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GAA

glucosidase, alpha; acid

別名:
LYAG
Species:
UniProtKB ID:
遺伝子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

製品番号
詳細
交差性
アプリケーション
Monoclonal Anti-GAA antibody produced in mouse, clone 3C6, purified immunoglobulin, buffered aqueous solution,
交差性
human
アプリケーション
ELISA
western blot
抗GAA抗体 ウサギ宿主抗体, Prestige Antibodies® Powered by Atlas Antibodies, affinity isolated antibody, buffered aqueous glycerol solution,
交差性
human
アプリケーション
immunohistochemistry
western blot
抗GAA ウサギ宿主抗体, affinity isolated antibody,
交差性
bovine, dog, guinea pig, human, mouse, rat
アプリケーション
immunohistochemistry
western blot
抗GAA ウサギ宿主抗体, Prestige Antibodies® Powered by Atlas Antibodies, affinity isolated antibody, buffered aqueous glycerol solution,
交差性
human
アプリケーション
immunohistochemistry
western blot
抗GAA抗体 マウス宿主抗体, purified immunoglobulin, buffered aqueous solution,
交差性
human
アプリケーション
western blot

Small Molecule - Inhibitor

製品番号
詳細
Species
1-デオキシノジリマイシン 塩酸塩, ,
Species
human
ボグリボース, ≥97.0% (TLC),
Species
human
N-メチル-1-デオキシノジリマイシン, ≥98%,
Species
mouse, rat
1,4-ジデオキシ-1,4-イミノ-D-アラビニトール 塩酸塩, enzyme inhibitor,
Species
mouse, rat, human

Kits

製品番号
詳細
Species
α-グルコシダーゼ活性アッセイキット, sufficient for 100 colorimetric tests,
Species
mouse, human, rat

esiRNA

製品番号
詳細
Species
MISSION® esiRNA, targeting human GAA,
Species
human
MISSION® esiRNA, targeting mouse Gaa,
Species
mouse