Description
PDHX polyclonal Antibody | BS8207 | Gentaur UK, US & Europe Distribution
Host: Rabbit
Reactivity: Human,Mouse,Rat
Application: WB IF
Application Range: WB 1:500 - 1:2000 IF 1:10 - 1:100
Background: The pyruvate dehydrogenase (PDH) complex is a nuclear-encoded mitochondrial matrix enzyme complex that functions as the primary link between glycolysis and the tricarboxylic acid (TCA) cycle by catalyzing the irreversible conversion of pyruvate into acetyl-CoA. E3BP (E3-binding protein), also known as PDHX (Pyruvate dehydrogenase protein X component) and Lipoyl-containing pyruvate dehydrogenase complex component X, is a 501 amino acid mitochondrial protein that is required for anchoring E3 to the E2 core of the PDH complex, an event that is essential for a functional PDH complex. Defects in the gene encoding E3BP result in pyruvate dehydrogenase E3-binding protein deficiency, which is similar to PDH deficiency and Leigh syndrome in clinical presentation. Symptoms of E3BP deficiency can include lactic acidosis, delayed development, seizures, diplegia, cerebellar ataxia, optic atrophy, facial dysmorphism and episodic weakness.
Storage & Stability: Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles.
Specificity: PDHX polyclonal Antibody detects endogenous levels of PDHX protein.
Molecular Weight: ~ 54 kDa
Note: For research use only, not for use in diagnostic procedure.
Alternative Names: Pyruvate dehydrogenase protein X component, mitochondrial; Dihydrolipoamide dehydrogenase-binding; protein of pyruvate dehydrogenase complex; E3-binding protein; E3BP; Lipoyl-containing pyruvate dehydrogenase complex component X; proX; PDHX; PDX1
Immunogen: Recombinant full length Human PDHX.
Conjugate: Unconjugated
Modification: Unmodification
Purification & Purity: The Antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is > 95% (by SDS-PAGE) .
Pathway: