ODPX rabbit pAb

Cat :A18787

Price： ¥900¥1600

Size： 50ul 100ul

Datasheet

MSDS

Source

Rabbit
Applications

WB
Reactivity

Human, Mouse
Dilution

WB 1：500-2000
Storage

-20°C/1 year
Specificity

This antibody detects endogenous levels of ODPX at Human/Mouse
Source/Purification

The antibody was affinity-purified from rabbit serum by affinity-chromatography using specific immunogen.
Immunogen

Synthesized peptide derived from human ODPX
Uniprot No

O00330
Form

Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
Clonality

Polyclonal
Isotype

IgG
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.[provided by RefSeq, Oct 2009],
Other

PDHX PDX1, ODPX
Mol.Wt (Da)

55110
Concentration

1 mg/ml