AG00989 HUTCHINSON-GILFORD PROGERIA SYNDROME; HGPS | LAMIN A/C; LMNA Skin, Skin Cell Line NTCC®细胞株-BioVector NTCC细胞库

Name细胞名称：NTCC® AG00989 HUTCHINSON-GILFORD PROGERIA SYNDROME; HGPS | LAMIN A/C; LMNA Skin, Skin Cell Line
哈金森-吉尔福德早衰综合症；全球定位系统|拉明空调； LMNA细胞株
Description描述：Donor was diagnosed with atypical progeria and an unspecified type of cachectic dwarfism. The culture was initiated on 2/11/69 using explants of minced skin and was designated Strain S.J. The cell morphology is fibroblast-like. Culture was frozen at PDL 22. This donor is heterozygous for a C-to-T substitution at nucleotide 2142 (2142C>T) in exon 11 of the LMNA gene, resulting in a missense mutation in codon 644 [Arg644Cys (R644C)]. This mutation affects only Lamin A. R644 is in the putative cleavage recognition sequence for the prelamin A endoprotease, Zmpste24.
Category分类：Fibroblast
ID编号:NTCC®-AG00989
Size/Quantity数量: 1 Flask/2 Vials
Biosafety Level生物安全级别:1
Shipping Info运输方式: RT/Dry Ice
Storage储存方式: RT/Liquid Nitrogen
Race:
Age年龄:20 YR
Gender性别:Male
Cell Type细胞类型: HUTCHINSON-GILFORD PROGERIA SYNDROME; HGPS | LAMIN A/C; LMNA
Affected State:Yes
Product:Fibroblast
Gene:LMNA
Mutations突变:ARG644CYS
Ethnicity:
Family:
Relationship:proband
Karyotype:
Tissue Type组织来源:Skin
Complete Growth Medium完全培养基: BioVector® Eagles Minimum Essential Medium with Earle's salts:Dulbecco's modified MEM with 2mM L-glutamine or equivalent Complete Medium
Subculturing传代方法:
Cryopreservation冻存方法:Freeze medium: Complete growth medium supplemented with 5% (v/v) DMSO
Storage temperature: liquid nitrogen vapor phase
Culture Conditions培养条件:Atmosphere: air, 95%; carbon dioxide (CO2), 5%
Temperature: 37.0°C
STR Profile鉴定数据:
References参考文献:

Supplier供应商:BioVector质粒载体菌株细胞蛋白抗体基因保藏中心
NTCC典型培养物保藏中心
Tel电话：400-800-2947
Email：Biovector@163.com
http://www.biovector.net