PDHX
pyruvate dehydrogenase complex, component X
Sinonimi:
DLDBP, E3BP, OPDX, PDX1, proX
Specie:
Human PDHX (8050), Mouse Pdhx (27402), Rat Pdhx (311254), domestic cat PDHX (101095373), dog PDHX (475942), Zebrafish pdhx (393532), domestic guinea pig Pdhx (100732258), naked mole-rat Pdhx (101703743), Horse PDHX (100059260), chicken PDHX (423154)
ID UniProtKB:
ID gene:
- Human(8050) 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]
- Mouse(27402) pyruvate dehydrogenase complex, component X
- Rat(311254) pyruvate dehydrogenase complex, component X
- domestic cat(101095373) pyruvate dehydrogenase complex, component X
- dog(475942) pyruvate dehydrogenase complex, component X
- Zebrafish(393532) pyruvate dehydrogenase complex, component X
- domestic guinea pig(100732258) pyruvate dehydrogenase complex, component X
- naked mole-rat(101703743) pyruvate dehydrogenase complex, component X
- Horse(100059260) pyruvate dehydrogenase complex, component X
- chicken(423154) pyruvate dehydrogenase complex, component X
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Antibodies
N° Catalogo
Descrizione
Reattività con
Applicazione
Anti-PDHX antibody produced in mouse, IgG fraction of antiserum, buffered aqueous solution,
Reattività con
human
Applicazione
immunofluorescence
western blot
western blot
Anti-PDHX antibody produced in rabbit, Prestige Antibodies® Powered by Atlas Antibodies, affinity isolated antibody, buffered aqueous glycerol solution,
Reattività con
human
Applicazione
immunohistochemistry
western blot
western blot
Anti-PDHX antibody produced in rabbit, Prestige Antibodies® Powered by Atlas Antibodies, affinity isolated antibody, buffered aqueous glycerol solution,
Reattività con
human
Applicazione
immunohistochemistry
western blot
western blot
Kits
N° Catalogo
Descrizione
Specie
Pyruvate Dehydrogenase Activity Assay Kit, sufficient for 100 colorimetric tests,
Specie
rat, mouse, human