PDHX Rabbit Polyclonal Antibody

CAT#: TA362094

PDHX Antibody - middle region

Product Images

Host: Rabbit Target Name: PDHX Sample Tissue: Human PANC1 Whole Cell lysates Antibody Dilution: 1ug/ml

Specifications

Product Data

• Applications: WB
• Reactivities: Human
• Host: Rabbit
• Clonality: Polyclonal
• Immunogen: The immunogen is a synthetic peptide directed towards the middle region of human PDHX
• Specificity: Expected reactivity: Human
• Formulation: Liquid. Purified antibody supplied in 1x PBS buffer with 0.09% (w/v) sodium azide and 2% sucrose.
• Concentration: lot specific
• Purification: Affinity purified
• Conjugation: Unconjugated
• Storage: For short term use, store at 2-8°C up to 1 week. For long term storage, store at -20°C in small aliquots to prevent freeze-thaw cycles.
• Predicted Protein Size: 55 kDa
• Gene Name: pyruvate dehydrogenase complex component X
• Database Link:
  NP_001128496.1
  Entrez Gene 8050 Human
  UniProt ID O00330
• 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.
• Synonyms: DLDBP; E3BP; OPDX; PDX1; proX