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Protein structure
PDHA1
Pyruvate dehydrogenase E1 subunit alpha 1PDHA
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. [provided by RefSeq, Mar 2010]
Top validated antibodies |
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| Proteintech Group |  18068-1-AP |
26 references | Polyclonal |
WB
EL
ICC
IP
IHC
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| Invitrogen Antibodies | 66119-1-IG |
Monoclonal |
WB
ICC
IP
IHC
FC
OA
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| GeneTex | GTX104015 |
3 references | Polyclonal |
WB
ICC
IP
IHC
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| Novus Biologicals | NB110-93479 |
40 references | Polyclonal |
WB
ICC
IP
FC
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| NovoPro Bioscience Inc. | 107461 |
Monoclonal |
WB
EL
ICC
IP
IHC
FC
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