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Cellosaurus CS1AN (CVCL_L471)

Cell line name CS1AN
Synonyms Cockayne Syndrome 1 ANn Arbor; UM106; UM 106; GM00739; GM-739; GM 739; GM739; GM00739A
Accession CVCL_L471
Resource Identification Initiative To cite this cell line use: CS1AN (RRID:CVCL_L471)
Comments Population: Caucasian.
Senescence: Senesces at 22 PDL (PubMed=6492896).
Doubling time: 50 hours (PubMed=8609460).
Omics: Transcriptome analysis by RNAseq.
Derived from site: In situ; Skin; UBERON=UBERON_0002097.
Cell type: Fibroblast of skin; CL=CL_0002620.
Sequence variations
  • Mutation; HGNC; 3438; ERCC6; Simple; p.Lys337Ter (c.1009A>T) (A1088T); ClinVar=VCV000550722; Zygosity=Heterozygous (PubMed=1339317).
  • Mutation; HGNC; 3438; ERCC6; Simple; p.Arg857Ter (c.2569C>T); ClinVar=VCV000553383; Zygosity=Heterozygous (PubMed=1339317).
Disease Cockayne syndrome type B (NCIt: C135726)
Cockayne syndrome (ORDO: Orphanet_191)
Species of origin Homo sapiens (Human) (NCBI Taxonomy: 9606)
Hierarchy Children:
CVCL_L472 (CS1AN-S3-G2)CVCL_A0JU (IUFi001-A)
Sex of cell Female
Age at sampling 3Y
Category Finite cell line
STR profile Source(s): PubMed=34271225

Penta D10,15
Penta E7,11

Run an STR similarity search on this cell line

Coriell L.L., Greene A.E.
The human genetic mutant cell repository: list of genetic variants, chromosomal aberrations and normal cell cultures submitted to the repository. 4th edition. October 1977.
(In) Institute for Medical Research (Camden, N.J.); pp.1-171; National Institutes of Health; Bethesda (1977)

Lehmann A.R., Kirk-Bell S., Mayne L.V.
Abnormal kinetics of DNA synthesis in ultraviolet light-irradiated cells from patients with Cockayne's syndrome.
Cancer Res. 39:4237-4241(1979)

PubMed=431551; DOI=10.1016/0027-5107(79)90194-5
Wade M.H., Chu E.H.-Y.
Effects of DNA damaging agents on cultured fibroblasts derived from patients with Cockayne syndrome.
Mutat. Res. 59:49-60(1979)

PubMed=7360141; DOI=10.1016/0027-5107(80)90180-3
Marshall R.R., Arlett C.F., Harcourt S.A., Broughton B.C.
Increased sensitivity of cell strains from Cockayne's syndrome to sister-chromatid-exchange induction and cell killing by UV light.
Mutat. Res. 69:107-112(1980)

PubMed=7264357; DOI=10.1111/1523-1747.ep12482447
Fujiwara Y., Ichihashi M., Kano Y., Goto K., Shimizu K.
A new human photosensitive subject with a defect in the recovery of DNA synthesis after ultraviolet-light irradiation.
J. Invest. Dermatol. 77:256-263(1981)

PubMed=7301938; DOI=10.1111/j.1751-1097.1981.tb09049.x
Day R.S. III, Ziolkowski C.H.J., DiMattina M.
Decreased host cell reactivation of UV-irradiated adenovirus 5 by fibroblasts from Cockayne's syndrome patients.
Photochem. Photobiol. 34:603-607(1981)

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)

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

PubMed=1339317; DOI=10.1016/0092-8674(92)90390-x
Troelstra C., van Gool A., de Wit J., Vermeulen W., Bootsma D., Hoeijmakers J.H.J.
ERCC6, a member of a subfamily of putative helicases, is involved in Cockayne's syndrome and preferential repair of active genes.
Cell 71:939-953(1992)

PubMed=8609460; DOI=10.1080/095530096146075
Alsbeih G.A., Fertil B., Arlett C.F., Malaise E.-P.
High split-dose recovery in hypersensitive human fibroblasts: a case of induced radioresistance?
Int. J. Radiat. Biol. 69:225-239(1996)

PubMed=12665480; DOI=10.1096/fj.02-0851com
Tuo J.-S., Jaruga P., Rodriguez H., Bohr V.A., Dizdaroglu M.
Primary fibroblasts of Cockayne syndrome patients are defective in cellular repair of 8-hydroxyguanine and 8-hydroxyadenine resulting from oxidative stress.
FASEB J. 17:668-674(2003)

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=19329487; DOI=10.1073/pnas.0902113106
Nardo T., Oneda R., Spivak G., Vaz B., Mortier L., Thomas P., Orioli D., Laugel V., Stary A., Hanawalt P.C., Sarasin A., Stefanini M.
A UV-sensitive syndrome patient with a specific CSA mutation reveals separable roles for CSA in response to UV and oxidative DNA damage.
Proc. Natl. Acad. Sci. U.S.A. 106:6209-6214(2009)

PubMed=34271225; DOI=10.1016/j.scr.2021.102456
Martins S., Hacheney I., Teichweyde N., Hildebrandt B., Krutmann J., Rossi A.
Generation of an induced pluripotent stem cell line (IUFi001) from a Cockayne syndrome patient carrying a mutation in the ERCC6 gene.
Stem Cell Res. 55:102456-102456(2021)

Cell line collections (Providers) Coriell; GM00739
Cell line databases/resources CLO; CLO_0028821
Encyclopedic resources Wikidata; Q54836433
Gene expression databases GEO; GSM3738754
GEO; GSM3738755
Entry history
Entry creation06-May-2013
Last entry update29-Jun-2023
Version number21