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英文名字：Biotinidase Activity MaxSpec® Assay Kit
货号 : 27544
规格：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
Cayman Chemcial从成立到现在一直深耕于癌症，凋亡，表观遗传学，神经科学，炎症，内分泌等领域。截止到目前，Cayman Chemcial也一直在不断地开发新产品来满足客户各式各样的需求，我们的愿景是提供性价比高的产品，让科研更方便更省心。Cayman Chemical扩充了其在分析试剂盒、有机化学及生物化学领域的研发实力，并成立了一个新的实验室专门用于GMP生产，同时还通过了ISO 9001, ISO 14001, and OHSAS 18001 的认证。
产品分类：
抗体
代谢相关试剂盒
氧化损伤试相关剂盒
药物筛选试剂盒
内分泌相关试剂盒
炎症相关试剂盒
动脉粥样硬化试剂盒
抑制剂类化合物
生物抑制剂类化合物
拮抗剂类化合物
激动剂类化合物
标准品类化合物
激素类化合物
脂类化合物
氧化还原剂类化合物
神经药物类化合物
信号通路小分子类
其他生化试剂等
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