全國服務熱線：
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
Cayman Chemcial從成立到現(xiàn)在一直深耕于癌癥，凋亡，表觀遺傳學，神經科學，炎癥，內分泌等領域。截止到目前，Cayman Chemcial也一直在不斷地開發(fā)新產品來滿足客戶各式各樣的需求，我們的愿景是提供性價比高的產品，讓科研更方便更省心。Cayman Chemical擴充了其在分析試劑盒、有機化學及生物化學領域的研發(fā)實力，并成立了一個新的實驗室專門用于GMP生產，同時還通過了ISO 9001, ISO 14001, and OHSAS 18001 的認證。
產品分類：
抗體
代謝相關試劑盒
氧化損傷試相關劑盒
藥物篩選試劑盒
內分泌相關試劑盒
炎癥相關試劑盒
動脈粥樣硬化試劑盒
抑制劑類化合物
生物抑制劑類化合物
拮抗劑類化合物
激動劑類化合物
標準品類化合物
激素類化合物
脂類化合物
氧化還原劑類化合物
神經藥物類化合物
信號通路小分子類
其他生化試劑等
更多Cayman Chemcial信息，請登錄https://www.caymanchem.com/或聯(lián)系南京澤維爾生物科技有限公司購買，我們保證產品質量，并擁有一流的服務和快捷的物流!
暫無數(shù)據(jù)
暫無數(shù)據(jù)
