Glucosidase Alpha, Acid (GaA) - the Gold Standard for Diagnosing Pompe Disease

 GlucosidaseAlpha, Acid (GaA) is a kind of amylum hydrolase enzyme, which catalyzes thehydrolysis of polysaccharides to produce alpha D- glucose. In addition, it alsopossesses transglycosylation activity, which can transfer the alpha -1,4-glycosidic bond to alpha-1,6- glycosidic bond or other form linkages, to obtainnonfermented isomalto- oligosaccharide, glycolipids and glycopeptide etc..

    The gene,which encode the GAA, is located at chromosome 17. Defects in this gene maylead to deficiency of GaA and glycogen accumulation in skeletal muscle, smoothmuscle & cardiac muscle tissue as the glycogen can’t be hydrolyzed by GaA. Then it may cause severeneuromuscular disease. This lysosomal storage disorder is called Pompe disease,also called glycogen storage disease II. In general population, incidence ofGAA varies from 1 / 40,000 to 1 / 300,000. This infrequent inherited metabolicdiseases presents autosomal recessive inheritance, characterized in aggravatedsexual skeletal muscle atrophy, weakness,and poor prognosis.

    With early and accurate diagnosis of Pompedisease, the patients can get target treatment and care in early stage. It canalso improve prognosis. In the diagnosis of Pompe disease, GaA can be used asthe gold standard.The recombinant GaA proteins, antibodies and quantitativeassay kits, developed by Cloud-CloneCorp., can be used forin vitro detection and study of GaA.

    GaArelated products in Cloud-clone Corp are shown below.

For more products, please check www.cloud-clone.com.