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Anti-Phospho-PDHA1-S293 Antibody (CABP1250)

SKU:
CABP1250
Product Type:
Antibody
Antibody Type:
Polyclonal Antibody
Reactivity:
Human
Mouse
Rat
Host Species:
Rabbit
Isotype:
IgG
Synonyms:
PDHA
PDHAD
PDHCE1A
PHE1A
Research Area:
Metabolism
$395 - $503
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Description

Product Name:Phospho-PDHA1-S293 Rabbit pAb
Product Code:CABP1250
Size:50uL, 100uL
Synonyms:PDHA, PDHAD, PDHCE1A, PHE1A
Applications:WB
Reactivity:Human, Mouse, Rat
Host Species:Rabbit
Immunogen:A phospho specific peptide corresponding to residues surrounding S293 of human PDHA1
Applications:WB
Recommended Dilutions:WB 1:500 - 1:2000
Reactivity:Human, Mouse, Rat
Positive Samples:293T, NIH/3T3
Immunogen:A phospho specific peptide corresponding to residues surrounding S293 of human PDHA1
Purification Method:Affinity purification
Storage:Store at -20°C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 0.05% BSA, 50% glycerol, pH7.3.
Isotype:IgG
Sequence:Email for sequence
Gene ID:5160
Uniprot:P08559
Cellular Location:Mitochondrion matrix
Calculated MW:40kDa
Observed MW:43KDa
UniProt Protein Function: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.
UniProt Protein Details:

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

Chromosomal Location of Human Ortholog: Xp22.1

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

Molecular Function:pyruvate dehydrogenase activity

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

Disease: Pyruvate Dehydrogenase E1-alpha Deficiency

NCBI Summary: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]
UniProt Code:P08559
NCBI GenInfo Identifier:129063
NCBI Gene ID:5160
NCBI Accession:P08559.3
UniProt Secondary Accession:P08559,Q53H41, Q5JPT8, Q9NP12, Q9UBJ8, Q9UBU0, Q9UNG4 Q9UNG5, A5YVE9, B2R5P7, B7Z3T7, B7Z3X5,
UniProt Related Accession:P08559
Molecular Weight:47,580 Da
NCBI Full Name:Pyruvate dehydrogenase E1 component subunit alpha, somatic form, mitochondrial
NCBI Synonym Full Names:pyruvate dehydrogenase (lipoamide) alpha 1
NCBI Official Symbol:PDHA1  
NCBI Official Synonym Symbols:PDHA; PDHAD; PHE1A; PDHCE1A  
NCBI Protein Information:pyruvate dehydrogenase E1 component subunit alpha, somatic form, mitochondrial
UniProt Protein Name:Pyruvate dehydrogenase E1 component subunit alpha, somatic form, mitochondrial
UniProt Synonym Protein Names:PDHE1-A type I
Protein Family:Pyruvate dehydrogenase E1 component
UniProt Gene Name:PDHA1  
UniProt Entry Name:ODPA_HUMAN

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