Myozyme is a brand name for alglucosidase alfa, a recombinant form of the human enzyme acid alpha-glucosidase. It is used in the treatment of Pompe disease, a rare and often fatal muscular disorder caused by an accumulation of glycogen in the lysosome due to deficiency of the enzyme acid alpha-glucosidase. Myozyme works by replacing the deficient enzyme, facilitating the breakdown of glycogen, and preventing its accumulation in cells. This enzyme replacement therapy has been a significant advancement in the management of Pompe disease, offering patients an opportunity for improved muscle function and a better quality of life. Myozyme is administered through intravenous infusion, and its use must be closely monitored by healthcare professionals due to the potential for allergic reactions and other side effects.