Lysosomal α-Glucosidase Activity Assay, Catalog: MA-0155

Catalog MA-0155

Size 100 wells

Sample Type Cell Lysate, Tissue Extracts, Serum, Other Biological Fluids

Method of Detection Fluorometric, Ex/Em = 360/460 nm

Assay Type Quantitative

Application A plate based simple, sensitive fluorometric assay to measure Lysosomal α-Glucosidase enzyme activity in a variety of biological samples.

Sensitivity In the low nanoUnits per well. 

Storage Conditions -20°C

Shipping Temperature Gel Pack

Shelf Life One year from the date of delivery

Lysosomal α-Glucosidase catalyzes the hydrolysis of  α-1,4 and α-1,6 glycosidic linkages  of glycogen, thereby breaking it down to supply glucose to cells. Unlike the cytosolic enzymes involved in glycogenolysis, lysosomal α-glucosidase operates as part of the autophagic pathway, turning over glycogen that is delivered to lysosomes, particularly from organelles and cellular debris. Genetic defects in its gene lead to a severe metabolic disorder called glycogen storage disease type II, or Pompe disease, characterized by the accumulation of glycogen in lysosomes due to reduced or absent lysosomal α-glucosidase activity. This damages skeletal and cardiac muscle, leading to muscular weakness and possible cardiorespiratory complications. AkrivisBio’s Lysosomal α-Glucosidase Activity Assay is a simple, sensitive way to assess enzyme activity  with a sensitivity in the low nanoUnits per well.