ALGLUCOSIDASE ALFA IS A RECOMBINANT FORM OF HUMAN ACID ALPHA GLUCOSIDASE GIVEN AS ENZYME REPLACEMENT THERAPY FOR THE TREATMENT OF LYSOSOMAL STORAGE DISEASE POMPE DISEASE (GLYCOGEN STORAGE DISEASE TYPE II). THIS IS A RARE FATAL AUTOSOMAL RECESSIVE DISORDER CAUSED BY A DEFICIENCY OF ACID ?-GLUCOSIDASE, WHICH CLEAVES ?-1,4- AND ?-1,6-GLUCOSIDIC LINKAGES IN LYSOSOMAL GLYCOGEN TO LIBERATE GLUCOSE. GLYCOGEN ACCUMULATION RESULTS IN PROGRESSIVE MYOPATHY, ESPECIALLY OF THE SKELETAL MUSCLES AND HEART.