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

Alternate Name : α-D-Glucosidase Activity Colorimetric Assay Kit , α-D-Glucoside Glucohydrolase Activity Colorimetric Assay Kit , α-Glucopyranosidase Activity Colorimetric Assay Kit, Maltase Activity Colorimetric Assay Kit, α-1,4-Glucosidase Activity Colorimetric Assay Kit, Glucoinvertase Activity Colorimetric Assay Kit

Tag Line :

Highly Sensitive α-Glucosidase Activity Assay

Storage Conditions: -20°C

Shelf life: 12 months

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)

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.

Description:

Lysosomal α-Glucosidase (GAA, Acid α-Glucosidase, Acid Maltase; EC 3.2.1.3) 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