全國服務(wù)熱線：
18852077652
英文名字：Biotinidase Activity MaxSpec® Assay Kit
貨號 : 27544
規(guī)格：1 ea
品牌：Cayman
Technical Information
Biotinidase is a peptidyl hydrolase that cleaves biocytin and biotinylated peptides in vivo to generate free biotin, an essential coenzyme for certain carboxylases involved in fatty acid, amino acid, and glucose metabolism.1,2 Early-onset biotinidase deficiency is due to deletion, insertion, substitution, or missense mutations in the biotinidase gene, BTD, that affect the activity of multiple carboxylases. This profound biotinidase deficiency results in seizures, ataxia, sensorineural hearing loss or vision loss, eczema, and respiratory disruptions without treatment. Partial biotinidase deficiency is due to a substitution mutation in the BTD gene resulting in a 70-90% reduction in biotinidase activity leading to symptom manifestation that may not occur until adolescence and occurs only under biological stress. Many symptoms resolve with treatment, but retinal degeneration, sensorineural hearing loss, and developmental delays cannot be reversed. Early detection of biotinidase deficiency is imperative to avoid permanent damage.
Shipping & Storage Information
Storage：-20°C
Shipping：Wet ice in continental US; may vary elsewhere Stability ≥ 1 year
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