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

Cloud-Clone Corp.

 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.

ProductHumanMouseRat
ProteinPRA177Hu01PRA177Mu01PRA177Ra01
PRA177Hu02PRA177Mu02PRA177Ra02
AntibodiesPAA177Hu01PAA177Mu01PAA177Ra01
PAA177Hu02PAA177Mu02PAA177Ra02
MAA177Hu22————
Assay KitsSEA177HuSEA177MuSEA177Ra

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