Background:
Biotin, also known as Anti-BTD抗体vitamin B7, is an essential water-soluble vitamin that is a cofactor in glucogenesis and in the metabolism of fatty acids and leucine. Biotinidase is a 523 amino acid enzyme that catalyzes the hydrolysis of biocytin to biotin and lysine. Secreted into extracellular space, biotinidase is expressed in liver, heart, placenta, brain, skeletal muscle, pancreas and kidney. Biotinidase contains one carbon-nitrogen hydrolase domain, which is involved in the reduction of organic nitrogen compounds and ammonia production. Defects in the gene encoding biotinidase are the cause of biotinidase deficiency, which is characterized by skin rash, ataxia, seizures, hearing loss, hypotonia and optic atrophy. These symptoms are due to the individual’s inability to reutilize biotin and can, therefore, Anti-BTD抗体typically be treated with the addition of free biotin.
Specificity:
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Rabbit Polyclonal IgG, Anti-BTD抗体affinity purified by Protein A.
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Reacts with: Human, Mouse, Rat, Chicken, Dog, Cow, Horse, Rabbit, Sheep, .
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Immunogen: KLH conjugated synthetic peptide derived from human Biotinidase.
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Predicted Molecular Weight: 57kDa.