General Notes
This product (Catalog No. DPABH-12773) is a rabbit-derived IgG polyclonal antibody raised against human PDHX, targeting the amino acid region 231–330 of the protein. This antibody exhibits cross-reactivity with PDHX from mouse, rat, and dog. It has been affinity purified by Protein A. 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.
DPABH-12773 is suitable for use in Western blot (WB) (1:500–1:2,000) application.
DPABH-12773 is supplied as a liquid formulation in 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. For long-term storage, keep at −20 °C. For short-term use, store at 2–8 °C. Avoid repeated freeze–thaw cycles to maintain antibody integrity and performance.
Alternative Names
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_HUMAN; 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
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