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Merck

GAA

glucosidase, alpha; acid

同義詞:
LYAG
物種:
UniProtKB ID:
基因 ID:
  • Human(2548) Summary: This gene encodes acid alpha-glucosidase, which is essential for the degradation of glycogen to glucose in lysosomes. Different forms of acid alpha-glucosidase are obtained by proteolytic processing. Defects in this gene are the cause of glycogen storage disease II, also known as Pompe's disease, which is an autosomal recessive disorder with a broad clinical spectrum. Three transcript variants encoding the same protein have been found for this gene. [provided by RefSeq, Jul 2008]
  • Mouse(14387) glucosidase, alpha, acid
  • Rat(367562) glucosidase, alpha, acid
  • Zebrafish(100002366) glucosidase, alpha; acid (Pompe disease, glycogen storage disease type II)
  • domestic cat(101086359) glucosidase, alpha; acid
  • cow(280798) glucosidase, alpha; acid
  • Horse(100056675) glucosidase, alpha; acid
  • dog(483352) glucosidase, alpha; acid
  • chicken(416462) glucosidase, alpha; acid (Pompe disease, glycogen storage disease type II)
  • sheep(101121692) glucosidase, alpha; acid
  • domestic guinea pig(100728030) glucosidase, alpha; acid
  • naked mole-rat(101718861) glucosidase, alpha; acid

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Antibodies

產品編號
描述
物種活性
應用
Monoclonal Anti-GAA antibody produced in mouse, clone 3C6, purified immunoglobulin, buffered aqueous solution,
物種活性
human
應用
ELISA
western blot
抗-GAA 兔抗, Prestige Antibodies® Powered by Atlas Antibodies, affinity isolated antibody, buffered aqueous glycerol solution,
物種活性
human
應用
immunohistochemistry
western blot
Anti-GAA antibody produced in rabbit, affinity isolated antibody,
物種活性
bovine, dog, guinea pig, human, mouse, rat
應用
immunohistochemistry
western blot
Anti-GAA antibody produced in mouse, purified immunoglobulin, buffered aqueous solution,
物種活性
human
應用
western blot

Small Molecule - Inhibitor

產品編號
描述
物種
1-Deoxynojirimycin hydrochloride, ,
物種
human
Voglibose, ≥97.0% (TLC),
物種
human
N-Methyl-1-deoxynojirimycin, ≥98%,
物種
mouse, rat
1,4-二脱氧-1,4-亚氨基-D-阿拉伯糖醇 盐酸盐, enzyme inhibitor,
物種
mouse, rat, human

Kits

產品編號
描述
物種
α-葡萄糖苷酶活性检测试剂盒, sufficient for 100 colorimetric tests,
物種
mouse, human, rat