Description:
XERODERMA PIGMENTOSUM, COMPLEMENTATION GROUP C; XPC
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Repository
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NIGMS Human Genetic Cell Repository
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| Subcollection |
Heritable Diseases |
| Class |
Disorders of Nucleotide and Nucleic Acid Metabolism |
| Class |
Repair Defective and Chromosomal Instability Syndromes |
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Cell Type
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Fibroblast
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Transformant
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Untransformed
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Race
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White
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Family Member
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4
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Relation to Proband
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brother
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Confirmation
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Clinical summary/Case history
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Species
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Homo sapiens
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Common Name
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Human
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Remarks
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| Passage Frozen |
8 |
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| IDENTIFICATION OF SPECIES OF ORIGIN |
Species of Origin Confirmed by Nucleoside Phosphorylase, Glucose-6-Phosphate Dehydrogenase, and Lactate Dehydrogenase Isoenzyme Electrophoresis |
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| Remarks |
XP2BE; ATCC CRL 1166; 19% of normal UV induced unscheduled DNA synthesis; 3 affected brothers |
| Fishel ML, Gamcsik MP, Delaney SM, Zuhowski EG, Maher VM, Karrison T, Moschel RC, Egorin MJ, Dolan ME, Role of glutathione and nucleotide excision repair in modulation of cisplatin activity with O6-benzylguanine Cancer chemotherapy and pharmacology55:333-42 2004 |
| PubMed ID: 15723259 |
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| Evans MK, Robbins JH, Ganges MB, Tarone RE, Nairn RS, Bohr VA, Gene-specific DNA repair in xeroderma pigmentosum complementation groups A, C, D, and F. Relation to cellular survival and clinical features. J Biol Chem268:4839-47 1993 |
| PubMed ID: 8444862 |
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| Evans MK, Taffe BG, Harris CC, Bohr VA, DNA strand bias in the repair of the p53 gene in normal human and xeroderma pigmentosum group C fibroblasts. Cancer Res53:5377-81 1993 |
| PubMed ID: 8221675 |
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| Colicos MA, Haj-Ahmad Y, Valerie K, Henderson EE, Rainbow AJ, Construction of a recombinant adenovirus containing the denV gene from bacteriophage T4 which can partially restore the DNA repair deficiency in xeroderma pigmentosum fibroblasts. Carcinogenesis12:249-55 1991 |
| PubMed ID: 1704821 |
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| Tyrrell RM, Amaudruz F, Evidence for two independent pathways of biologically effective excision repair from its rate and extent in cells cultured from sun-sensitive humans. Cancer Res47:3725-8 1987 |
| PubMed ID: 3109732 |
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| Cleaver JE, DNA repair deficiencies and cellular senescence are unrelated in xeroderma pigmentosum cell lines. Mech Ageing Dev27:189-96 1984 |
| PubMed ID: 6492896 |
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| Cleaver JE, Rapid complementation method for classifying excision repair-defective xeroderma pigmentosum cell strains. Somatic Cell Genet8:801-10 1982 |
| PubMed ID: 7163956 |
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| Kraemer KH, Buchanan JK, Stinson SF, Semiautomated autoradiographic measurement of DNA repair in normal and xeroderma pigmentosum cultured human fibroblasts. In Vitro16:609-15 1980 |
| PubMed ID: 7409831 |
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| Maher VM, Dorney DJ, Mendrala AL, Konze-Thomas B, McCormick JJ, DNA excision-repair processes in human cells can eliminate the cytotoxic and
mutagenic consequences of ultraviolet irradiation. Mutat Res62(2):311-23 1979 |
| PubMed ID: 503098 |
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| McCaw BA, Dipple A, Young S, Roberts JJ, Excision of hydrocarbon-DNA adducts and consequent cell survival in normal and
repair defective human cells. Chem Biol Interact22(2-3):139-51 1978 |
| PubMed ID: 699169 |
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| Kraemer KH, De Weerd-Kastelein EA, Robbins JH, Keijzer W, Barrett SF, Petinga RA, Bootsma D, Five complementation groups in xeroderma pigmentosum. Mutat Res33:327-40 1975 |
| PubMed ID: 1243579 |
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| Maher VM, Birch N, Otto JR, MacCormick JJ, Cytotoxicity of carcinogenic aromatic amides in normal and xeroderma pigmentosum
fibroblasts with different DNA repair capabilities. J Natl Cancer Inst54(6):1287-94 1975 |
| PubMed ID: 1133846 |
| Passage Frozen |
8 |
| Split Ratio |
1:2 |
| Temperature |
37 C |
| Percent CO2 |
5% |
| Medium |
Eagle's Minimum Essential Medium with Earle's salts and non-essential amino acids with 2mM L-glutamine or equivalent |
| Serum |
15% fetal bovine serum Not inactivated |
| Substrate |
None specified |
| Subcultivation Method |
trypsin-EDTA |
| Supplement |
- |
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