The PDHX Polyclonal Antibody (CAB6426) is an essential tool for researchers studying the Pyruvate Dehydrogenase Complex (PDC), a key enzyme complex involved in energy production through the citric acid cycle. This antibody, produced in rabbits, is highly sensitive and specific for detecting PDHX protein in human samples, making it ideal for Western blot analysis.PDHX is a crucial component of the PDC, playing a critical role in the conversion of pyruvate to acetyl-CoA, a fundamental step in cellular energy metabolism.
Dysregulation of this process has been linked to various metabolic disorders, making PDHX a promising target for therapeutic interventions. By using the PDHX Polyclonal Antibody, researchers can further explore the functions and regulation of PDHX in health and disease, advancing our understanding of metabolic pathways and potential treatment strategies.
Product Name:
PDHX Rabbit Polyclonal Antibody
SKU:
CAB6426
Size:
20uL, 100uL
Isotype:
IgG
Host Species:
Rabbit
Reactivity:
Human,Mouse,Rat
Immunogen:
Recombinant fusion protein containing a sequence corresponding to amino acids 1-300 of human PDHX (NP_003468.2).
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.
Purification Method:
Affinity purification
Gene ID:
8050
Storage Buffer:
Store at -20℃. Avoid freeze / thaw cycles.Buffer: PBS with 0.02% sodium azide,50% glycerol,pH7.3.
Western blot analysis of various lysates using PDHX Rabbit pAb (CAB6426) at 1:1000 dilution.Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (CABS014) at 1:10000 dilution.Lysates/proteins: 25μg per lane.Blocking buffer: 3% nonfat dry milk in TBST.Detection: ECL Basic Kit (AbGn00020).Exposure time: 5s.