product name
4-甲基伞形酮 α- D -吡喃葡萄糖苷, α-glucosidase substrate
质量水平
描述
α-glucosidase substrate
检测方案
≥99% (TLC)
形式
powder
溶解性
pyridine: 50 mg/mL, clear, colorless to faintly yellow
储存温度
−20°C
SMILES字符串
CC1=CC(=O)Oc2cc(O[C@H]3O[C@H](CO)[C@@H](O)[C@H](O)[C@H]3O)ccc12
InChI
1S/C16H18O8/c1-7-4-12(18)23-10-5-8(2-3-9(7)10)22-16-15(21)14(20)13(19)11(6-17)24-16/h2-5,11,13-17,19-21H,6H2,1H3/t11-,13-,14+,15-,16+/m1/s1
InChI key
YUDPTGPSBJVHCN-JZYAIQKZSA-N
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应用
4-甲基伞形基ǥ-D-吡喃葡糖苷已用于测定组织匀浆中的酸性 α-葡萄糖苷酶（GAA）活性。
生化/生理作用
4-甲基伞形基ǥ-D-吡喃葡糖苷是 ǥ-葡萄糖苷酶的荧光底物。产物 4-甲基伞形花酯在荧光光谱中显示出 440nm 处的峰值。
WGK
WGK 3
闪点(°F)
Not applicable
闪点(°C)
Not applicable
个人防护装备
Eyeshields, Gloves, type N95 (US)
法规信息
监管及禁止进口产品
Intrapleural administration of AAV9 improves neural and cardiorespiratory function in Pompe disease.
Darin J Falk et al.
Molecular therapy : the journal of the American Society of Gene Therapy, 21(9), 1661-1667 (2013-06-05)
Pompe disease is a neuromuscular disease resulting from deficiency in acid α-glucosidase (GAA), results in cardiac, skeletal muscle, and central nervous system (CNS) pathology. Enzyme replacement therapy (ERT) has been shown to partially correct cardiac and skeletal muscle dysfunction. However
Omid Motabar et al.
Analytical biochemistry, 390(1), 79-84 (2009-04-18)
Mutations in alpha-glucosidase cause accumulation of glycogen in lysosomes, resulting in Pompe disease, a lysosomal storage disorder. Small molecule chaperones that bind to enzyme proteins and correct the misfolding and mistrafficking of mutant proteins have emerged as a new therapeutic
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Human gene therapy, 27(1), 43-59 (2015-11-26)
Pompe disease is a progressive neuromuscular disorder caused by lysosomal accumulation of glycogen from a deficiency in acid alpha-glucosidase (GAA). Replacement of the missing enzyme is available by repeated protein infusions; however, efficacy is limited by immune response and inability
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