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

For Research Use Only. Not for use in diagnostic procedures.

Polyclonal

Rabbit Ig

Rabbit

This BDHA1 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 228-255 amino acids from the Central region of human BDHA1.

Peptide Affinity Purified Rabbit Polyclonal Antibody (Pab)

Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is purified through a protein A column, followed by peptide affinity purification.

Vial Concentration: 0.5 (lot specific)

Maintain refrigerated at 2-8 degree C for up to 6 months. For long term storage store at -20 degree C in small aliquots to prevent freeze-thaw cycles.

Small volumes of anti-PDHA1 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

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.

Cancer; Metabolism; Signal Transduction

Western Blot (WB), ELISA (EIA), Flow Cytometry (FC/FACS)

WB~~1:1000

BDHA1 Antibody (Center) western blot analysis in HepG2 cell line lysates (15ug/lane).This demonstrates the BDHA1 antibody detected the BDHA1 protein (arrow).

BDHA1 Antibody (Center) flow cytometric analysis of HepG2 cells (right histogram) compared to a negative control cell (left histogram).FITC-conjugated goat-anti-rabbit secondary antibodies were used for the analysis.

43296

pyruvate dehydrogenase (lipoamide) alpha 1

pyruvate dehydrogenase E1 component subunit alpha, somatic form, mitochondrial

Pyruvate dehydrogenase E1 component subunit alpha, somatic form, mitochondrial

PHE1A??[Similar Products]

ODPA_HUMAN

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]

PDHA1: a mitochondrial matrix enzyme that catalyzes the oxidative decarboxylation of pyruvate, producing acetyl-CoA and CO2. A key enzyme in controlling the balance between lipid and glucose oxidation depending on substrate availability. The pyruvate dehydrogenase (PDH) holoenzyme is a multi-enzyme complex (PDHC) that contains 20-30 copies of pyruvate decarboxylase tetramers (2 alpha:2 beta)(E1), 60 copies of dihydrolipoamide acetyltransferase (E2), six homodimers of dihydrolipoamide dehydrogenase (E3), plus E3 binding proteins. The activity of PDH is tightly regulated by phosphorylation. The phosphorylation of at least one of three specific serine residues in E1 subunit by PDHK inactivates the PDHC, while dephosphorylation by PDP restores its activity. Sites 1, 2, and 3 of PDHA1 are S293, S300, and S232, respectively. Four PDHK isoenzymes have been described, each with different site specificity: all four phosphorylate sites 1 and 2 but at different rates; for site 1 PDHK2 >PDHK4 >PDHK1 >PDHK3; for site 2, PDHK3> PDHK4 > PDHK2 > PDHK1. Only PDHK1 phosphorylates site 3. PDHA1 deficiency is the most common enzyme defect in patients with primary lactic acidosis.

Protein type: EC 1.2.4.1; Amino Acid Metabolism - valine, leucine and isoleucine biosynthesis; Carbohydrate Metabolism - pyruvate; Carbohydrate Metabolism - glycolysis and gluconeogenesis; Carbohydrate Metabolism - butanoate; Oxidoreductase; Mitochondrial; Carbohydrate Metabolism - citrate (TCA) cycle

Chromosomal Location of Human Ortholog: Xp22.1

Cellular Component: mitochondrion; mitochondrial matrix; pyruvate dehydrogenase complex; nucleus

Molecular Function: pyruvate dehydrogenase activity; pyruvate dehydrogenase (acetyl-transferring) activity

Biological Process: acetyl-CoA biosynthetic process from pyruvate; cellular metabolic process; glycolysis; tricarboxylic acid cycle; glucose metabolic process; regulation of acetyl-CoA biosynthetic process from pyruvate; pyruvate metabolic process

Disease: Pyruvate Dehydrogenase E1-alpha Deficiency

Glushakova, L.G., et al. Mol. Genet. Metab. 98(3):289-299(2009)
Joao Silva, M., et al. Eur. J. Pediatr. 168(1):17-22(2009)
Boichard, A., et al. Mol. Genet. Metab. 93(3):323-330(2008)

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