Description

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.

References
1.Eur J Paediatr Neurol. 2021 Mar;31:27-30. 2.Pathol Res Pract. 2019 Mar;215(3):478-482.

Specification
Aliases	PDHA; PDHAD; PHE1A; PDHCE1A
Entrez GeneID	5160
Swissprot	P08559
clone	2A2H7
WB Predicted band size	43.3kDa
Host/Isotype	Mouse IgG2b
Antibody Type	Primary antibody
Storage	Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze/thaw cycles.
Species Reactivity	Human
Immunogen	Purified recombinant fragment of human PDHA1 (AA: 241-390) expressed in E. Coli.
Formulation	Purified antibody in PBS with 0.05% sodium azide

Application
WB	1/500 - 1/2000
FCM	1/200 - 1/400
ELISA	1/10000