Human PDHA1 / C54G1 (aa 30-390) recombinant protein

Human PDHA1 / C54G1 (aa 30-390) recombinant protein

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• Material Safety Data Sheets (MSDS)

PDHA1, also known as C54G1, is an alpha subunit of pyruvate dehydrogenase. Pyruvate dehydrogenase, together with dihydrolipoamide acetyltransferase and lipoamide dehydrogenase, composes the pyruvate dehydrogenase (PDH) complex. The 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. PDHA1 plays a key role in the function of the PDH complex. Defects in PDHA1 can cause pyruvate dehydrogenase E1-alpha deficiency. Defects in PDHA1 also are the cause of X-linked Leigh syndrome (X-LS). X-LS is an early-onset progressive neurodegenerative disorder with a characteristic neuropathology consisting of focal, bilateral lesions in one or more areas of the central nervous system, including the brainstem, thalamus, basal ganglia, cerebellum, and spinal cord.

Pyruvate dehydrogenase E1 component subunit alpha, somatic form, mitochondrial

PHE1A; PDHA; PYRUVATE DEHYDROGENASE (LIPOAMIDE) ALPHA 1; RP11-723P2.1; PDHAD; MITOCHONDRIAL; PYRUVATE DEHYDROGENASE E1 ALPHA 1; SOMATIC FORM; RP23-36P16.4; PDHA-1; PYRUVATE DEHYDROGENASE E1ALPHA SUBUNIT; PYRUVATE DEHYDROGENASE E1 COMPONENT SUBUNIT ALPHA; PYRUVATE DEHYDROGENASE COMPLEX; PDHCE1A; E1-ALPHA POLYPEPTIDE 1; MITOCHONDRIAL; PDHE1-A TYPE I; PDHA1

P08559

PDHA1; PHE1A

Baculovirus-Insect Cells

A DNA sequence encoding the human PDHA1 (P08559-1) (Phe30-Ser390) was fused with two additional amino acids (Gly & Pro) at the N-terminus.

Human

The recombinant human PDHA1 consists of 363 amino acids and has a calculated molecular mass of 40.4 kDa. The recombinant protein migrates as an approximately 45 kDa band in SDS-PAGE under reducing conditions.

> 85 % as determined by SDS-PAGE