) [PubMed] [Europe PMC] [Abstract]

"The DNA sequence, annotation and analysis of human chromosome 3."
Nature 440:1194-1198(2006) [PubMed] [Europe PMC] [Abstract]

"The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)."
Genome Res. 14:2121-2127(2004) [PubMed] [Europe PMC] [Abstract]

"Expression cloning of a human DNA repair gene involved in Xeroderma pigmentosum group C."
Nature 359:70-73(1992) [PubMed] [Europe PMC] [Abstract]

Erratum
Nature 360:610-610(1992) [PubMed] [Europe PMC] [Abstract]

"XPC and human homologs of RAD23: intracellular localization and relationship to other nucleotide excision repair complexes."
Nucleic Acids Res. 24:2551-2559(1996) [PubMed] [Europe PMC] [Abstract]

"Two human homologs of Rad23 are functionally interchangeable in complex formation and stimulation of XPC repair activity."
Mol. Cell. Biol. 17:6924-6931(1997) [PubMed] [Europe PMC] [Abstract]

"Xeroderma pigmentosum group C protein complex is the initiator of global genome nucleotide excision repair."
Mol. Cell 2:223-232(1998) [PubMed] [Europe PMC] [Abstract]

"The xeroderma pigmentosum group C protein complex XPC-HR23B plays an important role in the recruitment of transcription factor IIH to damaged DNA."
J. Biol. Chem. 275:9870-9875(2000) [PubMed] [Europe PMC] [Abstract]

"Stable binding of human XPC complex to irradiated DNA confers strong discrimination for damaged sites."
J. Mol. Biol. 300:275-290(2000) [PubMed] [Europe PMC] [Abstract]

"Centrosome protein centrin 2/caltractin 1 is part of the xeroderma pigmentosum group C complex that initiates global genome nucleotide excision repair."
J. Biol. Chem. 276:18665-18672(2001) [PubMed] [Europe PMC] [Abstract]

"A molecular mechanism for DNA damage recognition by the xeroderma pigmentosum group C protein complex."
DNA Repair 1:95-107(2002) [PubMed] [Europe PMC] [Abstract]

"The carboxy-terminal domain of the XPC protein plays a crucial role in nucleotide excision repair through interactions with transcription factor IIH."
DNA Repair 1:449-461(2002) [PubMed] [Europe PMC] [Abstract]

"DNA bending by the human damage recognition complex XPC-HR23B."
DNA Repair 2:325-336(2003) [PubMed] [Europe PMC] [Abstract]

"Xeroderma pigmentosum group C protein interacts physically and functionally with thymine DNA glycosylase."
EMBO J. 22:164-173(2003) [PubMed] [Europe PMC] [Abstract]

"UV-induced ubiquitylation of XPC protein mediated by UV-DDB-ubiquitin ligase complex."
Cell 121:387-400(2005) [PubMed] [Europe PMC] [Abstract]

"Centrin 2 stimulates nucleotide excision repair by interacting with xeroderma pigmentosum group C protein."
Mol. Cell. Biol. 25:5664-5674(2005) [PubMed] [Europe PMC] [Abstract]

"Global, in vivo, and site-specific phosphorylation dynamics in signaling networks."
Cell 127:635-648(2006) [PubMed] [Europe PMC] [Abstract]

"Toward a global characterization of the phosphoproteome in prostate cancer cells: identification of phosphoproteins in the LNCaP cell line."
Electrophoresis 28:2027-2034(2007) [PubMed] [Europe PMC] [Abstract]

"ATM and ATR substrate analysis reveals extensive protein networks responsive to DNA damage."
Science 316:1160-1166(2007) [PubMed] [Europe PMC] [Abstract]

"Combining protein-based IMAC, peptide-based IMAC, and MudPIT for efficient phosphoproteomic analysis."
J. Proteome Res. 7:1346-1351(2008) [PubMed] [Europe PMC] [Abstract]

"A quantitative atlas of mitotic phosphorylation."
Proc. Natl. Acad. Sci. U.S.A. 105:10762-10767(2008) [PubMed] [Europe PMC] [Abstract]

"Large-scale phosphoproteome analysis of human liver tissue by enrichment and fractionation of phosphopeptides with strong anion exchange chromatography."
Proteomics 8:1346-1361(2008) [PubMed] [Europe PMC] [Abstract]

"Quantitative phosphoproteomic analysis of T cell receptor signaling reveals system-wide modulation of protein-protein interactions."
Sci. Signal. 2:RA46-RA46(2009) [PubMed] [Europe PMC] [Abstract]

"Quantitative phosphoproteomics reveals widespread full phosphorylation site occupancy during mitosis."
Sci. Signal. 3:RA3-RA3(2010) [PubMed] [Europe PMC] [Abstract]

"System-wide temporal characterization of the proteome and phosphoproteome of human embryonic stem cell differentiation."
Sci. Signal. 4:RS3-RS3(2011) [PubMed] [Europe PMC] [Abstract]

"An enzyme assisted RP-RPLC approach for in-depth analysis of human liver phosphoproteome."
J. Proteomics 96:253-262(2014) [PubMed] [Europe PMC] [Abstract]

"In vivo destabilization and functional defects of the xeroderma pigmentosum C protein caused by a pathogenic missense mutation."
Mol. Cell. Biol. 27:6606-6614(2007) [PubMed] [Europe PMC] [Abstract]

"An aromatic sensor with aversion to damaged strands confers versatility to DNA repair."
PLoS Biol. 5:E79-E79(2007) [PubMed] [Europe PMC] [Abstract]

"Versatile DNA damage detection by the global genome nucleotide excision repair protein XPC."
J. Cell Sci. 121:2850-2859(2008) [PubMed] [Europe PMC] [Abstract]

Erratum
J. Cell Sci. 121:2972-2972(2008)

Erratum
J. Cell Sci. 121:3991-3991(2008)

"Two-stage dynamic DNA quality check by xeroderma pigmentosum group C protein."
EMBO J. 28:2387-2399(2009) [PubMed] [Europe PMC] [Abstract]

"Two-step recognition of DNA damage for mammalian nucleotide excision repair: Directional binding of the XPC complex and DNA strand scanning."
Mol. Cell 36:642-653(2009) [PubMed] [Europe PMC] [Abstract]

"Dissection of the xeroderma pigmentosum group C protein function by site-directed mutagenesis."
Antioxid. Redox Signal. 14:2479-2490(2011) [PubMed] [Europe PMC] [Abstract]

"Photo-cross-linking of XPC-Rad23B to cisplatin-damaged DNA reveals contacts with both strands of the DNA duplex and spans the DNA adduct."
Biochemistry 49:669-678(2010) [PubMed] [Europe PMC] [Abstract]

"Stimulation of DNA glycosylase activities by XPC Protein Complex: Roles of protein-protein interactions."
J. Nucleic Acids 2010:455-459(2010) [PubMed] [Europe PMC] [Abstract]

"Flexibility and plasticity of human centrin 2 binding to the xeroderma pigmentosum group C protein (XPC) from nuclear excision repair."
Biochemistry 45:3653-3663(2006) [PubMed] [Europe PMC] [Abstract]

"The structure of the human centrin 2-xeroderma pigmentosum group C protein complex."
J. Biol. Chem. 281:18746-18752(2006) [PubMed] [Europe PMC] [Abstract]

"Structural, thermodynamic, and cellular characterization of human centrin 2 interaction with xeroderma pigmentosum group C protein."
J. Mol. Biol. 373:1032-1046(2007) [PubMed] [Europe PMC] [Abstract]

"A summary of mutations in the UV-sensitive disorders: xeroderma pigmentosum, Cockayne syndrome, and trichothiodystrophy."
Hum. Mutat. 14:9-22(1999) [PubMed] [Europe PMC] [Abstract]

"Characterization of molecular defects in Xeroderma pigmentosum group C."
Nat. Genet. 5:413-417(1993) [PubMed] [Europe PMC] [Abstract]

"Mutations in the XPC gene in families with xeroderma pigmentosum and consequences at the cell, protein, and transcript levels."
Cancer Res. 60:1974-1982(2000) [PubMed] [Europe PMC] [Abstract]