Full Product Name
Anti-PDHA1 Rabbit pAb
Product Synonym Names
Pyruvate dehydrogenase E1 component subunit alpha; somatic form; mitochondrial; PDHE1-A type I; PDHA1; PHE1A
Product Gene Name
anti-PDHA1 antibody
[Similar Products]
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
3D Structure
ModBase 3D Structure for P08559
Species Reactivity
Human, Mouse, Rat
Specificity
PDHA1 Antibody detects endogenous levels of total PDHA1
Purity/Purification
Affinity Chromatography
Form/Format
Phosphate buffered saline, pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol.
Immunogen
A synthesized peptide derived from human PDHA1
Subcellular Location
Mitochondrion matrix
Preparation and Storage
Store at 4 degree C for short term.
Store at-20 degree C for long term.
Avoid freeze/thaw cycle.
Other Notes
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.
Related Product Information for
anti-PDHA1 antibody
The PDH complex is composed of multiple copies of three enzymatic components: pyruvate dehydrogenase (E1), dihydrolipoamide acetyltransferase (E2) and lipoamide dehydrogenase (E3). Catalyzes the removal of CO2 from pyruvate. Mutations in the alpha subunits of pyruvate dehydrogenase (E1) lead to congenital defects that are usually associated with lactic acidosis, neurodegeneration and early death.
Applications Tested/Suitable for anti-PDHA1 antibody
Western Blot (WB), Immunohistochemistry (IHC), Immunocytochemistry (ICC), Immunofluorescence (IF), Immunoprecipitation (IP), Flow Cytometry (FC/FACS)
Application Notes for anti-PDHA1 antibody
WB: 1:500-1:2000
IHC: 1:50-1:200
ICC/IF: 1:50-1:200
IP: 1:50
FC/FACS: 1:50
Western Blot (WB) of anti-PDHA1 antibody
Western blot analysis of PDHA1 expression in HeLa cell lysate.

NCBI/Uniprot data below describe general gene information for PDHA1. It may not necessarily be applicable to this product.
NCBI Accession #
NP_000275.1
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NCBI GenBank Nucleotide #
NM_000284.3
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UniProt Primary Accession #
P08559
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UniProt Secondary Accession #
Q53H41; Q5JPT8; Q9NP12; Q9UBJ8; Q9UBU0; Q9UNG4; Q9UNG5; A5YVE9; B2R5P7; B7Z3T7; B7Z3X5[Other Products]
UniProt Related Accession #
P08559[Other Products]
NCBI Official Full Name
pyruvate dehydrogenase E1 component subunit alpha, somatic form, mitochondrial isoform 1
NCBI Official Synonym Full Names
pyruvate dehydrogenase E1 alpha 1 subunit
NCBI Official Symbol
PDHA1 [Similar Products]
NCBI Official Synonym Symbols
PDHA; PDHAD; PHE1A; PDHCE1A
[Similar Products]
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 [Similar Products]
UniProt Synonym Gene Names
PHE1A [Similar Products]
NCBI Summary for PDHA1
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 Comments for PDHA1
The pyruvate dehydrogenase complex catalyzes the overall conversion of pyruvate to acetyl-CoA and CO2, and thereby links the glycolytic pathway to the tricarboxylic cycle.
Research Articles on PDHA1
1. T splice-site mutation in the pyruvate dehydrogenase-alpha 1 (PDHA1) gene.">Case demonstrates fetal akinesia deformation sequence due to a de novo hemizygous c.498C > T splice-site mutation in the pyruvate dehydrogenase-alpha 1 (PDHA1) gene.
Precautions
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Disclaimer
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