GM23684 CARBAMOYL PHOSPHATE SYNTHETASE I DEFICIENCY | CARBAMOYL PHOSPHATE SYNTHETASE I; CPS1 B-Lymphocyte Cell Line NTCC®细胞株-BioVector NTCC细胞库

Name细胞名称：NTCC® GM23684 CARBAMOYL PHOSPHATE SYNTHETASE I DEFICIENCY | CARBAMOYL PHOSPHATE SYNTHETASE I; CPS1 B-Lymphocyte Cell Line
氨基甲酰磷酸合成酶 I 缺乏症 |氨基甲酰磷酸合成酶I； CP1细胞株
Description描述：Clinically affected; onset of symptoms at age 15 years; past hyperammonemic events; normal neurological evaluation at age 5 years; A>G transition at nucleotide 3101 in exon 25 of the CPS1 gene (3101A>G) resulting in the substitution of glycine for glutamic acid at codon 1034 [Glu1034Gly (E1034G)]; treatments include: citrulline arginine supplement, benzoate,sodium phenylbutyrate, and protein restriction;
Category分类：B-Lymphocyte
ID编号:NTCC®-GM23684
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: White
Age年龄:5 YR
Gender性别:Female
Cell Type细胞类型: CARBAMOYL PHOSPHATE SYNTHETASE I DEFICIENCY | CARBAMOYL PHOSPHATE SYNTHETASE I; CPS1
Affected State:Yes
Product:LCL
Gene:CPS1
Mutations突变:GLU1034GLY
Ethnicity:
Family:
Relationship:proband
Karyotype:
Tissue Type组织来源:Blood
Complete Growth Medium完全培养基: BioVector® Roswell Park Memorial Institute Medium 1640 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
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