AG07493
                                                
                                                Fibroblast from Skin, Arm
                                                
                                                
                                             
                                            
                                                
                                                    
                                                         Description:
                                                    
                                                    
                                                         
                                                            
                                                            HUTCHINSON-GILFORD PROGERIA SYNDROME; HGPS 
                                                            
                                                    
                                                 
                                                
                                                
                                                
                                                
                                             
                                         
                                     
                                    
                                        
                                            
                                            
                                            
                                            
                                                
                                                    
                                                        
                                                            
                                                            
                                                                
                                                                    
                                                                        | 
                                                                            Repository
                                                                         | 
                                                                        
                                                                            NIA Aging Cell Culture Repository
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	| Subcollection | 
	Heritable Diseases | 
 
                                                                
                                                                
                                                                
                                                                
                                                                
                                                                
                                                                
                                                                    
                                                                        | 
                                                                            Biopsy Source
                                                                         | 
                                                                        
                                                                            Arm
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                                                                        | 
                                                                            Cell Type
                                                                         | 
                                                                        
                                                                            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
                                                                         | 
                                                                        
                                                                            Fibroblast from Skin, Arm
                                                                         | 
                                                                     
                                                                
                                                                
                                                                    
                                                                        | 
                                                                            Race
                                                                         | 
                                                                        
                                                                            White
                                                                         | 
                                                                     
                                                                
                                                                
                                                                
                                                                
                                                                
                                                                
                                                                
                                                                
                                                                    
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                                                                            Family Member
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                                                                            1
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                                                                        | 
                                                                            Relation to Proband
                                                                         | 
                                                                        
                                                                            proband
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                                                                        | 
                                                                            Confirmation
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                                                                            Clinical summary/Case history
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                                                                        | 
                                                                            ISCN
                                                                         | 
                                                                        
                                                                            46,XX
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                                                                            Species
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                                                                            Homo sapiens
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                                                                        | 
                                                                            Common Name
                                                                         | 
                                                                        
                                                                            Human
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                                                                        | 
                                                                            Remarks
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	| PDL at Freeze | 
	14 | 
 
	| Passage Frozen | 
	6 | 
 
	|   | 
 
                                                                
	| 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 | 
	Donor diagnosed as having atypical progeria with features including alopecia, facial disproportion, small mandible, below 3% of normal height and weight, 3 times the normal level of hyaluronic acid, and circumoral blueness. The skin biopsy was taken ante-mortem from the upper left arm. The culture was initiated on 4/17/84 using explants of minced skin tissue. The cell morphology is fibroblast-like. The karyotype is 46,XX; normal diploid female.  The legacy karyotype description shown in this Remark may not be representative of the current available product. | 
 
                                                                
                                                             
                                                            
                                                            
                                                         
                                                     
                                                 
                                                
                                                    
                                                        
                                                            
                                                            
                                                            
                                                                
	| Fafián-Labora JA, Rodríguez-Navarro JA, O'Loghlen A, Small Extracellular Vesicles Have GST Activity and Ameliorate Senescence-Related Tissue Damage Cell metabolism: 2020 | 
 
	| PubMed ID: 32574561 | 
 
	|   | 
 
	| Tresini M, Lorenzini A, Torres C, Cristofalo VJ, Modulation of replicative senescence of diploid human cells by nuclear ERK signaling J Biol Chem282(6):4136-51 2007 | 
 
	| PubMed ID: 17145763 | 
 
	|   | 
 
	| Ouellette MM, McDaniel LD, Wright WE, Shay JW, Schultz RA, The establishment of telomerase-immortalized cell lines representing human
chromosome instability syndromes. Hum Mol Genet9(3):403-11 2000 | 
 
	| PubMed ID: 10655550 | 
 
	|   | 
 
	| 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 | 
 
                                                                
                                                             
                                                            
                                                         
                                                     
                                                 
                                                
                                                
                                                
                                                    
                                                        
                                                            
                                                            
                                                                
	| Cumulative PDL at Freeze | 
	14 | 
 
	| Passage Frozen | 
	6 | 
 
	| 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           | 
 
	| Substrate | 
	None specified | 
 
	| Subcultivation Method | 
	trypsin-EDTA | 
 
	| Supplement | 
	- | 
 
                                                                
                                                                
                                                             
                                                         
                                                     
                                                 
                                             
                                         
                                     
                                 
                                
                             
                         
                     
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