PDH-E1 Alpha Monoclonal Antibody

Ab type

Primary antibody

Background

The pyruvate dehydrogenase (PDH) complex is a nuclear-encoded mitochondrial multienzyme complex that 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. The PDH complex is composed of multiple copies of three enzymatic components: pyruvate dehydrogenase (E1), dihydrolipoamide acetyltransferase (E2) and lipoamide dehydrogenase (E3). The E1 enzyme is a heterotetramer of two alpha and two beta subunits. This gene encodes the E1 alpha 1 subunit containing the E1 active site, and plays a key role in the function of the PDH complex. Mutations in this gene are associated with pyruvate dehydrogenase E1-alpha deficiency and X-linked Leigh syndrome. Alternatively spliced transcript variants encoding different isoforms have been found for this gene.

Reactivity

Human,Mouse,Rat,Bovine,Dog,Pig

Clonality

Monoclonal

Host

Mouse

Isotype

IgG

Immunogen

Purified recombinant human PDH-E1α (C-terminus) protein fragments expressed in E.coli.

Storage Stability

-20°C for one year

Concentration

1 mg/ml

MolecularWeight_Da

N/A

synonyms

PDHA1; PHE1A; Pyruvate dehydrogenase E1 component subunit alpha; somatic form, mitochondrial; PDHE1-A type I