Lysosomes are membrane-enclosed organelles that contain an array of enzymes capable of breaking down proteins, nucleic acids, carbohydrates, and lipids. Lysosomes are enzyme sacs within cells that digest large molecules and recycle the fragments to other parts of the cell. Several critical enzymes are required for this process. If one of these enzymes becomes defective as a result of a mutation, the large molecules accumulate within the cell, eventually killing it. Lysosomal storage diseases (LSD) are a group of inherited genetic disorders caused by defective lysosomal metabolism and subsequent substrate accumulation. The abnormal accumulation of various toxic materials in the […]
