Xpc

Summary

Gene Symbol: Xpc
Description: xeroderma pigmentosum, complementation group C
Alias: DNA repair protein complementing XP-C cells homolog, p125, xeroderma pigmentosum group C-complementing protein homolog
Species: mouse
Products:     Xpc

Top Publications

  1. van der Spek P, Visser C, Hanaoka F, Smit B, Hagemeijer A, Bootsma D, et al. Cloning, comparative mapping, and RNA expression of the mouse homologues of the Saccharomyces cerevisiae nucleotide excision repair gene RAD23. Genomics. 1996;31:20-7 pubmed
    ..Because of the close chromosomal proximity of human XPC and HHR23B, the mouse XPC chromosomal location was determined (6D)...
  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
    ..reactions (edema and erythema) four congenic mouse strains carrying different defects 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 ..
  3. Okuda Y, Nishi R, Ng J, Vermeulen W, van der Horst G, Mori T, et al. Relative levels of the two mammalian Rad23 homologs determine composition and stability of the xeroderma pigmentosum group C protein complex. DNA Repair (Amst). 2004;3:1285-95 pubmed
    ..Recently, the proteins have been shown to stabilize xeroderma pigmentosum group C (XPC) protein that is involved in DNA damage recognition for nucleotide excision repair (NER)...
  4. Hoogervorst E, Van Oostrom C, Beems R, van Benthem J, van den Berg J, van Kreijl C, et al. 2-AAF-induced tumor development in nucleotide excision repair-deficient mice is associated with a defect in global genome repair but not with transcription coupled repair. DNA Repair (Amst). 2005;4:3-9 pubmed
    ..of these separate sub-pathways in tumor suppression, we exposed mice deficient for either TCR (Csb), GGR (Xpc) or both (Xpa) to 300 ppm 2-acetylaminofluorene (in feed, ad libitum) in a unique comparative exposure experiment...
  5. van Oosten M, Stout G, Backendorf C, Rebel H, de Wind N, Darroudi F, et al. Mismatch repair protein Msh2 contributes to UVB-induced cell cycle arrest in epidermal and cultured mouse keratinocytes. DNA Repair (Amst). 2005;4:81-9 pubmed
    ..In a previous study, we found UVB-induced accumulation of tetraploid (4N) keratinocytes in the epidermis of Xpc(-/-) mice (no GGR), but not in Xpa(-/-) (no TCR and no GGR) or in wild-type (WT) mice...
  6. Melis J, Wijnhoven S, Beems R, Roodbergen M, van den Berg J, Moon H, et al. Mouse models for xeroderma pigmentosum group A and group C show divergent cancer phenotypes. Cancer Res. 2008;68:1347-53 pubmed publisher
    ..of DNA damage is nucleotide excision repair, in which, among others, the Xeroderma pigmentosum group C (XPC) and group A (XPA) proteins are involved...
  7. Cheo D, Meira L, Hammer R, Burns D, Doughty A, Friedberg E. Synergistic interactions between XPC and p53 mutations in double-mutant mice: neural tube abnormalities and accelerated UV radiation-induced skin cancer. Curr Biol. 1996;6:1691-4 pubmed
    ..To examine the consequences of a combined deficiency of the XPC and the p53 proteins in mice, we generated double-mutant animals...
  8. Sands A, Abuin A, Sanchez A, Conti C, Bradley A. High susceptibility to ultraviolet-induced carcinogenesis in mice lacking XPC. Nature. 1995;377:162-5 pubmed
    ..We have isolated a mouse homologue of the human gene for XP group C and generated XPC-deficient mice by using embryonic stem cell technology...
  9. van der Horst G, Meira L, Gorgels T, de Wit J, Velasco Miguel S, Richardson J, et al. UVB radiation-induced cancer predisposition in Cockayne syndrome group A (Csa) mutant mice. DNA Repair (Amst). 2002;1:143-57 pubmed
    ..Strikingly, inactivation of one Xpc allele (encoding a component of the damage recognition complex involved in the global genome repair sub-pathway) in ..

Scientific Experts

More Information

Publications62

  1. Cheo D, Ruven H, Meira L, Hammer R, Burns D, Tappe N, et al. Characterization of defective nucleotide excision repair in XPC mutant mice. Mutat Res. 1997;374:1-9 pubmed
    ..In order to model this disease in mice a mutation in the mouse XPC gene was generated and used to replace a wild-type XPC allele in mouse embryonic stem cells by homologous ..
  2. Wijnhoven S, Hoogervorst E, de Waard H, van der Horst G, van Steeg H. Tissue specific mutagenic and carcinogenic responses in NER defective mouse models. Mutat Res. 2007;614:77-94 pubmed
    ..GG-NER defects in mice predispose to cancer, both spontaneous as well as UV-induced. As such these models (Xpa, Xpc and Xpe) recapitulate the human xeroderma pigmentosum (XP) syndrome...
  3. 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
    ..in mammalian global genome nucleotide excision repair (GG-NER) is performed by the xeroderma pigmentosum group C (XPC)/HR23B protein complex...
  4. Berg R, Ruven H, Sands A, de Gruijl F, Mullenders L. Defective global genome repair in XPC mice is associated with skin cancer susceptibility but not with sensitivity to UVB induced erythema and edema. J Invest Dermatol. 1998;110:405-9 pubmed
    ..In line with human data, both XPA and XPC knockout mice have been shown to have an increased susceptibility to UVB induced squamous cell carcinomas...
  5. Masutani C, Sugasawa K, Yanagisawa J, Sonoyama T, Ui M, Enomoto T, et al. 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. 1994;13:1831-43 pubmed
    ..All RAD23 derivatives share a ubiquitin-like N-terminus. The nature of the XP-C defect implies that the complex exerts a unique function in the genome-overall repair pathway which is important for prevention of skin cancer. ..
  6. de Boer J, van Steeg H, Berg R, Garssen J, de Wit J, van Oostrum C, et al. Mouse model for the DNA repair/basal transcription disorder trichothiodystrophy reveals cancer predisposition. Cancer Res. 1999;59:3489-94 pubmed
    ..These findings have important implications for the etiology of the human disorder and for the impact of NER on carcinogenesis. ..
  7. van der Pluijm I, Garinis G, Brandt R, Gorgels T, Wijnhoven S, Diderich K, et al. Impaired genome maintenance suppresses the growth hormone--insulin-like growth factor 1 axis in mice with Cockayne syndrome. PLoS Biol. 2007;5:e2 pubmed
    ..Importantly, wild-type mice exposed to a low dose of chronic genotoxic stress recapitulated this response, thereby pointing to a novel link between genome instability and the age-related decline of the somatotroph axis. ..
  8. Reis A, Cheo D, Meira L, Greenblatt M, Bond J, Nahari D, et al. Genotype-specific Trp53 mutational analysis in ultraviolet B radiation-induced skin cancers in Xpc and Xpc Trp53 mutant mice. Cancer Res. 2000;60:1571-9 pubmed
    ..the Trp53 gene from UVB radiation-induced skin cancers in Trp53+/+ and Trp53+/- mutant mice of all three possible Xpc genotypes...
  9. Shen H, Cheo D, Friedberg E, Storb U. The inactivation of the XP-C gene does not affect somatic hypermutation or class switch recombination of immunoglobulin genes. Mol Immunol. 1997;34:527-33 pubmed
    ..Furthermore, we found that Ig gene switch recombination also is normal in these mice. ..
  10. de Boer J, Hoeijmakers J. Cancer from the outside, aging from the inside: mouse models to study the consequences of defective nucleotide excision repair. Biochimie. 1999;81:127-37 pubmed
    ..This review will focus on data obtained thus far and discuss further utilization of the mouse mutants for unraveling some of the fascinating and medically relevant aspects associated with defects in NER and related processes. ..
  11. Ito S, Yamane M, Ohtsuka S, Niwa H. The C-terminal region of Xpc is dispensable for the transcriptional activity of Oct3/4 in mouse embryonic stem cells. FEBS Lett. 2014;588:1128-35 pubmed publisher
    ..It was reported that the Xpc DNA repair complex is involved in this process...
  12. Verhofstad N, Pennings J, Van Oostrom C, van Benthem J, Van Schooten F, van Steeg H, et al. Benzo(a)pyrene induces similar gene expression changes in testis of DNA repair proficient and deficient mice. BMC Genomics. 2010;11:333 pubmed publisher
    ..and in testis, and removal of these lesions is less efficient in nucleotide excision repair deficient Xpc-/- mice than in wild type mice...
  13. Rebel H, Kram N, Westerman A, Banus S, van Kranen H, de Gruijl F. Relationship between UV-induced mutant p53 patches and skin tumours, analysed by mutation spectra and by induction kinetics in various DNA-repair-deficient mice. Carcinogenesis. 2005;26:2123-30 pubmed
    ..e. xeroderma pigmentosum A (Xpa), Xpc and Cockayne syndrome B (Csb) and wild-type mice...
  14. Luo C, Sheng J, Hu M, Haluska F, Cui R, Xu Z, et al. Loss of ARF sensitizes transgenic BRAFV600E mice to UV-induced melanoma via suppression of XPC. Cancer Res. 2013;73:4337-48 pubmed publisher
    ..BRAF(V600E) and ARF deletion synergize to inhibit nucleotide excision repair by epigenetically repressing XPC and inhibiting the E2F4/DP1 complex...
  15. Tsai P, Nielen M, van der Horst G, Colenbrander B, Heesterbeek J, van Vlissingen J. The effect of DNA repair defects on reproductive performance in nucleotide excision repair (NER) mouse models: an epidemiological approach. Transgenic Res. 2005;14:845-57 pubmed
    ..in five Nucleotide Excision Repair (NER) mutant mouse models on a C57BL/6 genetic background, namely CSA, CSB, XPA, XPC [models for the human DNA repair disorders Cockayne Syndrome (CS) and xeroderma pigmentosum (XP), respectively] and ..
  16. Miccoli L, Burr K, Hickenbotham P, Friedberg E, Angulo J, Dubrova Y. The combined effects of xeroderma pigmentosum C deficiency and mutagens on mutation rates in the mouse germ line. Cancer Res. 2007;67:4695-9 pubmed
    ..at two expanded simple tandem repeat (ESTR) loci were studied in the germ line of xeroderma pigmentosum group C (Xpc) knockout mice defective in global genome nucleotide excision repair...
  17. Cheo D, Burns D, Meira L, Houle J, Friedberg E. Mutational inactivation of the xeroderma pigmentosum group C gene confers predisposition to 2-acetylaminofluorene-induced liver and lung cancer and to spontaneous testicular cancer in Trp53-/- mice. Cancer Res. 1999;59:771-5 pubmed
    ..of nucleotide excision repair in protection against chemical carcinogenesis in internal organs, we treated XPC mutant (XPC-/-) mice with 2-acetylaminofluorene and NOH-2-acetylaminofluorene...
  18. Ikehata H, Saito Y, Yanase F, Mori T, Nikaido O, Ono T. Frequent recovery of triplet mutations in UVB-exposed skin epidermis of Xpc-knockout mice. DNA Repair (Amst). 2007;6:82-93 pubmed
    Mutations of the Xpc gene cause a deficiency in global genome repair, a subpathway of nucleotide excision repair (NER), in mammalian cells...
  19. Nahari D, McDaniel L, Task L, Daniel R, Velasco Miguel S, Friedberg E. Mutations in the Trp53 gene of UV-irradiated Xpc mutant mice suggest a novel Xpc-dependent DNA repair process. DNA Repair (Amst). 2004;3:379-86 pubmed
    ..We previously developed a mouse model of skin cancer with mutations in the xeroderma pigmentosum group C gene (Xpc)...
  20. Yang G, Curley D, Bosenberg M, Tsao H. Loss of xeroderma pigmentosum C (Xpc) enhances melanoma photocarcinogenesis in Ink4a-Arf-deficient mice. Cancer Res. 2007;67:5649-57 pubmed
    ..development, we set out to test the hypothesis that abrogation of NER, by deletion of the xeroderma pigmentosum C (Xpc) gene, will heighten melanoma photocarcinogenesis in an Ink4a-Arf-deficient background...
  21. Raad H, Serrano Sanchez M, Harfouche G, Mahfouf W, Bortolotto D, Bergeron V, et al. NADPH Oxidase-1 Plays a Key Role in Keratinocyte Responses to UV Radiation and UVB-Induced Skin Carcinogenesis. J Invest Dermatol. 2017;137:1311-1321 pubmed publisher
    ..Our results show that inhibition of NOX1 activation could be a promising target for the prevention and treatment of UVB-induced skin cancer in nucleotide excision repair-proficient and -deficient patients. ..
  22. Ananthaswamy H, Ouhtit A, Evans R, Gorny A, Khaskina P, Sands A, et al. Persistence of p53 mutations and resistance of keratinocytes to apoptosis are associated with the increased susceptibility of mice lacking the XPC gene to UV carcinogenesis. Oncogene. 1999;18:7395-8 pubmed
    ..xeroderma pigmentosum (XP) patients, transgenic mice lacking nucleotide excision repair (NER) genes such as XPA and XPC are extremely susceptible to ultraviolet (UV)-induced skin cancer...
  23. Rainey R, Ng S, Llamas J, van der Horst G, Segil N. Mutations in Cockayne Syndrome-Associated Genes (Csa and Csb) Predispose to Cisplatin-Induced Hearing Loss in Mice. J Neurosci. 2016;36:4758-70 pubmed publisher
    ..and group B (Csb(-/-)) mice are hypersensitive to cisplatin, in contrast to global genome repair-deficient Xpc(-/-) mice, both in vitro and in vivo We show that sensory hair cells in Csa(-/-) and Csb(-/-) mice fail to remove ..
  24. Hosseini M, Mahfouf W, Serrano Sanchez M, Raad H, Harfouche G, Bonneu M, et al. Premature skin aging features rescued by inhibition of NADPH oxidase activity in XPC-deficient mice. J Invest Dermatol. 2015;135:1108-1118 pubmed publisher
    ..expression, β-galactosidase activity, and reactive oxygen species, which increase with age, were higher in young Xpc(-/-) mice than in young Xpc(+/+) ones...
  25. Berg R, Rebel H, van der Horst G, van Kranen H, Mullenders L, van Vloten W, et al. Impact of global genome repair versus transcription-coupled repair on ultraviolet carcinogenesis in hairless mice. Cancer Res. 2000;60:2858-63 pubmed
    ..GGR for UV effects on the skin, we have used hairless knockout mouse strain lacking either TCR (CSB -/-) or GGR (XPC -/-)...
  26. Tsaalbi Shtylik A, Verspuy J, Jansen J, Rebel H, Carlée L, van der Valk M, et al. Error-prone translesion replication of damaged DNA suppresses skin carcinogenesis by controlling inflammatory hyperplasia. Proc Natl Acad Sci U S A. 2009;106:21836-41 pubmed publisher
    ..In conclusion, error-prone TLS suppresses tumor-promoting activities of UV light, thereby controlling skin carcinogenesis. ..
  27. Uehara Y, Ikehata H, Furuya M, Kobayashi S, He D, Chen Y, et al. XPC is involved in genome maintenance through multiple pathways in different tissues. Mutat Res. 2009;670:24-31 pubmed publisher
    In an attempt to evaluate the role of the Xpc gene in maintaining genomic stability in vivo under normal conditions, the age-dependent accumulation of spontaneous mutations in different tissues was analyzed in Xpc-deficient lacZ-..
  28. Cattoglio C, Zhang E, Grubisic I, Chiba K, Fong Y, Tjian R. Functional and mechanistic studies of XPC DNA-repair complex as transcriptional coactivator in embryonic stem cells. Proc Natl Acad Sci U S A. 2015;112:E2317-26 pubmed publisher
    ..We recently identified the DNA-repair complex xeroderma pigmentosum C (XPC)-RAD23B-CETN2 as a stem cell coactivator (SCC) required for OCT4/SOX2 transcriptional activation...
  29. Jansen J, Tsaalbi Shtylik A, Hendriks G, Gali H, Hendel A, Johansson F, et al. Separate domains of Rev1 mediate two modes of DNA damage bypass in mammalian cells. Mol Cell Biol. 2009;29:3113-23 pubmed publisher
    ..These gaps are inducers of DNA damage signaling leading to an irreversible G(2) arrest. Our results corroborate a model in which Rev1-mediated DNA damage bypass at postreplicative gaps quenches irreversible DNA damage responses. ..
  30. Zattra E, Coleman C, Arad S, Helms E, Levine D, Bord E, et al. Polypodium leucotomos extract decreases UV-induced Cox-2 expression and inflammation, enhances DNA repair, and decreases mutagenesis in hairless mice. Am J Pathol. 2009;175:1952-61 pubmed publisher
    ..To evaluate photoprotective effects of Polypodium leucotomos extract (PL), hairless Xpc(+/-) mice were fed for 10 days with PL (300 mg/kg) or vehicle then UV-irradiated, once...
  31. Stout G, Oosten M, Acherrat F, Wit J, Vermeij W, Mullenders L, et al. Selective DNA damage responses in murine Xpa-/-, Xpc-/- and Csb-/- keratinocyte cultures. DNA Repair (Amst). 2005;4:1337-44 pubmed
    ..In irradiated Xpc(-/-) keratinocytes (GG-NER deficient) the loss of replicating S-phase cells was associated with a gradual build-up ..
  32. Klein J, Beems R, Zwart P, Hamzink M, Zomer G, van Steeg H, et al. Intestinal toxicity and carcinogenic potential of the food mutagen 2-amino-1-methyl-6-phenylimidazo[4,5-b]pyridine (PhIP) in DNA repair deficient XPA-/- mice. Carcinogenesis. 2001;22:619-26 pubmed
    ..Furthermore, DNA repair deficient XPC(-/-) mice, which are still able to repair DNA damage in actively transcribed genes, did not display any toxicity ..
  33. 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
    ..and cell cycle progression in the 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)...
  34. Kölgen W, van Steeg H, van der Horst G, Hoeijmakers J, van Vloten W, de Gruijl F, et al. Association of transcription-coupled repair but not global genome repair with ultraviolet-B-induced Langerhans cell depletion and local immunosuppression. J Invest Dermatol. 2003;121:751-6 pubmed
    ..b>XPC mice, which were defective in global genome repair, were as sensitive to ultraviolet-B-induced local suppression of ..
  35. Bergink S, Toussaint W, Luijsterburg M, Dinant C, Alekseev S, Hoeijmakers J, et al. Recognition of DNA damage by XPC coincides with disruption of the XPC-RAD23 complex. J Cell Biol. 2012;196:681-8 pubmed publisher
    ..deoxyribonucleic acid (DNA) lesions by the global genome nucleotide excision repair subpathway is performed by the XPC-RAD23-CEN2 complex...
  36. Meira L, Cheo D, Hammer R, Burns D, Reis A, Friedberg E. Genetic interaction between HAP1/REF-1 and p53. Nat Genet. 1997;17:145 pubmed
  37. Melis J, Kuiper R, Zwart E, Robinson J, Pennings J, van Oostrom C, et al. Slow accumulation of mutations in Xpc-/- mice upon induction of oxidative stress. DNA Repair (Amst). 2013;12:1081-6 pubmed publisher
    b>XPC is one of the key DNA damage recognition proteins in the global genome repair route of the nucleotide excision repair (NER) pathway...
  38. Fischer J, Kumar M, Day T, Hardy T, Hamilton S, Besch Williford C, et al. The Xpc gene markedly affects cell survival in mouse bone marrow. Mutagenesis. 2009;24:309-16 pubmed publisher
    The XPC protein (encoded by the xeroderma pigmentosum Xpc gene) is a key DNA damage recognition factor that is required for global genomic nucleotide excision repair (G-NER)...
  39. Boonstra A, van Oudenaren A, Baert M, van Steeg H, Leenen P, van der Horst G, et al. Differential ultraviolet-B-induced immunomodulation in XPA, XPC, and CSB DNA repair-deficient mice. J Invest Dermatol. 2001;117:141-6 pubmed
    ..this study the immunologic consequences of specific nucleotide excision repair defects in three mouse models, XPA, XPC, and CSB mutant mice, were investigated...
  40. Li L, Peterson C, Legerski R. Sequence of the mouse XPC cDNA and genomic structure of the human XPC gene. Nucleic Acids Res. 1996;24:1026-8 pubmed
    The full length-mouse XPC cDNA contains a 2703 bp orf which encodes a polypeptide containing 900 amino acids. Overall, there is 75% identity in nucleotide sequence and 73% identity in amino acid sequence between mouse and human genes...
  41. Jarrett S, Wolf Horrell E, Christian P, Vanover J, Boulanger M, Zou Y, et al. PKA-mediated phosphorylation of ATR promotes recruitment of XPA to UV-induced DNA damage. Mol Cell. 2014;54:999-1011 pubmed publisher
    ..This study mechanistically links cAMP-PKA signaling to NER and illustrates potential benefits of cAMP pharmacological rescue to reduce UV mutagenesis in MC1R-defective, melanoma-susceptible individuals. ..
  42. Stout G, Blasco M. Telomere length and telomerase activity impact the UV sensitivity syndrome xeroderma pigmentosum C. Cancer Res. 2013;73:1844-54 pubmed publisher
    ..we investigated a hypothesized role for telomerase and telomere dysfunction in the pathobiology of XP by comparing Xpc(-/-)-mutant mice and Xpc(-/-)G1-G3Terc(-/-) double-mutant mice and exposed them to UV radiation...
  43. Hollander M, Philburn R, Patterson A, Velasco Miguel S, Friedberg E, Linnoila R, et al. Deletion of XPC leads to lung tumors in mice and is associated with early events in human lung carcinogenesis. Proc Natl Acad Sci U S A. 2005;102:13200-5 pubmed
    ..We identify XPC and Gadd45a as genes within each of these regions involved in lung tumor initiation and progression, respectively...
  44. Melis J, Speksnijder E, Kuiper R, Salvatori D, Schaap M, Maas S, et al. Detection of genotoxic and non-genotoxic carcinogens in Xpc(-/-)p53(+/-) mice. Toxicol Appl Pharmacol. 2013;266:289-97 pubmed publisher
    ..We now show that the Xpc*p53 mouse model preserves all the beneficial traits of the Xpa*p53 model for sub-chronic carcinogen identification ..
  45. Nijhof J, van Pelt C, Mulder A, Mitchell D, Mullenders L, de Gruijl F. Epidermal stem and progenitor cells in murine epidermis accumulate UV damage despite NER proficiency. Carcinogenesis. 2007;28:792-800 pubmed
    ..Accumulation of 6-4PP as well as CPD did, however, occur in basal cells in the epidermis of DNA repair-deficient Xpc-/- mice...
  46. Stout G, Westdijk D, Calkhoven D, Pijper O, Backendorf C, Willemze R, et al. Epidermal transit of replication-arrested, undifferentiated keratinocytes in UV-exposed XPC mice: an alternative to in situ apoptosis. Proc Natl Acad Sci U S A. 2005;102:18980-5 pubmed
    ..In Xpc(-/-) mice deficient in global genome nucleotide excision repair (GGR), a cell-cycle arrest of epidermal cells in ..
  47. Schenten D, Gerlach V, Guo C, Velasco Miguel S, Hladik C, White C, et al. DNA polymerase kappa deficiency does not affect somatic hypermutation in mice. Eur J Immunol. 2002;32:3152-60 pubmed
    ..However, pol kappa-deficient embryonic fibroblasts are abnormally sensitive to killing following exposure to ultraviolet (UV) radiation, suggesting a role of pol kappa in translesion DNA synthesis. ..
  48. Lynch H, Fusaro R, Johnson J. Xeroderma pigmentosum. Complementation group C and malignant melanoma. Arch Dermatol. 1984;120:175-9 pubmed
  49. Revet I, Feeney L, Tang A, Huang E, Cleaver J. Dysmyelination not demyelination causes neurological symptoms in preweaned mice in a murine model of Cockayne syndrome. Proc Natl Acad Sci U S A. 2012;109:4627-32 pubmed publisher
    ..Mice mutated in Csb were crossed with mice lacking Xpc (Xp-c(-/-)), the global genome repair gene, to enhance the pathological symptoms. These Cs-b(m/m)...
  50. Kawasumi M, Lemos B, Bradner J, Thibodeau R, Kim Y, Schmidt M, et al. Protection from UV-induced skin carcinogenesis by genetic inhibition of the ataxia telangiectasia and Rad3-related (ATR) kinase. Proc Natl Acad Sci U S A. 2011;108:13716-21 pubmed publisher
    ..we generated transgenic mice with diminished ATR function in skin and crossed them into a UV-sensitive background, Xpc(-/-). Unlike caffeine, this genetic approach was selective and had no effect on ATM activation...
  51. Dominguez Brauer C, Chen Y, Brauer P, Pimkina J, Raychaudhuri P. ARF stimulates XPC to trigger nucleotide excision repair by regulating the repressor complex of E2F4. EMBO Rep. 2009;10:1036-42 pubmed publisher
    ..with increased expression of the damaged-DNA recognition protein xeroderma pigmentosum, complementation group C (XPC)...
  52. Friedberg E, Bond J, Burns D, Cheo D, Greenblatt M, Meira L, et al. Defective nucleotide excision repair in xpc mutant mice and its association with cancer predisposition. Mutat Res. 2000;459:99-108 pubmed
    ..We have generated mouse strains defective in the Xpc gene, which is required for nucleotide excision repair (NER) of DNA...
  53. Meira L, Devaraj S, Kisby G, Burns D, Daniel R, Hammer R, et al. Heterozygosity for the mouse Apex gene results in phenotypes associated with oxidative stress. Cancer Res. 2001;61:5552-7 pubmed