biological source
human
recombinant
expressed in E. coli
assay
≥70% (SDS-PAGE)
form
buffered aqueous glycerol solution
mol wt
~47 kDa
NCBI accession no.
application(s)
cell analysis
shipped in
dry ice
storage temp.
−70°C
Gene Information
human ... PDHA1(5160)
Biochem/physiol Actions
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.
Physical form
Supplied in 50mM sodium phosphate, pH 7.0, 300mM NaCl, 150mM imidazole, 0.1mM PMSF, 0.25mM DTT, 25% glycerol.
Preparation Note
after opening, aliquot into smaller quantities and store at -70 °C. Avoid repeating handling and multiple freeze/thaw cycles
signalword
Danger
hcodes
Hazard Classifications
Eye Irrit. 2 - Repr. 1B - Skin Irrit. 2
存储类别
6.1C - Combustible acute toxic Cat.3 / toxic compounds or compounds which causing chronic effects
wgk
WGK 1
flash_point_f
Not applicable
flash_point_c
Not applicable
法规信息
常规特殊物品
此项目有
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.
相关内容
Instructions
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