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Home Recombinant Human Proteins All PDHX Proteins

PDHX (NM_003477) Purified Human Protein

Specifications Citations Customer Service Product Documents
Cat. No. Description Datasheet Price Availability*  
TP303138 Recombinant protein of human pyruvate dehydrogenase complex, component X (PDHX), nuclear gene encoding mitochondrial protein, transcript variant 1, 20 ug (Larger pack size?) $680 In Stock
TA302585 Goat Polyclonal Antibody against Pyruvate dehydrogenase / PDHX $325 3-7 Days
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OriGene Data
Species:Human Expression Host:HEK293
Expression cDNA Clone or AA Sequence
Recombinant protein was produced with TrueORF clone, RC203138. Click on the TrueORF clone link to view cDNA and protein sequences.
Tag:C-terminal MYC/DDK Predicted MW:53.9 kDa
Purity:> 80% as determined by SDS-PAGE and Coomassie blue staining
Concentration:>50 ug/mL as determined by microplate BCA method
Buffer:25 mM Tris.HCl, pH 7.3, 100 mM glycine, 10% glycerol.
Preparation: Recombinant protein was captured through anti-DDK affinity column followed by conventional chromatography steps.
Protein Families:
Protein Pathways:
Protein Data
Protein Image
 

Reference Data
RefSeq: NP_003468 RefSeq Size: 2365 RefSeq ORF: 1506
LocusID: 8050 Cytogenetic: 11p13  
Synonyms : DLDBP; E3BP; OPDX; PDX1; proX
Summary: The pyruvate dehydrogenase (PDH) complex is located in the mitochondrial matrix and catalyzes the conversion of pyruvate to acetyl coenzyme A. The PDH complex thereby links glycolysis to Krebs cycle. The PDH complex contains three catalytic subunits, E1, E2, and E3, two regulatory subunits, E1 kinase and E1 phosphatase, and a non-catalytic subunit, E3 binding protein (E3BP). This gene encodes the E3 binding protein subunit; also known as component X of the pyruvate dehydrogenase complex. This protein tethers E3 dimers to the E2 core of the PDH complex. Defects in this gene are a cause of pyruvate dehydrogenase deficiency which results in neurological dysfunction and lactic acidosis in infancy and early childhood. This protein is also a minor antigen for antimitochondrial antibodies. These autoantibodies are present in nearly 95% of patients with the autoimmune liver disease primary biliary cirrhosis (PBC). In PBC, activated T lymphocytes attack and destroy epithelial cells in the bile duct where this protein is abnormally distributed and overexpressed. PBC eventually leads to cirrhosis and liver failure. Alternative splicing results in multiple transcript variants encoding distinct isoforms.[provided by RefSeq, Oct 2009].
*: Inventory for certain proteins may be limited due to low expression level. Delivery time may vary from web posted schedule. Contact techsupport@origene.com for specific inventory information
**: DDK-tag is the same as FLAG tag. Flag® is a registered trademark of Sigma-Aldrich

 

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