PDHX Antibody
DLDBP, E3BP, OPDX, PDX1, proX, Component X.
Description
Product Specs
The Pyruvate Dehydrogenase Complex, Component X, often referred to as PDHX, is responsible for encoding the E3 binding protein subunit within the PDH complex. This complex is composed of three catalytic subunits. PDHX's primary function is to connect E3 dimers to the E2 core of pyruvate dehydrogenase complexes found in eukaryotes. This specific binding process is crucial for the proper functioning of the PDH complex.
The solution is provided at a concentration of 1mg/ml and contains PBS with a pH of 7.4, 10% Glycerol, and 0.02% Sodium Azide.
The PDHX antibody has undergone rigorous testing using both ELISA and Western blot analysis to confirm its specificity and reactivity. However, it's important to note that optimal results may vary depending on the specific application. Therefore, it is recommended to titrate the reagent for each unique investigation.
DLDBP, E3BP, OPDX, PDX1, proX, Component X.
PDHX antibody was purified from mouse ascitic fluids by protein-A affinity chromatography.
PAT1E11AT
Anti-human PDHX mAb, is derived from hybridization of mouse F0 myeloma cells with spleen cells from BALB/c mice immunized with recombinant human PDHX amino acids 54-501 purified from E. coli.
Mouse IgG1 heavy chain and κ light chain.
Product Science Overview
The Pyruvate Dehydrogenase Complex (PDC) is a crucial multi-enzyme complex that plays a pivotal role in cellular respiration. It catalyzes the conversion of pyruvate into acetyl-CoA, thereby linking glycolysis to the citric acid cycle. This complex is composed of multiple subunits, including the E1, E2, and E3 catalytic subunits, as well as regulatory subunits and a non-catalytic subunit known as Component X or E3 Binding Protein (E3BP).
The PDC is located in the mitochondrial matrix and is essential for energy production in cells. The E1 subunit, also known as pyruvate dehydrogenase, is responsible for the decarboxylation of pyruvate. The E2 subunit, dihydrolipoyl transacetylase, transfers the resulting acetyl group to CoA, forming acetyl-CoA. The E3 subunit, dihydrolipoyl dehydrogenase, regenerates the oxidized form of lipoamide, a cofactor required for the E2 subunit’s activity .
Component X, also known as the E3 Binding Protein (E3BP), is a non-catalytic subunit that plays a crucial role in the structural integrity and function of the PDC. It tethers the E3 dimers to the E2 core of the complex, ensuring proper assembly and stability . This protein is encoded by the PDHX gene and is essential for the proper functioning of the PDC.
Mutations in the PDHX gene can lead to pyruvate dehydrogenase deficiency, a metabolic disorder characterized by neurological dysfunction and lactic acidosis. This condition typically presents in infancy or early childhood and can result in severe developmental delays and other health issues .
Component X is also a minor antigen for antimitochondrial antibodies, which are present in nearly 95% of patients with primary biliary cirrhosis (PBC). PBC is an autoimmune liver disease where activated T lymphocytes attack and destroy epithelial cells in the bile duct, leading to cirrhosis and liver failure .
Mouse anti-human antibodies are commonly used in research to study the function and structure of human proteins. These antibodies are produced by immunizing mice with human proteins or peptides, leading to the generation of specific antibodies that can bind to the target protein. In the case of Component X, mouse anti-human antibodies can be used to investigate its role in the PDC and its involvement in various diseases .
