Pages
Products

PDHX


Official Full Name
pyruvate dehydrogenase complex component X
Organism
Homo sapiens
Gene ID
8050
Background
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]
Synonyms
E3BP; OPDX; PDX1; proX; DLDBP; PDHXD

Cat.No. Product Name Price
SHH372756 shRNA set against Human PDHX (NM_003477.2) Inquiry
SHH372764 shRNA set against Rat PDHX (NM_001044242.1) Inquiry
SHW001794 shRNA set against Chicken PDHX (NM_001031187) Inquiry
SHW016726 shRNA set against Danio rerio PDHX (NM_200560) Inquiry
Cat.No. Product Name Price
CDCL147621 Human PDHX ORF clone (NM_001166158.1) Inquiry
CDCR371800 Rat Pdhx ORF Clone(NM_001044242.1) Inquiry
CDFH013982 Human PDHX cDNA Clone(NM_001135024.1) Inquiry
CDFH013983 Human PDHX cDNA Clone(NM_001166158.1) Inquiry
CDFR004767 Rat Pdhx cDNA Clone(NM_001044242.1) Inquiry
MiUTR1H-07738 PDHX miRNA 3'UTR clone Inquiry
MiUTR1R-03162 PDHX miRNA 3'UTR clone Inquiry
MiUTR3H-10214 PDHX miRNA 3'UTR clone Inquiry
MiUTR3H-10215 PDHX miRNA 3'UTR clone Inquiry
CDCB163269 Chicken PDHX ORF Clone (NM_001031187) Inquiry
CDCB178201 Danio rerio PDHX ORF Clone (NM_200560) Inquiry
CDCB187984 Rabbit PDHX ORF clone (XM_008269678.1) Inquiry
CDCL147617 Mouse PDHX ORF clone (NM_001135024.1) Inquiry
CDCL147619 Human PDHX ORF clone (NM_003477.2) Inquiry
CDCR272508 Mouse Pdhx ORF Clone(NM_175094.5) Inquiry
CDCS408037 Human PDHX ORF Clone (BC010389) Inquiry
Quick Inquiry