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Lysosomal α-Glucosidase (GAA) Activity Assay Kit (Fluorometric)

Highly Sensitive α-Glucosidase Activity Assay
Catalog #: K187

Product Details

Cat # +Size K187-100
Size 100 assays
Kit Summary • Detection method- Fluorescence (368/460 nm)
• Applications:
- Measurement of lysosomal α-Glucosidase activity in biological samples
- Assay uses a specific substrate for the detection of GAA activity
Detection Method Fluorescence (368/460 nm)
Species Reactivity Eukaryotes
Applications · Measurement of Lysosomal α-Glucosidase activity in various tissues/cells.
Features & Benefits • Rapid and easy-to-use.
• Highly-sensitive & high-throughput capable
• This assay kit can measure0.05 µU of α-glucosidase activity in a variety of sample.
Kit Components • GAA Assay Buffer
• GAA Stop Buffer
• GAA Substrate (in DMSO)
• 4-Methylumbelliferone Standard (5 mM)
• GAA Positive Control
Storage Conditions -20°C
Shipping Conditions Gel Pack
USAGE For Research Use Only! Not For Use in Humans.


Lysosomal α-Glucosidase (GAA, Acid α-Glucosidase, Acid Maltase; EC is an exo-1,4 and exo-1,6-α-Glucosidase, which is essential for the degradation of glycogen to glucose in lysosomes. Deficiency in GAA activity results in the accumulation of glycogen within the lysosome, leading to Glycogen storage disease type II [also termed acid maltase deficiency (AMD) or Pompe disease]. Pompe disease is an inherited disorder of glycogen metabolism with broad spectrum of clinical phenotypes. Cardiac and skeletal muscles are the major target tissues and GAA activity in dry blood spots has been used to screen, and diagnose Pompe Disease. BioVision’s Lysosomal α-Glucosidase (GAA) Activity Assay Kit provides a simple way to monitor GAA activity in a wide variety of Biological Samples. In this kit, GAA cleaves a synthetic specific substrate releasing a fluorophore, which can be easily quantified (Ex/Em= 368/460 nm). The assay is specific, sensitive- it can detect as low as 0.05 µU of GAA activity

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