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Pyruvate Dehydrogenase Alpha (PDHa)

PDHCE1A; PHE1A; PHE1-A; PDHA1; PDH-A; Pyruvate Dehydrogenase(lipoamide)Alpha 1; Pyruvate Dehydrogenase E1 Component Subunit Alpha,Aomatic Form,Mitochondrial

The pyruvate dehydrogenase complex is a nuclear-encoded mitochondrial matrix multienzyme complex that provides the primary link between glycolysis and the tricarboxylic acid (TCA) cycle by catalyzing the irreversible conversion of pyruvate into acetyl-CoA. The PDH complex is composed of multiple copies of 3 enzymes: E1 (PDHA1); dihydrolipoyl transacetylase (DLAT) (E2); and dihydrolipoyl dehydrogenase (DLD) (E3). The E1 enzyme is a heterotetramer of 2 alpha and 2 beta subunits. The E1-alpha subunit contains the E1 active site and plays a key role in the function of the PDH complex .Pyruvate dehydrogenase (E1) performs the first two reactions within the pyruvate dehydrogenase complex (PDC): a decarboxylation of substrate 1 (pyruvate) and a reductive acetylation of substrate 2 (lipoic acid).

Organism species: Homo sapiens (Human)

PRODUCT TYPE CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPW036Hu01 Recombinant Pyruvate Dehydrogenase Alpha (PDHa) Positive Control; Immunogen; SDS-PAGE; WB.
Antibodies MAW036Hu01 Monoclonal Antibody to Pyruvate Dehydrogenase Alpha (PDHa) WB; IHC; ICC; IP.
PAW036Hu01 Polyclonal Antibody to Pyruvate Dehydrogenase Alpha (PDHa) WB; IHC; ICC; IF.
Assay Kits CLW036Hu01 CLIA Kit for Pyruvate Dehydrogenase Alpha (PDHa) CLIA assay for Antigen Detection
ELW036Hu01 ELISA Kit for Pyruvate Dehydrogenase Alpha (PDHa) Enzyme-linked immunosorbent assay for Antigen Detection

Organism species: Mus musculus (Mouse)

PRODUCT TYPE CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPW036Mu01 Recombinant Pyruvate Dehydrogenase Alpha (PDHa) Positive Control; Immunogen; SDS-PAGE; WB.
Antibodies MAW036Mu01 Monoclonal Antibody to Pyruvate Dehydrogenase Alpha (PDHa) WB; IHC; ICC; IP.
PAW036Mu01 Polyclonal Antibody to Pyruvate Dehydrogenase Alpha (PDHa) WB; IHC; ICC; IF.
Assay Kits CLW036Mu01 CLIA Kit for Pyruvate Dehydrogenase Alpha (PDHa) CLIA assay for Antigen Detection
ELW036Mu01 ELISA Kit for Pyruvate Dehydrogenase Alpha (PDHa) Enzyme-linked immunosorbent assay for Antigen Detection

Organism species: Rattus norvegicus (Rat)

PRODUCT TYPE CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPW036Ra01 Recombinant Pyruvate Dehydrogenase Alpha (PDHa) Positive Control; Immunogen; SDS-PAGE; WB.
Antibodies MAW036Ra01 Monoclonal Antibody to Pyruvate Dehydrogenase Alpha (PDHa) WB; IHC; ICC; IP.
PAW036Ra01 Polyclonal Antibody to Pyruvate Dehydrogenase Alpha (PDHa) WB; IHC; ICC; IF.
Assay Kits CLW036Ra01 CLIA Kit for Pyruvate Dehydrogenase Alpha (PDHa) CLIA assay for Antigen Detection
ELW036Ra01 ELISA Kit for Pyruvate Dehydrogenase Alpha (PDHa) Enzyme-linked immunosorbent assay for Antigen Detection

Organism species: Canis familiaris; Canine (Dog)

PRODUCT TYPE CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPW036Ca01 Recombinant Pyruvate Dehydrogenase Alpha (PDHa) Positive Control; Immunogen; SDS-PAGE; WB.
Antibodies MAW036Ca01 Monoclonal Antibody to Pyruvate Dehydrogenase Alpha (PDHa) WB; IHC; ICC; IP.
PAW036Ca01 Polyclonal Antibody to Pyruvate Dehydrogenase Alpha (PDHa) WB; IHC; ICC; IF.
Assay Kits CLW036Ca01 CLIA Kit for Pyruvate Dehydrogenase Alpha (PDHa) CLIA assay for Antigen Detection
ELW036Ca01 ELISA Kit for Pyruvate Dehydrogenase Alpha (PDHa) Enzyme-linked immunosorbent assay for Antigen Detection

Organism species: Sus scrofa; Porcine (Pig)

PRODUCT TYPE CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPW036Po01 Recombinant Pyruvate Dehydrogenase Alpha (PDHa) Positive Control; Immunogen; SDS-PAGE; WB.
Antibodies MAW036Po01 Monoclonal Antibody to Pyruvate Dehydrogenase Alpha (PDHa) WB; IHC; ICC; IP.
PAW036Po01 Polyclonal Antibody to Pyruvate Dehydrogenase Alpha (PDHa) WB; IHC; ICC; IF.
Assay Kits CLW036Po01 CLIA Kit for Pyruvate Dehydrogenase Alpha (PDHa) CLIA assay for Antigen Detection
ELW036Po01 ELISA Kit for Pyruvate Dehydrogenase Alpha (PDHa) Enzyme-linked immunosorbent assay for Antigen Detection

Organism species: Bos taurus; Bovine (Cattle)

PRODUCT TYPE CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPW036Bo01 Recombinant Pyruvate Dehydrogenase Alpha (PDHa) Positive Control; Immunogen; SDS-PAGE; WB.
Antibodies MAW036Bo01 Monoclonal Antibody to Pyruvate Dehydrogenase Alpha (PDHa) WB; IHC; ICC; IP.
PAW036Bo01 Polyclonal Antibody to Pyruvate Dehydrogenase Alpha (PDHa) WB; IHC; ICC; IF.
Assay Kits CLW036Bo01 CLIA Kit for Pyruvate Dehydrogenase Alpha (PDHa) CLIA assay for Antigen Detection
ELW036Bo01 ELISA Kit for Pyruvate Dehydrogenase Alpha (PDHa) Enzyme-linked immunosorbent assay for Antigen Detection

Organism species: Oryctolagus cuniculus (Rabbit)

PRODUCT TYPE CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPW036Rb01 Recombinant Pyruvate Dehydrogenase Alpha (PDHa) Positive Control; Immunogen; SDS-PAGE; WB.
Antibodies MAW036Rb01 Monoclonal Antibody to Pyruvate Dehydrogenase Alpha (PDHa) WB; IHC; ICC; IP.
PAW036Rb01 Polyclonal Antibody to Pyruvate Dehydrogenase Alpha (PDHa) WB; IHC; ICC; IF.
Assay Kits CLW036Rb01 CLIA Kit for Pyruvate Dehydrogenase Alpha (PDHa) CLIA assay for Antigen Detection
ELW036Rb01 ELISA Kit for Pyruvate Dehydrogenase Alpha (PDHa) Enzyme-linked immunosorbent assay for Antigen Detection

Organism species: Rhesus monkey (Simian)

PRODUCT TYPE CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPW036Mo01 Recombinant Pyruvate Dehydrogenase Alpha (PDHa) Positive Control; Immunogen; SDS-PAGE; WB.
Antibodies MAW036Mo01 Monoclonal Antibody to Pyruvate Dehydrogenase Alpha (PDHa) WB; IHC; ICC; IP.
PAW036Mo01 Polyclonal Antibody to Pyruvate Dehydrogenase Alpha (PDHa) WB; IHC; ICC; IF.
Assay Kits CLW036Mo01 CLIA Kit for Pyruvate Dehydrogenase Alpha (PDHa) CLIA assay for Antigen Detection
ELW036Mo01 ELISA Kit for Pyruvate Dehydrogenase Alpha (PDHa) Enzyme-linked immunosorbent assay for Antigen Detection

Reference

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"An amino acid substitution in the pyruvate dehydrogenase E1 alpha gene, affecting mitochondrial import of the precursor protein."
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"Mutation analysis of the pyruvate dehydrogenase E1 alpha gene in eight patients with a pyruvate dehydrogenase complex deficiency."
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"Three new mutations of the pyruvate dehydrogenase alpha subunit: a point mutation (M181V), 3 bp deletion (-R282), and 16 bp insertion/frameshift (K358SVS-->TVDQS)."
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"Biochemical and molecular analysis of an X-linked case of Leigh syndrome associated with thiamin-responsive pyruvate dehydrogenase deficiency."
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"Arginine 302 mutations in the pyruvate dehydrogenase E1alpha subunit gene: identification of further patients and in vitro demonstration of pathogenicity."
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