GM15876
Fibroblast from Skin, Unspecified
Description:
XERODERMA PIGMENTOSUM, COMPLEMENTATION GROUP A; XPA
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Repository
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NIGMS Human Genetic Cell Repository
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| Subcollection |
Heritable Diseases
Engineered Cell Cultures |
| Class |
Repair Defective and Chromosomal Instability Syndromes |
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Biopsy Source
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Unspecified
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Cell Type
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Fibroblast
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Tissue Type
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Skin
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Transformant
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Simian Virus 40
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Sample Source
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Fibroblast from Skin, Unspecified
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Race
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Asian
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Family Member
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1
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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 |
6.9 |
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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 |
The cell line (also known as XP20S-pCAH19WS) is a derivative of the SV40 Transformed and immortalized xeroderma pigmentosum complementation group A fibroblast XP20S (GM04312), which was subsequently transfected with the full length cDNA of the XPAC gene to correct the abnormal phenotype; GM15876A shows normal UV sensitivity, judged by survival of UV damaged Shuttle vector plasmid, restoration of transcription of a UV damaged CAT expression vector, and by a return to the normal spectrum of shuttle vector mutations found in control cells (Levy et al, Carcinogenesis 16:1557-63,1995); the full length XPAC cDNA is stably expressed using the cytomegalovirus enhancer and chicken B-actin promoter (Miyamoto et al, J Biol Chem 267:12182-87,1992), and the XPA protein in GM15876A is over expressed by a factor of 14 compared with the normal, repair-competent SV40 Transformed GM00637 (Levy et al, Carcinogenesis 16:15757-63,1995); the corrected phenotype of GM15876A is stable; selection for the XPAC gene is not required |
| Sarmini L, Meabed M, Emmanouil E, Atsaves G, Robeska E, Karwowski BT, Campalans A, Gimisis T, Khobta A, Requirement of transcription-coupled nucleotide excision repair for the removal of a specific type of oxidatively induced DNA damage Nucleic acids research: 2023 |
| PubMed ID: 37026475 |
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| Kitsera N, Rodriguez-Alvarez M, Emmert S, Carell T, Khobta A, Nucleotide excision repair of abasic DNA lesions Nucleic acids research: 2019 |
| PubMed ID: 31226203 |
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| Kong YW, Dreaden EC, Morandell S, Zhou W, Dhara SS, Sriram G, Lam FC, Patterson JC, Quadir M, Dinh A, Shopsowitz KE, Varmeh S, Yilmaz ÖH, Lippard SJ, Reinhardt HC, Hemann MT, Hammond PT, Yaffe MB, Enhancing chemotherapy response through augmented synthetic lethality by co-targeting nucleotide excision repair and cell-cycle checkpoints Nature communications11:4124 2018 |
| PubMed ID: 32807787 |
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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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| Jowsey PA, Williams FM, Blain PG, DNA damage responses in cells exposed to sulphur mustard Toxicology letters209:1-10 2011 |
| PubMed ID: 22119920 |
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| Yoder K, Sarasin A, Kraemer K, McIlhatton M, Bushman F, Fishel R, The DNA repair genes XPB and XPD defend cells from retroviral infection Proceedings of the National Academy of Sciences of the United States of America103:4622-7 2006 |
| PubMed ID: 16537383 |
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| Reynolds M, Peterson E, Quievryn G, Zhitkovich A, Human Nucleotide Excision Repair Efficiently Removes Chromium-DNA Phosphate Adducts and Protects Cells against Chromate Toxicity. J Biol Chem279(29):30419-24 2004 |
| PubMed ID: 15087443 |
| Split Ratio |
1:3 |
| Temperature |
37 C |
| Percent CO2 |
10% |
| Percent O2 |
AMBIENT |
| Medium |
Dulbecco Modified Eagles Medium (high glucose) with 2mM L-glutamine or equivalent |
| Serum |
15% fetal bovine serum Not inactivated |
| Supplement |
- |
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