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Amino acids 31-390 constitute the expression domain of recombinant Human PDHA1. The theoretical molecular weight of the PDHA1 protein is 47.5 kDa. This protein is generated in a e.coli-based system. The PDHA1 gene fragment has been modified by fusing the N-terminal 10xHis tag and C-terminal Myc tag, providing convenience in detecting and purifying the recombinant PDHA1 protein during the following stages.The human pyruvate dehydrogenase E1 component subunit alpha, somatic form, mitochondrial (PDHA1) is a key enzyme subunit involved in the pyruvate dehydrogenase complex, a crucial component of cellular energy metabolism. PDHA1 carries the E1 (pyruvate decarboxylase) enzymatic activity. PDHA1 plays a central role in regulating the entry of pyruvate into the citric acid cycle, influencing the overall rate of cellular energy production through aerobic respiration. Mutations in the PDHA1 gene can lead to pyruvate dehydrogenase complex deficiency, a rare genetic disorder associated with neurological and developmental abnormalities. PDHA1 deficiency is linked to metabolic disorders with neurological symptoms, such as Leigh syndrome. Understanding the structure and function of PDHA1 is crucial for elucidating the molecular basis of these disorders.
Amino acids 31-390 constitute the expression domain of recombinant Human PDHA1. The theoretical molecular weight of the PDHA1 protein is 47.5 kDa. This protein is generated in a e.coli-based system. The PDHA1 gene fragment has been modified by fusing the N-terminal 10xHis tag and C-terminal Myc tag, providing convenience in detecting and purifying the recombinant PDHA1 protein during the following stages.The human pyruvate dehydrogenase E1 component subunit alpha, somatic form, mitochondrial (PDHA1) is a key enzyme subunit involved in the pyruvate dehydrogenase complex, a crucial component of cellular energy metabolism. PDHA1 carries the E1 (pyruvate decarboxylase) enzymatic activity. PDHA1 plays a central role in regulating the entry of pyruvate into the citric acid cycle, influencing the overall rate of cellular energy production through aerobic respiration. Mutations in the PDHA1 gene can lead to pyruvate dehydrogenase complex deficiency, a rare genetic disorder associated with neurological and developmental abnormalities. PDHA1 deficiency is linked to metabolic disorders with neurological symptoms, such as Leigh syndrome. Understanding the structure and function of PDHA1 is crucial for elucidating the molecular basis of these disorders.
| Cat.No | ACP01996 | Target Name | PDHA1 |
|---|---|---|---|
| Target Synonyms | PDHE1-A type I | Form | Liquid or Lyophilized powder |
| Expression System | E.coli | Expression Range | 31-390aa |
| Mol Weight | 47.5 kDa | Protein Length | Partial |
| Purity | Greater than 85% as determined by SDS-PAGE. | Storage Buffer | 5%-50% glycerol. Lyophilized powder form: the buffer before lyophilization is Tris/PBS-based buffer, 6% Trehalose, Liquid form: default storage buffer is Tris/PBS-based buffer, pH 8.0. |
| Target Species | Human | Uniprot ID | P08559 |
|---|
Uniprot Id
P08559
Target Species
Human
Target Name
PDHA1
Target Full Name
Pyruvate dehydrogenase E1 component subunit alpha, somatic form, mitochondrial
Target Function
The pyruvate dehydrogenase complex catalyzes the overall conversion of pyruvate to acetyl-CoA and CO(2), and thereby links the glycolytic pathway to the tricarboxylic cycle.
Target Involvement
Pyruvate dehydrogenase E1-alpha deficiency (PDHAD)
Target Subcellular Location
Mitochondrion matrix.
Target Tissue Specificity
Ubiquitous.
Target Research Area
Metabolism
Target Synonyms
ODPA_HUMAN; PDH; PDHA; PDHA1; PDHCE1A; PDHE1 A type I ; PDHE1-A type I; PHE1A; Pyruvate Dehydrogenase (lipoamide) alpha 1; Pyruvate dehydrogenase complex, E1 alpha polypeptide 1; Pyruvate Dehydrogenase E1 alpha; Pyruvate dehydrogenase E1 component subunit alpha; Pyruvate dehydrogenase E1 component subunit alpha, somatic form, mitochondrial
Target Background
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.
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