PDHX polyclonal Antibody | BS8207

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: