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Cellosaurus XP3BR (CVCL_M286)

[Text version]
Cell line name XP3BR
Synonyms Xeroderma Pigmentosum 3 BRighton; GM03616; GM3616
Accession CVCL_M286
Resource Identification Initiative To cite this cell line use: XP3BR (RRID:CVCL_M286)
Comments Senescence: Senesces at 17 PDL (PubMed=6492896).
Caution: Could be identical to IMG-990 XP3Br2 (Cellosaurus=CVCL_IM57).
Derived from site: In situ; Skin; UBERON=UBERON_0002097.
Cell type: Fibroblast of skin; CL=CL_0002620.
Sequence variations
  • Mutation; HGNC; 3437; ERCC5; Simple; p.Asp499Ilefs*23 (c.1494delA); ClinVar=VCV000016575; Zygosity=Heterozygous (PubMed=11841555).
  • Mutation; HGNC; 3437; ERCC5; Simple; p.Lys917Asnfs*64 (c.2751delA); ClinVar=VCV000016576; Zygosity=Heterozygous (PubMed=11841555).
Disease Xeroderma pigmentosum, complementation group G (NCIt: C3969)
Xeroderma pigmentosum (ORDO: Orphanet_910)
Species of origin Homo sapiens (Human) (NCBI Taxonomy: 9606)
Originate from same individual CVCL_ZP69 ! XP3BR LCL
Sex of cell Male
Age at sampling 6-7Y
Category Finite cell line
Publications

PubMed=11219864; DOI=10.1093/carcin/1.9.745
Arlett C.F., Harcourt S.A., Lehmann A.R., Stevens S., Ferguson-Smith M.A., Morley W.N.
Studies on a new case of xeroderma pigmentosum (XP3BR) from complementation group G with cellular sensitivity to ionizing radiation.
Carcinogenesis 1:745-751(1980)

PubMed=7067035; DOI=10.1093/carcin/3.1.33
Teo I.A., Arlett C.F.
The response of a variety of human fibroblast cell strains to the lethal effects of alkylating agents.
Carcinogenesis 3:33-37(1982)

PubMed=6307543; DOI=10.1093/carcin/4.8.1075
Teo I.A., Lehmann A.R., Muller R., Rajewsky M.F.
Similar rate of O6-ethylguanine elimination from DNA in normal human fibroblast and xeroderma pigmentosum cell strains not transformed by SV40.
Carcinogenesis 4:1075-1077(1983)

PubMed=6600729; DOI=10.1080/09553008314550171
Arlett C.F., Priestley A.
Defective recovery from potentially lethal damage in some human fibroblast cell strains.
Int. J. Radiat. Biol. Relat. Stud. Phys. Chem. Med. 43:157-167(1983)

PubMed=6492896; DOI=10.1016/0047-6374(84)90044-7
Cleaver J.E.
DNA repair deficiencies and cellular senescence are unrelated in xeroderma pigmentosum cell lines.
Mech. Ageing Dev. 27:189-196(1984)

PubMed=2973075; DOI=10.2307/3577479
Fertil B., Deschavanne P.J., Debieu D., Malaise E.-P.
Correlation between PLD repair capacity and the survival curve of human fibroblasts in exponential growth phase: analysis in terms of several parameters.
Radiat. Res. 116:74-88(1988)

PubMed=1702221; DOI=10.1073/pnas.87.24.9908; PMCID=PMC55283
Satokata I., Tanaka K., Miura N., Miyamoto I., Satoh Y., Kondo S., Okada Y.
Characterization of a splicing mutation in group A xeroderma pigmentosum.
Proc. Natl. Acad. Sci. U.S.A. 87:9908-9912(1990)

PubMed=2175267; DOI=10.1016/0014-4827(90)90012-Y
Klein B., Pastink A., Odijk H., Westerveld A., van der Eb A.J.
Transformation and immortalization of diploid xeroderma pigmentosum fibroblasts.
Exp. Cell Res. 191:256-262(1990)

CLPUB00447
Mulivor R.A., Suchy S.F.
1992/1993 catalog of cell lines. NIGMS human genetic mutant cell repository. 16th edition. October 1992.
(In misc. document) Institute for Medical Research (Camden, N.J.) NIH 92-2011; pp.1-918; National Institutes of Health; Bethesda; USA (1992)

PubMed=11841555; DOI=10.1046/j.0022-202x.2001.01673.x
Lalle P., Nouspikel T., Constantinou A., Thorel F., Clarkson S.G.
The founding members of xeroderma pigmentosum group G produce XPG protein with severely impaired endonuclease activity.
J. Invest. Dermatol. 118:344-351(2002)

PubMed=15572672; DOI=10.1128/mcb.24.24.10670-10680.2004; PMCID=PMC533987
Thorel F., Constantinou A., Dunand-Sauthier I., Nouspikel T., Lalle P., Raams A., Jaspers N.G.J., Vermeulen W., Shivji M.K.K., Wood R.D., Clarkson S.G.
Definition of a short region of XPG necessary for TFIIH interaction and stable recruitment to sites of UV damage.
Mol. Cell. Biol. 24:10670-10680(2004)

PubMed=18079351; DOI=10.1259/bjr/27072321
Arlett C.F., Green M.H.L., Rogers P.B., Lehmann A.R., Plowman P.N.
Minimal ionizing radiation sensitivity in a large cohort of xeroderma pigmentosum fibroblasts.
Br. J. Radiol. 81:51-58(2008)

PubMed=23370536; DOI=10.1038/jid.2013.54
Schafer A., Schubert S., Gratchev A., Seebode C., Apel A., Laspe P., Hofmann L., Ohlenbusch A., Mori T., Kobayashi N., Schurer A., Schon M.P., Emmert S.
Characterization of three XPG-defective patients identifies three missense mutations that impair repair and transcription.
J. Invest. Dermatol. 133:1841-1849(2013)

Cross-references
Cell line collections (Providers) Coriell; GM03616
Cell line databases/resources CLO; CLO_0017187
Encyclopedic resources Wikidata; Q54838159
Entry history
Entry creation05-Nov-2013
Last entry update29-Jun-2023
Version number17