我们是Lysosomal Alpha Glucosidase (GAA) Antibody(货号:abx112735)的供应商,欢迎通过生意宝平台采购Lysosomal Alpha Glucosidase (GAA) Antibody(货号:abx112735),北京裕恒丰科技有限公司
Acid alpha-glucosidase (GAA) is an enzyme that is essential in the degradation of glycogen to glucose in the lysosome (1). Defects in GAA are the cause of glycogen storage disease II, also known as Pompe's disease, which is a rare autosomal recessive metabolic disorder that damages muscle ...
BACKGROUND Acid alphaglucosidase (GAA) is an enzyme that is essential in the degradation of glycogen to glucose in the lysosome (1). Defects in GAA are the cause of glycogen storage disease II, also known as ...
Rat Lysosomal Alpha Glucosidase (GAA) Protein 供应商 Abbexa 产品货号 abx066803 产品报价 ￥询价/10ug ￥询价/50ug ￥询价/100ug ￥询价/200ug ￥询价/1mg 产品说明书 点击查看 购买方式 银行转账、电汇、支票、现金,在线支付宝及网银支付,或直接与我们电话联系400-6800-868。
Basic informationMore Product Name:LYSOSOMAL ALPHA-GLUCOSIDASE S AND IS Synonyms:LYSOSOMAL ALPHA-GLUCOSIDASE S AND IS CAS: MF: MW:0 EINECS: Mol File:Mol File Please select the suppliers Recommend You Select Member Companies Company Name:Shanghai isotope chemical co.,ltd ...
描述: Inhibition of human lysosomal alpha glucosidase assessed as release of p-nitrophenol at 1000 uM using p-nitrophenyl glycoside as substrate by spectrometric assay relative to control Format: BAO_0000357 Organism: Homo sapiens Target: Lysosomal alpha-glucosidase (ALA2608) Document: ALA5131499 In...
The gene coding for human lysosomal alpha-glucosidase was cloned and its structure was determined. The gene is approx. 20 kb long, and contains 20 exons. The first exon is non-coding. The coding sequence of the putative catalytic site domain is interrupted in the middle by an intron of 101...
1978. The molecular heterogeneity of purified hu- man liver lysosomal a-glucosidase (Acid a-glucosidase). Arch. Bioehem. Biophys. 185: 511- 524Murray AK, Brown BI, Brown DH (1978) The molecular heterogeneity of purified human liver lysosomal alpha-glucosidase (acid alpha-glucosidase). Arch ...
· Alpha-岩藻糖苷沉积症(Alpha-fucosidosis):临床症状包括进展性神经运动恶化(progressive neuromotor deterioration),惊厥(包括肌痉挛性发作,myoclonic seizures),粗面容(coarse facial features),骨骼营养不良(dysostosis multiplex),弥漫性躯体性血管角化瘤(angiokeratoma corporis diffusum),肝脾肿大和发育迟缓。
Metrics Abstract Pompe disease, a rare lysosomal storage disease caused by deficiency of the lysosomal acid α-glucosidase (GAA), is characterized by glycogen accumulation, triggering severe secondary cellular damage and resulting in progressive motor handicap and premature death. Numerous disease-causing ...