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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XPC-cDNA
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Relation to Proband
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proband
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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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| PDL at Freeze |
5.01 |
| Passage Frozen |
3 |
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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 |
Line XP4PA-SE2; derived from XP4PA-SV-EB (GM15983) by stable transfection with XPC-cDNA using the plasmid pXPC3; fully corrected |
| Hung KF, Sidorova JM, Nghiem P, Kawasumi M, The 6-4 photoproduct is the trigger of UV-induced replication blockage and ATR activation Proceedings of the National Academy of Sciences of the United States of America: 2020 |
| PubMed ID: 32444488 |
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| Enoiu M, Jiricny J, Schärer OD, Repair of cisplatin-induced DNA interstrand crosslinks by a replication-independent pathway involving transcription-coupled repair and translesion synthesis Nucleic acids research40:8953-64 2012 |
| PubMed ID: 22810206 |
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| Emmert S, Kobayashi N, Khan SG, Kraemer KH, The xeroderma pigmentosum group C gene leads to selective repair of cyclobutane
pyrimidine dimers rather than 6-4 photoproducts. Proc Natl Acad Sci U S A.97(5):2151-6 2000 |
| PubMed ID: 10681431 |
| Passage Frozen |
3 |
| Split Ratio |
1:6 |
| Temperature |
37 C |
| Percent CO2 |
5% |
| Percent O2 |
10% |
| Medium |
Dulbecco Modified Eagles Medium (high glucose) with 2mM L-glutamine or equivalent |
| Serum |
15% fetal bovine serum Not inactivated |
| Substrate |
Commercially-treated plastic |
| Supplement |
- |
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