ABSTRACT
Lysosomes are intracellular organelles containing a large number of diferent enzymes at an acid pH, whose main function is the degradation of macromolecules. The lysosomal storage disorders are each caused by a speciic enzyme deiciency resulting in abnormal storage of partially degraded macromolecules in the lysosomes. Sterols are steroid alcohols, and they are found in fatty tissues. Steroid hormones afect the development and growth of the sex organs. Lipids, more commonly known as fats, are an important component of cell membranes and are stored in the body as an energy reserve. Lipid disorders are caused by a defect in certain enzymes that normally break down and process lipids so that the body can use them as an energy source. In Anderson-Fabry disease there is a deficiency of the enzyme alpha-galactosidase A, which results in the build-up of a fatty substance called globotriaosylceramide, in various organs.
