GM00038
Fibroblast from Skin, Unspecified
Description:
APPARENTLY HEALTHY INDIVIDUAL
|
Repository
|
NIGMS Human Genetic Cell Repository
|
| Subcollection |
Apparently Healthy Collection |
|
Biopsy Source
|
Unspecified
|
|
Cell Type
|
Fibroblast
|
|
Tissue Type
|
Skin
|
|
Transformant
|
Untransformed
|
|
Sample Source
|
Fibroblast from Skin, Unspecified
|
|
Race
|
Black/African American
|
|
Family Member
|
1
|
|
Relation to Proband
|
daughter
|
|
Confirmation
|
Karyotypic analysis and Case history
|
|
Species
|
Homo sapiens
|
|
Common Name
|
Human
|
|
Remarks
|
|
| PDL at Freeze |
5.57 |
| Passage Frozen |
12 |
| |
| 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 |
| |
| Remarks |
46,XX; 2% of cells show random chromosome loss and 4% show random chromosomal aberrations; skin biopsy; mother is GM00043B |
| Sprecher U, Dsouza J, Marisat M, Barasch D, Mishra K, Kakhlon O, Manor J, Anikster Y, Weil M, In depth profiling of dihydrolipoamide dehydrogenase deficiency in primary patients fibroblasts reveals metabolic reprogramming secondary to mitochondrial dysfunction Molecular genetics and metabolism reports42:101172 2025 |
| PubMed ID: 39802097 |
| |
| A Single-Cell Metabolic Profiling Characterizes Human Aging via SlipChip-SERS, Fugang Liu, Jiaqing Liu, Yang Luo, Siyi Wu, Xu Liu, Haoran Chen, Zhewen Luo, Haitao Yuan, Feng Shen, Fangfang Zhu, Jian Ye Adv Sci (Weinh).42:101172 2024 |
| PubMed ID: 39231358 |
| |
| Park S, Oh AY, Hong BS, Shin YJ, Jang H, Seo H, Kang SM, Woo TG, Park HP, Jeong J, Kim HJ, Kim BH, Kwon Y, Park BJ, The therapeutic effect of DX2 inhibition in nicotine-induced lung cancer progression Molecular therapy Oncology32:200875 2024 |
| PubMed ID: 39351074 |
| |
| Vidak S, Serebryannyy LA, Pegoraro G, Misteli T, Activation of endoplasmic reticulum stress in premature aging via the inner nuclear membrane protein SUN2 Cell reports42:112534 2023 |
| PubMed ID: 37210724 |
| |
| Kaul L, Grundmann CE, Köll-Weber M, Löffler H, Weiz A, Zannettino ACW, Richter K, Süss R, A Thermosensitive, Chitosan-Based Hydrogel as Delivery System for Antibacterial Liposomes to Surgical Site Infections Pharmaceutics14:112534 2022 |
| PubMed ID: 36559332 |
| |
| Langer C, Köll-Weber M, Holzer M, Hantel C, Süss R, Mitotane Nanocarriers for the Treatment of Adrenocortical Carcinoma: Evaluation of Albumin-Stabilized Nanoparticles and Liposomes in a Preclinical In Vitro Study with 3D Spheroids Pharmaceutics14:112534 2022 |
| PubMed ID: 36145639 |
| |
| Ramarajan MG, Saraswat M, Budhraja R, Garapati K, Raymond K, Pandey A, Mass spectrometric analysis of chondroitin sulfate-linked peptides Journal of proteins and proteomics13:187-203 2022 |
| PubMed ID: 36213313 |
| |
| Wedge ME, Jennings VA, Crupi MJF, Poutou J, Jamieson T, Pelin A, Pugliese G, de Souza CT, Petryk J, Laight BJ, Boileau M, Taha Z, Alluqmani N, McKay HE, Pikor L, Khan ST, Azad T, Rezaei R, Austin B, He X, Mansfield D, Rose E, Brown EEF, Crawford N, Alkayyal A, Surendran A, Singaravelu R, Roy DG, Migneco G, McSweeney B, Cottee ML, Jacobus EJ, Keller BA, Yamaguchi TN, Boutros PC, Geoffrion M, Rayner KJ, Chatterjee A, Auer RC, Diallo JS, Gibbings D, tenOever BR, Melcher A, Bell JC, Ilkow CS, Virally programmed extracellular vesicles sensitize cancer cells to oncolytic virus and small molecule therapy Nature communications13:1898 2022 |
| PubMed ID: 35393414 |
| |
| Kang SM, Yoon MH, Ahn J, Kim JE, Kim SY, Kang SY, Joo J, Park S, Cho JH, Woo TG, Oh AY, Chung KJ, An SY, Hwang TS, Lee SY, Kim JS, Ha NC, Song GY, Park BJ, Progerinin, an optimized progerin-lamin A binding inhibitor, ameliorates premature senescence phenotypes of Hutchinson-Gilford progeria syndrome Communications biology4:5 2021 |
| PubMed ID: 33398110 |
| |
| Ligezka AN, Radenkovic S, Saraswat M, Garapati K, Ranatunga W, Krzysciak W, Yanaihara H, Preston G, Brucker W, McGovern RM, Reid JM, Cassiman D, Muthusamy K, Johnsen C, Mercimek-Andrews S, Larson A, Lam C, Edmondson AC, Ghesquière B, Witters P, Raymond K, Oglesbee D, Pandey A, Perlstein EO, Kozicz T, Morava E, Sorbitol Is a Severity Biomarker for PMM2-CDG with Therapeutic Implications Annals of neurology4:5 2021 |
| PubMed ID: 34652821 |
| |
| Ng BG, Sosicka P, Fenaille F, Harroche A, Vuillaumier-Barrot S, Porterfield M, Xia ZJ, Wagner S, Bamshad MJ, Vergnes-Boiteux MC, Cholet S, Dalton S, Dell A, Dupré T, Fiore M, Haslam SM, Huguenin Y, Kumagai T, Kulik M, McGoogan K, Michot C, Nickerson DA, Pascreau T, Borgel D, Raymond K, Warad D, University of Washington Center for Mendelian Genomics (UW-CMG) D, Flanagan-Steet H, Steet R, Tiemeyer M, Seta N, Bruneel A, Freeze HH, A mutation in SLC37A4 causes a dominantly inherited congenital disorder of glycosylation characterized by liver dysfunction American journal of human genetics108:1040-1052 2021 |
| PubMed ID: 33964207 |
| |
| Ashikawa H, Mogi H, Honda T, Nakamura H, Murayama T, Beneficial effects of primidone in Niemann-Pick disease type C (NPC)-model cells and mice: Reduction of unesterified cholesterol levels in cells and extension of lifespan in mice European journal of pharmacology896:173907 2020 |
| PubMed ID: 33503462 |
| |
| Even A, Morelli G, Turchetto S, Shilian M, Bail RL, Laguesse S, Krusy N, Brisker A, Brandis A, Inbar S, Chariot A, Saudou F, Dietrich P, Dragatsis I, Brone B, Broix L, Rigo JM, Weil M, Nguyen L, ATP-citrate lyase promotes axonal transport across species Nature communications12:5878 2020 |
| PubMed ID: 34620845 |
| |
| 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 |
| |
| Song X, Ma F, Herrup K, Accumulation of Cytoplasmic DNA Due to ATM Deficiency Activates the Microglial Viral Response System with Neurotoxic Consequences The Journal of neuroscience : the official journal of the Society for Neuroscience39:6378-6394 2019 |
| PubMed ID: 31189575 |
| |
| Fleischer JG, Schulte R, Tsai HH, Tyagi S, Ibarra A, Shokhirev MN, Huang L, Hetzer MW, Navlakha S, Predicting age from the transcriptome of human dermal fibroblasts Genome Biology19:6378-6394 2018 |
| PubMed ID: 30567591 |
| |
| Flint M, Chatterjee P, Lin DL, McMullan LK, Shrivastava-Ranjan P, Bergeron É, Lo MK, Welch SR, Nichol ST, Tai AW, Spiropoulou CF, A genome-wide CRISPR screen identifies N-acetylglucosamine-1-phosphate transferase as a potential antiviral target for Ebola virus Nature communications10:285 2018 |
| PubMed ID: 30655525 |
| |
| Ng BG, Lourenço CM, Losfeld ME, Buckingham KJ, Kircher M, Nickerson DA, Shendure J, Bamshad MJ, University of Washington Center for Mendelian Genomics MJ, Freeze HH, Mutations in the translocon-associated protein complex subunit SSR3 cause a novel congenital disorder of glycosylation Journal of inherited metabolic disease42:993-997 2018 |
| PubMed ID: 30945312 |
| |
| Nguyen D, Stutz R, Schorr S, Lang S, Pfeffer S, Freeze HH, Förster F, Helms V, Dudek J, Zimmermann R, Proteomics reveals signal peptide features determining the client specificity in human TRAP-dependent ER protein import Nature communications9:3765 2017 |
| PubMed ID: 30217974 |
| |
| Batenburg NL, Mitchell TR, Leach DM, Rainbow AJ, Zhu XD, Cockayne Syndrome group B protein interacts with TRF2 and regulates telomere length and stability Nucleic acids research40:9661-74 2012 |
| PubMed ID: 22904069 |
| |
| Candelario J, Chen LY, Marjoram P, Reddy S, Comai L., A filtering strategy identifies FOXQ1 as a potential effector of lamin A dysfunction. Aging4:567-77 2012 |
| PubMed ID: 22948034 |
| |
| McKenna DJ, Doherty BA, Downes CS, McKeown SR, McKelvey-Martin VJ, Use of the comet-FISH assay to compare DNA damage and repair in p53 and hTERT genes following ionizing radiation PloS one7:e49364 2012 |
| PubMed ID: 23145163 |
| |
| Candelario J, Borrego S, Reddy S, Comai L, Accumulation of distinct prelamin A variants in human diploid fibroblasts differentially affects cell homeostasis Experimental cell research317:319-29 2010 |
| PubMed ID: 20974128 |
| |
| Hadzic T, Aykin-Burns N, Zhu Y, Coleman MC, Leick K, Jacobson GM, Spitz DR, Paclitaxel combined with inhibitors of glucose and hydroperoxide metabolism enhances breast cancer cell killing via H2O2-mediated oxidative stress Free radical biology & medicine48:1024-33 2009 |
| PubMed ID: 20083194 |
| |
| Pegoraro G, Kubben N, Wickert U, Göhler H, Hoffmann K, Misteli T, Ageing-related chromatin defects through loss of the NURD complex Nature cell biology11:1261-7 2009 |
| PubMed ID: 19734887 |
| |
| Hoerter JD, Ward CS, Bale KD, Gizachew AN, Graham R, Reynolds J, Ward ME, Choi C, Kagabo JL, Sauer M, Kuipers T, Hotchkiss T, Banner N, Chellson RA, Ohaeri T, Gant L, Vanderhill L, Effect of UVA fluence rate on indicators of oxidative stress in human dermal fibroblasts International journal of biological sciences4:63-70 2007 |
| PubMed ID: 18311331 |
| |
| Yamauchi Y, Reid PC, Sperry JB, Furukawa K, Takeya M, Chang CC, Chang TY, Plasma membrane rafts complete cholesterol synthesis by participating in retrograde movement of precursor sterols The Journal of biological chemistry282:34994-5004 2007 |
| PubMed ID: 17916561 |
| |
| Merla G, Howald C, Henrichsen CN, Lyle R, Wyss C, Zabot MT, Antonarakis SE, Reymond A, Submicroscopic deletion in patients with williams-beuren syndrome influences expression levels of the nonhemizygous flanking genes American journal of human genetics79:332-41 2006 |
| PubMed ID: 16826523 |
| |
| Pal A, Severin F, Lommer B, Shevchenko A, Zerial M, Huntingtin-HAP40 complex is a novel Rab5 effector that regulates early endosome motility and is up-regulated in Huntington's disease The Journal of cell biology172:605-18 2006 |
| PubMed ID: 16476778 |
| |
| Rydberg B, Cooper B, Cooper PK, Holley WR, Chatterjee A, Dose-dependent misrejoining of radiation-induced DNA double-strand breaks in human fibroblasts: experimental and theoretical study for high- and low-LET radiation Radiation research163:526-34 2005 |
| PubMed ID: 15850414 |
| |
| Zhu Z, Ramos J, Kampa K, Adimoolam S, Sirisawad M, Yu Z, Chen D, Naumovski L, Lopez CD, Control of ASPP2/(53BP2L) protein levels by proteasomal degradation modulates p53 apoptotic function The Journal of biological chemistry280:34473-80 2005 |
| PubMed ID: 16091363 |
| |
| Csoka AB, Cao H, Sammak PJ, Constantinescu D, Schatten GP, Hegele RA, Novel lamin A/C gene (LMNA) mutations in atypical progeroid syndromes. J Med Genet41(4):304-8 2004 |
| PubMed ID: 15060110 |
| |
| Csoka AB, English SB, Simkevich CP, Ginzinger DG, Butte AJ, Schatten GP, Rothman FG, Sedivy JM, Genome-scale expression profiling of Hutchinson-Gilford progeria syndrome reveals widespread transcriptional misregulation leading to mesodermal/mesenchymal defects and accelerated atherosclerosis. Aging Cell3(4):235-43 2004 |
| PubMed ID: 15268757 |
| |
| Huusko P, Ponciano-Jackson D, Wolf M, Kiefer JA, Azorsa DO, Tuzmen S, Weaver D, Robbins C, Moses T, Allinen M, Hautaniemi S, Chen Y, Elkahloun A, Basik M, Bova GS, Bubendorf L, Lugli A, Sauter G, Schleutker J, Ozcelik H, Elowe S, Pawson T, Trent JM, Carpten JD, Kallioniemi OP, Mousses S, Nonsense-mediated decay microarray analysis identifies mutations of EPHB2 in human prostate cancer. Nat Genet36(9):979-83 2004 |
| PubMed ID: 15300251 |
| |
| Yan Q, Hunt PR, Frelin L, Vida TA, Pevsner J, Bean AJ, mVps24p functions in EGF receptor sorting/trafficking from the early endosome Experimental cell research304:265-73 2004 |
| PubMed ID: 15707591 |
| |
| Famulski KS, Al-Hijailan RS, Dobler K, Pienkowska M, Al-Mohanna F, Paterson MC, Aberrant sensing of extracellular Ca2+ by cultured ataxia telangiectasia fibroblasts. Oncogene22(3):471-5 2003 |
| PubMed ID: 12545170 |
| |
| Rubbi CP, Milner J, Disruption of the nucleolus mediates stabilization of p53 in response to DNA damage and other stresses. EMBO J22(22):6068-77 2003 |
| PubMed ID: 14609953 |
| |
| Rubbi CP, Milner J, p53 is a chromatin accessibility factor for nucleotide excision repair of DNA
damage. EMBO J22(4):975-86 2003 |
| PubMed ID: 12574133 |
| |
| Tuo J, Jaruga P, Rodriguez H, Bohr VA, Dizdaroglu M, Primary fibroblasts of Cockayne syndrome patients are defective in cellular
repair of 8-hydroxyguanine and 8-hydroxyadenine resulting from oxidative stress. FASEB J17(6):668-74 2003 |
| PubMed ID: 12665480 |
| |
| Burgess JW, Kiss RS, Zheng H, Zachariah S, Marcel YL, Trypsin-sensitive and lipid-containing sites of the macrophage extracellular matrix bind apolipoprotein A-I and participate in ABCA1-dependent cholesterol efflux. J Biol Chem[epub ahead of print]:668-74 2002 |
| PubMed ID: 12050168 |
| |
| Rubbi CP, Milner J, Analysis of nucleotide excision repair by detection of single-stranded DNA transients. Carcinogenesis22(11):1789-96 2001 |
| PubMed ID: 11698340 |
| |
| Vassiliou G, Benoist F, Lau P, Kavaslar GN, McPherson R, The low density lipoprotein receptor-related protein contributes to selective uptake of high density lipoprotein cholesteryl esters by SW872 liposarcoma cells and primary human adipocytes. J Biol Chem276(52):48823-30 2001 |
| PubMed ID: 11604390 |
| |
| Le Page F, Kwoh EE, Avrutskaya A, Gentil A, Leadon SA, Sarasin A, Cooper PK, Transcription-coupled repair of 8-oxoguanine: requirement for XPG, TFIIH, and
CSB and implications for Cockayne syndrome. Cell101(2):159-71 2000 |
| PubMed ID: 10786832 |
| |
| Cooper MP, Balajee AS, Bohr VA, The C-terminal domain of p21 inhibits nucleotide excision repair In vitro and In vivo. Mol Biol Cell10:2119-29 1999 |
| PubMed ID: 10397753 |
| |
| Dianov G, Bischoff C, Sunesen M, Bohr VA, Repair of 8-oxoguanine in DNA is deficient in Cockayne syndrome group B cells. Nucleic Acids Res27(5):1365-8 1999 |
| PubMed ID: 9973627 |
| |
| Piret B, Schoonbroodt S, Piette J, The ATM protein is required for sustained activation of NF-kappaB following DNA damage. Oncogene18(13):2261-71 1999 |
| PubMed ID: 10327072 |
| |
| Wang XW, Zhan Q, Coursen JD, Khan MA, Kontny HU, Yu L, Hollander MC, O'Connor PM, Fornace AJ Jr, Harris CC, GADD45 induction of a G2/M cell cycle checkpoint. Proc Natl Acad Sci U S A96:3706-11 1999 |
| PubMed ID: 10097101 |
| |
| Ford JM, Hanawalt PC, Expression of wild-type p53 is required for efficient global genomic nucleotide excision repair in UV-irradiated human fibroblasts. J Biol Chem272:28073-80 1997 |
| PubMed ID: 9346961 |
| |
| Nouspikel T, Lalle P, Leadon SA, Cooper PK, Clarkson SG, A common mutational pattern in Cockayne syndrome patients from xeroderma pigmentosum group G: implications for a second XPG function. Proc Natl Acad Sci U S A94:3116-21 1997 |
| PubMed ID: 9096355 |
| |
| Lobrich M, Rydberg B, Cooper PK, Repair of x-ray-induced DNA double-strand breaks in specific Not I restriction fragments in human fibroblasts: joining of correct and incorrect ends. Proc Natl Acad Sci U S A92:12050-4 1995 |
| PubMed ID: 8618842 |
| |
| Mirzayans R, Famulski KS, Enns L, Fraser M, Paterson MC, Characterization of the signal transduction pathway mediating gamma ray- induced inhibition of DNA synthesis in human cells: indirect evidence for involvement of calmodulin but not protein kinase C nor p53. Oncogene11:1597-605 1995 |
| PubMed ID: 7478584 |
| |
| Wang XW, Yeh H, Schaeffer L, Roy R, Moncollin V, Egly JM, Wang Z, Freidberg EC, Evans MK, Taffe BG, et al, p53 modulation of TFIIH-associated nucleotide excision repair activity. Nat Genet10:188-95 1995 |
| PubMed ID: 7663514 |
| |
| Galloway AM, Liuzzi M, Paterson MC, Metabolic processing of cyclobutyl pyrimidine dimers and (6-4) photoproducts in UV-treated human cells. Evidence for distinct excision-repair pathways. J Biol Chem269:974-80 1994 |
| PubMed ID: 8288650 |
| |
| Evans MK, Taffe BG, Harris CC, Bohr VA, DNA strand bias in the repair of the p53 gene in normal human and xeroderma pigmentosum group C fibroblasts. Cancer Res53:5377-81 1993 |
| PubMed ID: 8221675 |
| |
| Leadon SA, Cooper PK, Preferential repair of ionizing radiation-induced damage in the transcribed strand of an active human gene is defective in Cockayne syndrome. Proc Natl Acad Sci U S A90(22):10499-503 1993 |
| PubMed ID: 8248136 |
| |
| Mirzayans R, Dietrich K, Paterson MC, Aphidicolin and 1-beta-D-arabinofuranosylcytosine strongly inhibit transcriptionally active DNA repair in normal human fibroblasts. Carcinogenesis14:2621-6 1993 |
| PubMed ID: 8269635 |
| |
| Zhen W, Evans MK, Haggerty CM, Bohr VA, Deficient gene specific repair of cisplatin-induced lesions in Xeroderma pigmentosum and Fanconi's anemia cell lines. Carcinogenesis14:919-24 1993 |
| PubMed ID: 8504485 |
| |
| Nagasawa H, Little JB, Tsang NM, Saunders E, Tesmer J, Strniste GF, Effect of dose rate on the survival of irradiated human skin fibroblasts. Radiat Res132:375-9 1992 |
| PubMed ID: 1475361 |
| |
| Mirzayans R, Paterson MC, Dose-dependent increase in repair of
1-beta-D-arabinofuranosylcytosine-detectable DNA lesions in UV-treated xeroderma
pigmentosum (group A) fibroblasts. Mutat Res262(3):151-7 1991 |
| PubMed ID: 2002813 |
| |
| Kantor GJ, Barsalou LS, Hanawalt PC, Selective repair of specific chromatin domains in UV-irradiated cells from xeroderma pigmentosum complementation group C. Mutat Res235:171-80 1990 |
| PubMed ID: 2342504 |
| |
| Milne R, Theolis R Jr, Maurice R, Pease RJ, Weech PK, Rassart E, Fruchart JC, Scott J, Marcel YL, The use of monoclonal antibodies to localize the low density lipoprotein receptor-binding domain of apolipoprotein B. J Biol Chem264:19754-60 1989 |
| PubMed ID: 2479639 |
| |
| Mirzayans R, Smith BP, Paterson MC, Hypersensitivity to cell killing and faulty repair of 1-beta-D- arabinofuranosylcytosine-detectable sites in human (ataxia- telangiectasia) fibroblasts treated with 4-nitroquinoline 1-oxide. Cancer Res49:5523-9 1989 |
| PubMed ID: 2507129 |
| |
| Young BR, Painter RB, Radioresistant DNA synthesis and human genetic diseases. Hum Genet82:113-7 1989 |
| PubMed ID: 2722185 |
| |
| Bender MA, Viola MV, Fiore J, Thompson MH, Leonard RC, Normal G2 chromosomal radiosensitivity and cell survival in the cancer family syndrome [see comments] Cancer Res48:2579-84 1988 |
| PubMed ID: 3356018 |
| |
| Mirzayans R, Liuzzi M, Paterson MC, Methylmethanesulfonate-induced DNA damage and its repair in cultured human
fibroblasts: normal rates of induction and removal of alkali-labile sites in
xeroderma pigmentosum (group A) cells. Carcinogenesis9(12):2257-63 1988 |
| PubMed ID: 3191570 |
| |
| Nagasawa H, Burke MJ, Little FF, McCone EF, Chan GL, Little JB, Multiple abnormalities in the ultraviolet light response of cultured fibroblasts derived from patients with the basal cell nevus syndrome. Teratog Carcinog Mutagen8:25-33 1988 |
| PubMed ID: 2897722 |
| |
| Nagasawa H, Little JB, Radiosensitivities of ten apparently normal human diploid fibroblast strains to cell killing, G2-phase chromosomal aberrations, and cell cycle delay. Cancer Res48:4535-8 1988 |
| PubMed ID: 3396006 |
| |
| Nagasawa H, Zamansky GB, McCone EF, Arundel CM, Matkin E, Little JB, Spontaneous transformation to anchorage-independent growth of a xeroderma pigmentosum fibroblast cell strain. J Invest Dermatol88:149-53 1987 |
| PubMed ID: 3805755 |
| |
| Bohr VA, Okumoto DS, Hanawalt PC, Survival of UV-irradiated mammalian cells correlates with efficient DNA repair in an essential gene. Proc Natl Acad Sci U S A83:3830-3 1986 |
| PubMed ID: 3459159 |
| |
| Nove J, Little JB, Mayer PJ, Troilo P, Nichols WW, Hypersensitivity of cells from a new chromosomal-breakage syndrome to DNA-damaging agents. Mutat Res163:255-62 1986 |
| PubMed ID: 3785261 |
| |
| Coates PM, Hale DE, Stanley CA, Corkey BE, Cortner JA, Genetic deficiency of medium-chain acyl coenzyme A dehydrogenase: studies in cultured skin fibroblasts and peripheral mononuclear leukocytes. Pediatr Res19:671-6 1985 |
| PubMed ID: 4022673 |
| |
| Hale DE, Batshaw ML, Coates PM, Frerman FE, Goodman SI, Singh I, Stanley CA, Long-chain acyl coenzyme A dehydrogenase deficiency: an inherited cause of nonketotic hypoglycemia. Pediatr Res19:666-71 1985 |
| PubMed ID: 4022672 |
| |
| Mirzayans R, Paterson MC, Waters R, Defective repair of a class of 4NQO-induced alkali-labile DNA lesions in xeroderma pigmentosum complementation group A fibroblasts. Carcinogenesis6:555-9 1985 |
| PubMed ID: 3921271 |
| |
| Nagasawa H, Latt SA, Lalande ME, Little JB, Effects of X-irradiation on cell-cycle progression, induction of chromosomal aberrations and cell killing in ataxia telangiectasia (AT) fibroblasts. Mutat Res148:71-82 1985 |
| PubMed ID: 3969079 |
| |
| Wooten MW, Rudick VL, Rudick MJ, Higgins ML, The effects of proteins secreted by fibroblasts from patients with cystic fibrosis on hamster tracheal explants. In Vitro Cell Dev Biol21:207-15 1985 |
| PubMed ID: 4008434 |
| |
| Donnelly PV, Reed P, DiFerrante N, Synthesis and sulfation of glycosaminoglycans in fibroblasts from a patient with Lowe's syndrome. Connect Tissue Res13:89-98 1984 |
| PubMed ID: 6242399 |
| |
| Mitchel RE, Chan A, Smith BP, Child SD, Paterson MC, The effects of hyperthermia and ionizing radiation in normal and ataxia telangiectasia human fibroblast lines. Radiat Res99(3):627-35 1984 |
| PubMed ID: 6473716 |
| |
| Smith CA, Okumoto DS, Nature of DNA repair synthesis resistant to inhibitors of polymerase alpha in human cells. Biochemistry23:1383-91 1984 |
| PubMed ID: 6426505 |
| |
| Tamm I, Kikuchi T, Wang E, Pfeffer LM, Growth rate of control and beta-interferon-treated human fibroblast populations over the course of their in vitro life span. Cancer Res44:2291-6 1984 |
| PubMed ID: 6722770 |
| |
| Daddona PE, Mitchell BS, Meuwissen HJ, Davidson BL, Wilson JM, Koller CA, Adenosine deaminase deficiency with normal immune function. An acidic enzyme mutation. J Clin Invest72:483-92 1983 |
| PubMed ID: 6603477 |
| |
| Li FP, Little JB, Bech-Hansen NT, Paterson MC, Arlett C, Garnick MB, Mayer RJ, Acute leukemia after radiotherapy in a patient with Turcot's syndrome. Impaired colony formation in skin fibroblast cultures after irradiation. Am J Med74:343-8 1983 |
| PubMed ID: 6572034 |
| |
| Nagasawa H, Little JB, Comparison of kinetics of X-ray-induced cell killing in normal, ataxia telangiectasia and hereditary retinoblastoma fibroblasts. Mutat Res109:297-308 1983 |
| PubMed ID: 6843573 |
| |
| Willecke K, Muller D, Druge PM, Frixen U, Schafer R, Dermietzel R, Hulser D, Isolation and characterization of Chinese hamster cells defective in cell-cell coupling via gap junctions. Exp Cell Res144:95-113 1983 |
| PubMed ID: 6840215 |
| |
| Bech-Hansen NT, Sell BM, Mulvihill JJ, Paterson MC, Association of in vitro radiosensitivity and cancer in a family with acute myelogenous leukemia. Cancer Res41:2046-50 1981 |
| PubMed ID: 6940653 |
| |
| Smith PJ, Paterson MC, Abnormal responses to mid-ultraviolet light of cultured fibroblasts from patients with disorders featuring sunlight sensitivity. Cancer Res41:511-8 1981 |
| PubMed ID: 6256067 |
| |
| Smith PJ, Paterson MC, Kraemer KH, In vitro radiosensitivity in a patient with dermatomyositis and cancer [letter] Lancet1:216-7 1981 |
| PubMed ID: 6109878 |
| |
| Walker IG, Alkaline sucrose sedimentation analysis as an indicator of repair capability of xeroderma pigmentosum fibroblasts for 4-nitroquinoline-1- oxide damage. Carcinogenesis2:691-5 1981 |
| PubMed ID: 6793257 |
| |
| Yu RL, Aronson MM, Nichols WW, High-resolution bands in human fibroblast chromosomes induced by actinomycin D. Cytogenet Cell Genet31:111-4 1981 |
| PubMed ID: 7307580 |
| |
| Daddona PE, Frohman MA, Kelley WN, Human adenosine deaminase and its binding protein in normal and adenosine deaminase-deficient fibroblast cell strains. J Biol Chem255:5681-7 1980 |
| PubMed ID: 7380831 |
| |
| Reiner EJ, Moran TF, High-performance pyrolysis-gas chromatography: potential for differentiating cystic fibrosis cells. J Chromatogr221:371-6 1980 |
| PubMed ID: 7217305 |
| |
| Smith PJ, Paterson MC, Gamma-ray induced inhibition of DNA synthesis in ataxia telangiectasia fibroblasts is a function of excision repair capacity. Biochem Biophys Res Commun97:897-905 1980 |
| PubMed ID: 6162467 |
| |
| Smith PJ, Paterson MC, Defective DNA repair and increased lethality in ataxia telangiectasia cells exposed to 4-nitroquinoline-1-oxide. Nature287:747-9 1980 |
| PubMed ID: 6776412 |
| |
| Vincent RA Jr, Fink AJ, Huang PC, Unscheduled DNA synthesis in cultured ataxia telangiectasia fibroblast- like cells. Mutat Res72:245-9 1980 |
| PubMed ID: 7442694 |
| |
| Hamilton TA, Wada HG, Sussman HH, Human placental cell surface antigens:expression by cultured cells of diverse phenotypic origin. J Supramol Struct11:503-15 1979 |
| PubMed ID: 544928 |
| |
| Paterson, Gamma-ray hypersensitivity and faulty DNA repair in cultured cells from humans exhibiting familial cancer proneness. Proc 6th Int Cong Rad Res, Tokyo(1979) p 484:503-15 1979 |
| PubMed ID: 544928 |
| |
| Sheridan RB 3d, Huang PC, Endonucleolytic activity for gamma-irradiated DNA in normal and ataxia telangiectasia fibroblast cell extracts. Mutat Res63:387-92 1979 |
| PubMed ID: 522879 |
| |
| Williams JI, Friedberg EC, Deoxyribonucleic acid excision repair in chromatin after ultraviolet irradiation of human fibroblasts in culture. Biochemistry18:3965-72 1979 |
| PubMed ID: 486406 |
| |
| Tan YH, Greene AE, Subregional localization of the gene(s) governing the human interferon induced antiviral state in man. J Gen Virol32:153-5 1976 |
| PubMed ID: 182912 |
| |
| Tan YH, Chou EL, Lundh N, Regulation of chromosome 21-directed anti-viral gene(s) as a consequence of age. Nature257:310-2 1975 |
| PubMed ID: 1161032 |
| Passage Frozen |
12 |
| 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 |
- |
|