BS8207

antibody from Bioworld Technology, Inc
Targeting: PDHX DLDBP, E3BP, OPDX, PDX1, proX
Provider product page for BS8207
Western blot
Immunohistochemistry

Antibody data

Product number
BS8207 - Provider product page
Provider
Bioworld Technology, Inc
Product name
PDHX polyclonal antibody
Antibody type
Polyclonal
Antigen
Recombinant full length Human PDHX.
Description
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.
Reactivity
Human
Host
Rabbit
Isotype
IgG
Vial size
100ul
Concentration
1 mg/ml
Storage
Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles.
PDHX protein structure - BS8207 shown in red.