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.
仕様
Synonyms：Dihydrolipoamide dehydrogenase binding protein of pyruvate dehydrogenase complex,Dihydrolipoamide dehydrogenase-binding protein of pyruvate dehydrogenase complex,DLDBP,E3 binding protein,E3-binding protein,E3BP,Lipoyl containing pyruvate dehydrogenase complex component X,Lipoyl-containing pyruvate dehydrogenase complex component X,mitochondrial,ODPX,OPDX,PDHX,PDX 1,PDX1,Pro X,proX,Pyruvate dehydrogenase complex component X,Pyruvate dehydrogenase complex lipoyl containing component X,Pyruvate dehydrogenase complex, E3 binding protein subunit,Pyruvate dehydrogenase protein X component,Pyruvate dehydrogenase protein X component mitochondrial
Host：Rabbit
Reactivity：Human,Mouse,Rat
Applications：WB,IF
Concentration：1mg/ml
Immunogen：Recombinant protein of human PDHX
Purification Method：Affinity purification
Clonality：Polyclonal
Conjugation：Unconjugated
Buffer：Buffer： PBS with 0.02％ sodium azide, 50％ glycerol, pH7.3.
Dilution：WB1：500 - 1：2000 IF1：10 - 1：100
Gene_ID（human）：8050
Swissprot：O00330
Isotype：IgG
Research Areas：Cancer, Metabolism, Signal transduction
