Antibody data
- Antibody Data
- Antigen structure
- References [0]
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- Validations
- Western blot [1]
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- 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.
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Supportive validation
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- Bioworld Technology, Inc (provider)
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- Experimental details
- WesternBlot (WB) analysis of PDHX polyclonal antibody