AG05229
Fibroblast from Skin, Thigh
Description:
WERNER SYNDROME; WRN REPLICATION FOCUS-FORMING ACTIVITY 1, INCLUDED; FFA1, INCLUDED
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Repository
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NIA Aging Cell Culture Repository
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| Subcollection |
Heritable Diseases |
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Biopsy Source
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Thigh
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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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Untransformed
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Sample Source
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Fibroblast from Skin, Thigh
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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 |
4.25 |
| 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 and by Chromosome Analysis |
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| Remarks |
The donor had features of short stature, gray hair, hyperpigmentation of skin, juvenile bilateral cataracts, atrophic skin and subcutaneous tissue, and hypogonadism. Diabetes was not detected at time of biopsy. The family history is negative and the parents are nonconsanguineous. The culture was initiated on 10/14/81 from explants of minced skin tissue which had been frozen after biopsy. The cell morphology is fibroblast-like. Same donor as AG12799. The culture is a mosaic with karyotype: 46,XY/46,XY,t(12;12)(q13;q24)/46,XY, -4,-21,t(5;8)(q15;q22),+2mar; balanced; 37%/13%/13% with 37% of cells examined showing random chromosomal abnormalities. The legacy karyotype description shown in this Remark may not be representative of the current available product. |
| Della Valle F, Reddy P, Yamamoto M, Liu P, Saera-Vila A, Bensaddek D, Zhang H, Prieto Martinez J, Abassi L, Celii M, Ocampo A, Nuñez Delicado E, Mangiavacchi A, Aiese Cigliano R, Rodriguez Esteban C, Horvath S, Izpisua Belmonte JC, Orlando V, LINE-1 RNA causes heterochromatin erosion and is a target for amelioration of senescent phenotypes in progeroid syndromes Science translational medicine14:eabl6057 2022 |
| PubMed ID: 35947677 |
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| Kang SM, Yoon MH, Lee SJ, Ahn J, Yi SA, Nam KH, Park S, Woo TG, Cho JH, Lee J, Ha NC, Park BJ, Human WRN is an intrinsic inhibitor of progerin, abnormal splicing product of lamin A Scientific reports11:9122 2020 |
| PubMed ID: 33907225 |
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| Oganesian L, Karlseder J, 5' C-rich telomeric overhangs are an outcome of rapid telomere truncation events DNA repair11:9122 2012 |
| PubMed ID: 23347616 |
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| Davis T, Kipling D, Assessing the role of stress signalling via p38 MAP kinase in the premature senescence of Ataxia Telangiectasia and Werner syndrome fibroblasts Biogerontology10:253-66 2008 |
| PubMed ID: 18830681 |
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| Crabbe L, Jauch A, Naeger CM, Holtgreve-Grez H, Karlseder J, Telomere dysfunction as a cause of genomic instability in Werner syndrome Proceedings of the National Academy of Sciences of the United States of America104:2205-10 2007 |
| PubMed ID: 17284601 |
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| Davis T, Wyllie FS, Rokicki MJ, Bagley MC, Kipling D, The role of cellular senescence in Werner syndrome: toward therapeutic intervention in human premature aging Annals of the New York Academy of Sciences1100:455-69 2007 |
| PubMed ID: 17460211 |
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| Britt-Compton B, Rowson J, Locke M, Mackenzie I, Kipling D, Baird DM, Structural stability and chromosome-specific telomere length is governed by cis-acting determinants in humans Human molecular genetics15:725-33 2006 |
| PubMed ID: 16421168 |
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| Davis T, Haughton MF, Jones CJ, Kipling D, Prevention of accelerated cell aging in the Werner syndrome Annals of the New York Academy of Sciences1067:243-7 2006 |
| PubMed ID: 16803993 |
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| Davis T, Baird DM, Haughton MF, Jones CJ, Kipling D, Prevention of Accelerated Cell Aging in Werner Syndrome Using a p38 Mitogen-Activated Protein Kinase Inhibitor. J Gerontol A Biol Sci Med Sci60(11):1386-93 2005 |
| PubMed ID: 16339323 |
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| Baird DM, Davis T, Rowson J, Jones CJ, Kipling D, Normal telomere erosion rates at the single cell level in Werner syndrome fibroblast cells. Hum Mol Genet13(14):1515-24 2004 |
| PubMed ID: 15150162 |
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| Davis T, Faragher RG, Jones CJ, Kipling D, Investigation of the Signaling Pathways Involved in the Proliferative Life Span Barriers in Werner Syndrome Fibroblasts. Ann N Y Acad Sci1019:274-277 2004 |
| PubMed ID: 15247028 |
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| Davis T, Singhrao SK, Wyllie FS, Haughton MF, Smith PJ, Wiltshire M, Wynford-Thomas D, Jones CJ, Faragher RG, Kipling D, Telomere-based proliferative lifespan barriers in Werner-syndrome fibroblasts involve both p53-dependent and p53-independent mechanisms. J Cell Sci116(Pt 7):1349-57 2003 |
| PubMed ID: 12615976 |
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| von Kobbe C, Harrigan JA, May A, Opresko PL, Dawut L, Cheng WH, Bohr VA, Central role for the Werner syndrome protein/poly(ADP-ribose) polymerase 1 complex in the poly(ADP-ribosyl)ation pathway after DNA damage. Mol Cell Biol23(23):8601-13 2003 |
| PubMed ID: 14612404 |
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| Choi D, Whittier PS, Oshima J, Funk WD, Telomerase expression prevents replicative senescence but does not fully reset mRNA expression patterns in Werner syndrome cell strains. FASEB J15(6):1014-20 2001 |
| PubMed ID: 11292662 |
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| Wyllie FS, Jones CJ, Skinner JW, Haughton MF, Wallis C, Wynford-Thomas D, Faragher RG, Kipling D, Telomerase prevents the accelerated cell ageing of Werner syndrome fibroblasts. Nat Genet24(1):16-7 2000 |
| PubMed ID: 10615119 |
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| Cowles EA, Brauker JH, Anderson RL, Turnover of sulfated glycosaminoglycans in fibroblasts derived from patients with Werner's syndrome. Exp Cell Res168:347-57 1987 |
| PubMed ID: 3100317 |
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| Goto M, Tanimoto K, Horiuchi Y, Sasazuki T, Family analysis of Werner's syndrome: a survey of 42 Japanese families with a review of the literature. Clin Genet19:8-15 1981 |
| PubMed ID: 7460386 |
| Passage Frozen |
8 |
| Split Ratio |
1:3 |
| Temperature |
37 C |
| Percent CO2 |
5% |
| Percent O2 |
AMBIENT |
| 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 |
| Supplement |
- |
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