Description
Background/Introduction: alpha-Glucosidase breaks down alpha-1,4 linked polysaccharides to glucose, which can be utilized as a source of energy. In the biotechnology industry, alpha-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 alpha-glucosidase deficiency. In this disease, glycogen accumulates in the lysosomes, resulting in progressive muscle weakness, heart failure and other neurological symptoms. The assay is initiated with the enzymatic hydrolysis of the glucoside by alpha-Glucosidase. The enzyme catalysed reaction products p-nitrophenol, can be measured at a colorimetric readout at 400 nm