Lysosomal enzyme deficiency

A lysosome is a body containing a series of acid hydrolases surrounded by a single layer of lipoprotein membrane in the cytoplasm. It is an organelle with a single-layer membrane sac-like structure in the cell. The lysosome contains many kinds of hydrolytic enzymes that can decompose many kinds of substances. The lysosome is likened to the "enzyme warehouse" and "digestive system" in the cell. The enzymes in the lysosome are hydrolytic enzymes, and generally the optimum pH is 5, so they are all acid hydrolytic enzymes. If the enzymes in the lysosome are released, they will digest the whole cell. Generally not released to the internal environment, mainly for intracellular digestion. Congenital lysosomal disease is a type of metabolic genetic disease caused by mutations in certain genes on the chromosome and a congenital lack of a certain lysosomal enzyme. Due to the lack of enzymes or structural defects of the enzymes, the corresponding substrates in the cells cannot be degraded and stored, and they accumulate in the secondary lysosomes, causing cell metabolic disorders, so they are also called lysosomal storage diseases. Mucopolysaccharidosis is a group of diseases caused by the inability of acid mucopolysaccharide molecules (aminodextran) to be degraded due to lysosomal enzyme defects, resulting in a large amount of mucopolysaccharide deposition in tissues and increased mucopolysaccharide excretion in urine.

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