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AG03513 Fibroblast from Skin, Arm

Description:

HUTCHINSON-GILFORD PROGERIA SYNDROME; HGPS

Affected:

Yes

Sex:

Male

Age:

13 YR (At Sampling)

  • Overview
  • Characterizations
  • Phenotypic Data
  • Publications
  • External Links
  • Culture Protocols

Overview

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Repository NIA Aging Cell Culture Repository
Subcollection Heritable Diseases
Biopsy Source Arm
Cell Type Fibroblast
Tissue Type Skin
Transformant Untransformed
Sample Source Fibroblast from Skin, Arm
Race White
Ethnicity MEXICAN
Family Member 1
Relation to Proband proband
Confirmation Clinical summary/Case history
Species Homo sapiens
Common Name Human
Remarks Donor has classical features of progeria, but no signs of heart disease. Parents and sibs are clinically unaffected. The biopsy was taken antemortem on 5/15/79 from skin on the medial forearm. The culture was initiated using explants of minced skin tissue. The cell morphology is fibroblast-like. The karyotype is 46,XY; normal diploid male. An increased thermolabile component of triosephosphate isomerase has been reported for this culture. A lymphoblast culture from same donor is AG03506. The legacy karyotype description shown in this Remark may not be representative of the current available product.

Characterizations

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PDL at Freeze 5.05
Passage Frozen 8
 
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
 

Phenotypic Data

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Remarks Donor has classical features of progeria, but no signs of heart disease. Parents and sibs are clinically unaffected. The biopsy was taken antemortem on 5/15/79 from skin on the medial forearm. The culture was initiated using explants of minced skin tissue. The cell morphology is fibroblast-like. The karyotype is 46,XY; normal diploid male. An increased thermolabile component of triosephosphate isomerase has been reported for this culture. A lymphoblast culture from same donor is AG03506. The legacy karyotype description shown in this Remark may not be representative of the current available product.

Publications

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Maynard S, Hall A, Galanos P, Rizza S, Yamamoto T, Gram HH, Munk SHN, Shoaib M, Sørensen CS, Bohr VA, Lerdrup M, Maya-Mendoza A, Bartek J, Lamin A/C impairments cause mitochondrial dysfunction by attenuating PGC1a and the NAMPT-NAD+ pathway Nucleic acids research50:9948-9965 2022
PubMed ID: 36099415
 
Chen X, Yao H, Kashif M, Revêchon G, Eriksson M, Hu J, Wang T, Liu Y, Tüksammel E, Strömblad S, Ahearn IM, Philips MR, Wiel C, Ibrahim MX, Bergo MO, A small-molecule ICMT inhibitor delays senescence of Hutchinson-Gilford progeria syndrome cells eLife10:9948-9965 2020
PubMed ID: 33526168
 
Mu X, Tseng C, Hambright WS, Matre P, Lin CY, Chanda P, Chen W, Gu J, Ravuri S, Cui Y, Zhong L, Cooke JP, Niedernhofer LJ, Robbins PD, Huard J, Cytoskeleton stiffness regulates cellular senescence and innate immune response in Hutchinson-Gilford Progeria Syndrome Aging cell10:9948-9965 2019
PubMed ID: 32710480
 
Lan YY, Heather JM, Eisenhaure T, Garris CS, Lieb D, Raychowdhury R, Hacohen N, Extranuclear DNA accumulates in aged cells and contributes to senescence and inflammation Aging cell10:e12901 2018
PubMed ID: 30706626
 
Li Y, Zhou G, Bruno IG, Zhang N, Sho S, Tedone E, Lai TP, Cooke JP, Shay JW, Transient introduction of human telomerase mRNA improves hallmarks of progeria cells Aging cell18:e12979 2018
PubMed ID: 31152494
 
Luo YB, Mitrpant C, Johnsen R, Fabian V, Needham M, Fletcher S, Wilton SD, Mastaglia FL, Investigation of splicing changes and post-translational processing of LMNA in sporadic inclusion body myositis Int J Clin Exp Pathol6(9):1723-33 2013
PubMed ID: 24040437
 
Luo YB, Mitrpant C, Johnsen RD, Fabian VA, Fletcher S, Mastaglia FL, Wilton SD., Investigation of age-related changes in LMNA splicing and expression of progerin in human skeletal muscles Int J Clin Exp Pathol6(12):2778-86 2013
PubMed ID: 24294364
 
Chen CY, Chi YH, Mutalif RA, Starost MF, Myers TG, Anderson SA, Stewart CL, Jeang KT., Accumulation of inner nuclear envelope protein Sun1 is pathogenic in progeric and dystrophic laminopathies. Cell149:565-77 2012
PubMed ID: 22541428
 
Britt-Compton B, Wyllie F, Rowson J, Capper R, Jones RE, Baird DM, Telomere dynamics during replicative senescence are not directly modulated by conditions of oxidative stress in IMR90 fibroblast cells Biogerontology149:565-77 2008
PubMed ID: 19214769
 
Decker ML, Chavez E, Vulto I, Lansdorp PM, Telomere length in Hutchinson-Gilford progeria syndrome Mechanisms of ageing and development130:377-83 2008
PubMed ID: 19428457
 
Glynn MW, Glover TW, Incomplete processing of mutant lamin A in Hutchinson-Gilford progeria leads to nuclear abnormalities, which are reversed by farnesyltransferase inhibition Human molecular genetics14:2959-69 2005
PubMed ID: 16126733
 
Huang S, Chen L, Libina N, Janes J, Martin GM, Campisi J, Oshima J, Correction of cellular phenotypes of Hutchinson-Gilford Progeria cells by RNA interference Human genetics118:444-50 2005
PubMed ID: 16208517
 
Reddel CJ, Weiss AS, Lamin A expression levels are unperturbed at the normal and mutant alleles but display partial splice site selection in Hutchinson-Gilford progeria syndrome Journal of medical genetics41:715-7 2004
PubMed ID: 15342704
 
Giro M, Davidson JM, Familial co-segregation of the elastin phenotype in skin fibroblasts from Hutchinson-Gilford progeria. Mech Ageing Dev70:163-36 1993
PubMed ID: 8246632
 
Winkles JA, O'Connor ML, Friesel R, Altered regulation of platelet-derived growth factor A-chain and c-fos gene expression in senescent progeria fibroblasts. J Cell Physiol144:313-25 1990
PubMed ID: 2166059
 
Nakamura KD, Turturro A, Hart RW, Elevated c-myc expression in progeria fibroblasts. Biochem Biophys Res Commun155:996-1000 1988
PubMed ID: 3421979
 
Sephel GC, Sturrock A, Giro MG, Davidson JM, Increased elastin production by progeria skin fibroblasts is controlled by the steady-state levels of elastin mRNA. J Invest Dermatol90:643-7 1988
PubMed ID: 3361140
 
Oliver CN, Ahn BW, Moerman EJ, Goldstein S, Stadtman ER, Age-related changes in oxidized proteins. J Biol Chem262:5488-91 1987
PubMed ID: 3571220
 
Mokrasch LC, Cell membrane transport enzymes in cultured dermal fibroblasts from progeroid donors: a comparison to other human fibroblasts. Gerontology32:202-6 1986
PubMed ID: 3770489
 
Chapman ML, Zaun MR, Gracy RW, Changes in NAD levels in human lymphocytes and fibroblasts during aging and in premature aging syndromes. Mech Ageing Dev21:157-67 1983
PubMed ID: 6223188
 
Tollefsbol TO, Zaun MR, Gracy RW, Increased lability of triosephosphate isomerase in progeria and Werner's syndrome fibroblasts. Mech Ageing Dev20:93-101 1982
PubMed ID: 7176709

External Links

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dbSNP dbSNP ID: 14675
NCBI GTR 176670 HUTCHINSON-GILFORD PROGERIA SYNDROME; HGPS
OMIM 176670 HUTCHINSON-GILFORD PROGERIA SYNDROME; HGPS
Omim Description HUTCHINSON-GILFORD PROGERIA SYNDROME; HGPS
  PROGERIA

Culture Protocols

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Passage Frozen 8
Split Ratio 1:4
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 -
Pricing
Commercial:
$257.00USD
Academic &
Non-profit:
$103.00USD
NIA Grantees:
$47.00USD
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