Acid alpha-glucosidase is an enzyme found in the lysosomes of cells. It plays a crucial role in breaking down complex carbohydrates, specifically glycogen, into simpler sugars. This process is essential for maintaining energy balance in the body. Deficiency of acid alpha-glucosidase leads to a genetic disorder known as Pompe disease. This condition results in the accumulation of glycogen in tissues, particularly affecting muscles and the heart. Symptoms can include muscle weakness and respiratory issues, highlighting the importance of this enzyme in overall health.