Lysosomal alpha-Glucosidase Activity Assay Kit (Fluorometric) (ab252887)
Key features and details
- Assay type: Quantitative
- Detection method: Fluorescent
- Platform: Microplate (12 x 8 well strips)
- Sample type: Cell Lysate, Other biological fluids, Tissue Homogenate
- Sensitivity: 0.05 µU
Overview
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Product name
Lysosomal alpha-Glucosidase Activity Assay Kit (Fluorometric)
See all alpha-glucosidase kits -
Detection method
Fluorescent -
Sample type
Other biological fluids, Cell Lysate, Tissue Homogenate -
Assay type
Quantitative -
Sensitivity
0.05 µU -
Assay duration
Multiple steps standard assay -
Species reactivity
Reacts with: Mouse, Human -
Product overview
Lysosomal alpha-Glucosidase Activity Assay Kit (Fluorometric) ab252887 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 and sensitive, it can detect as low as 0.05 µU of GAA activity.
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Tested applications
Suitable for: ELISAmore details -
Platform
Microplate (12 x 8 well strips)
Properties
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Storage instructions
Store at -20°C. Please refer to protocols. -
Components 100 tests 4-Methylumbelliferone Standard (5 mM) 1 x 35µl GAA Assay Buffer 1 x 25ml GAA Positive Control 1 vial GAA Stop Buffer 1 x 25ml GAA Substrate (in DMSO) 1 x 280µl -
Relevance
a-Glucosidase breaks down a-1,4 linked polysaccharides to glucose, which can be utilized as a source of energy. In the biotechnology industry, a-glucosidase is used to produce glucose from intermediate breakdown products of starch hydrolysis generated by enzymes such as amylase. Pompe disease, one of the 12 known glycogen storage diseases, is an autosomal recessive metabolic disorder attributed to a- glucosidase deficiency. In this disease, glycogen accumulates in the lysosomes, resulting in progressive muscle weakness, heart failure and other neurological symptoms. -
Cellular localization
Lysosome. Lysosome membrane. -
Alternative names
- Acid Maltase
- Aglucosidase Alfa
- EC 3.2.1.20
see all -
Database links
- Entrez Gene: 2548 Human
- Entrez Gene: 14387 Mouse
- Omim: 606800 Human
- SwissProt: P10253 Human
- SwissProt: P70699 Mouse
- Unigene: 1437 Human
- Unigene: 4793 Mouse
Images
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Lysosomal alpha-Glucosidase Activity Assay Kit (Fluorometric) ab252887 Example Standard Curve
4-Methylumbelliferone (4-MU) standard curve, results from multiple experiments.
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Lysosomal alpha-Glucosidase Activity Assay Kit (Fluorometric) Example DataMeasurement of GAA activity in human pool serum (5 µl, ultra-concentrate).
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Lysosomal alpha-Glucosidase Activity Assay Kit (Fluorometric) Example DataMeasurement of GAA activity in mouse heart extracts (3 µg protein), mouse muscle extracts (2 µg protein) and 3T3 cell lysates (0.5 µg protein).