الفهرس | Only 14 pages are availabe for public view |
Abstract Lysosomes are membrane-bound organelles that contain acid hydrolases. Lysosomes are found in all eukaryotes, from protozoa to humans, except for mature mammalian red blood cells. In fungi and plants lysosomes are called vacuoles. Controlled intracellular digestion takes place in the lysosome’s interior by acid hydrolases. Lysosomes are involved in the turnover of the cell’s own organelles via autophagy. Selective lysosomal degradation pathway is activated by nutrient deprivation and stress. Lysosomal dysfunction leads to various human diseases. Lysosomes storage diseases are inherited metabolic disorders caused by the lack or the reduction of lysosomal acid hydrolase activity; this leads to lysosome overload by the undigested substrates. Other diseases, such as gout and arthritis resulted from inappropriate release of lysosomal hydrolases. This review summarizes some lysosomal enzymes and their diagnostic significance in some metabolic diseases. |