AG07076
Fibroblast from Skin, Skin
Description:
COCKAYNE SYNDROME TYPE A - 216400
Repository
|
NIA Aging Cell Culture Repository
|
Subcollection |
Heritable Diseases |
Class |
Repair Defective and Chromosomal Instability Syndromes |
Biopsy Source
|
Skin
|
Cell Type
|
Fibroblast
|
Tissue Type
|
Skin
|
Transformant
|
Untransformed
|
Sample Source
|
Fibroblast from Skin, Skin
|
Race
|
White
|
Family Member
|
1
|
Relation to Proband
|
proband
|
Confirmation
|
Clinical summary/Case history
|
ISCN
|
46,XX
|
Species
|
Homo sapiens
|
Common Name
|
Human
|
Remarks
|
|
PDL at Senescence |
22 |
PDL at Freeze |
5 |
Passage Frozen |
2 |
|
IDENTIFICATION OF SPECIES OF ORIGIN |
Species of Origin Confirmed by Chromosome Analysis |
|
Remarks |
The donor has clinical features of photophobia, dwarfism, mental retardation, cataracts, retinopathy and optic atrophy. The skin biopsy was taken ante-mortem on 9/22/83. The culture was initiated using explants of minced skin tissue. The cell morphology is fibroblast-like. The karyotype is 46,XX; normal diploid female. A lymphoblast culture from the same donor is AG07075. The legacy karyotype description shown in this Remark may not be representative of the current available product. |
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 |
PDL at Senescence |
22 |
Cumulative PDL at Freeze |
5 |
Passage Frozen |
2 |
Split Ratio |
1:4 |
Temperature |
37 C |
Percent CO2 |
5% |
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 |
- |
|
|