生物来源
human
重组
expressed in E. coli
检测方案
≥70% (SDS-PAGE)
形式
buffered aqueous glycerol solution
分子量
~47 kDa
NCBI登记号
应用
cell analysis
运输
dry ice
储存温度
−70°C
基因信息
human ... PDHA1(5160)
生化/生理作用
PDHA1 pyruvate dehydrogenase (lipoamide) alpha 1 is a member of the pyruvate dehydrogenase (PDH) complex. PDHA1 is a nuclear-encoded mitochondrial multienzyme complex which catalyzes the overall conversion of pyruvate to acetyl-CoA and CO(2), and provides the primary link between glycolysis and the tricarboxylic acid (TCA) cycle . PDHA1 plays a critical role in the brain that usually obtains all of its energy from the aerobic oxidation of glucose. Mutations in the PDHA1 are associated with pyruvate dehydrogenase E1-alpha deficiency and X-linked Leigh syndrome.
外形
Supplied in 50mM sodium phosphate, pH 7.0, 300mM NaCl, 150mM imidazole, 0.1mM PMSF, 0.25mM DTT, 25% glycerol.
制备说明
after opening, aliquot into smaller quantities and store at -70 °C. Avoid repeating handling and multiple freeze/thaw cycles
警示用语：
Danger
危险声明
危险分类
Eye Irrit. 2 - Repr. 1B - Skin Irrit. 2
WGK
WGK 1
闪点(°F)
Not applicable
闪点(°C)
Not applicable
法规信息
常规特殊物品
K Chun et al.
Human molecular genetics, 2(4), 449-454 (1993-04-01)
Human PDH complex deficiency is an extremely heterogeneous disease in its presentation and clinical course. In an investigation at the level of the gene into ten cases of PDH complex (E1) deficiency, we found that all had mutations in the
Pyruvate dehydrogenase deficiency.
G K Brown et al.
Journal of medical genetics, 31(11), 875-879 (1994-11-01)
商品
Fatty acid synthesis supports cancer cell proliferation, essential for membrane generation, protein modification, and bioenergetics.
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