Lysosomal lipase is a form of

lipase Lipase ( ) is a family of enzymes that catalyzes the hydrolysis of fats. Some lipases display broad substrate scope including esters of cholesterol, phospholipids, and of lipid-soluble vitamins and sphingomyelinases; however, these are usually tr ...

which functions

intracellular This glossary of biology terms is a list of definitions of fundamental terms and concepts used in biology, the study of life and of living organisms. It is intended as introductory material for novices; for more specific and technical definitions ...

ly, in the

lysosome A lysosome () is a membrane-bound organelle found in many animal cells. They are spherical vesicles that contain hydrolytic enzymes that can break down many kinds of biomolecules. A lysosome has a specific composition, of both its membrane prot ...

Biochemical Significance

The primary function of lysosomal lipase is to hydrolyze lipids such as triglycerides and cholesterol. These fats are transported and degraded into free fatty acids. Lysosomal lipases function optimally at an acidic pH which are complementary with the environment found in the lysosomal lumen. These enzymes were believed to only hydrolyze the lipids found in organelle membranes and extracellular lipids. However, recent studies suggest that lysosomal lipases also play a significant role in the degradation of cytosolic lipids, a characteristic that was previously limited to neutral lipases. The ability of the lysosome to degrade a diverse set of cargo is attributed to the lysosomal lipase and other soluble hydrolases. These enzymes include sulphatases, phosphatases, peptidases, glycosidases, and nucleases. The biochemical role of these enzymes are observed in various pathways, specifically in lipid catabolism. At the intracellular level, the byproducts released by the lysosomal lipase are recycled for membrane assembly and energy production. In addition, these enzymes participate in the production of specific fatty acids necessary for the metabolic reprogramming of CD8+ memory T cells, macrophage alternative activation, and lipid mediator synthesis. As observed, the degradation of these lipids are essential to maintain homeostasis within the body. The absence or decreased activity of this enzyme could lead to various metabolic disorders.

Clinical significance

A deficiency associated with

lysosomal acid lipase deficiency Lysosomal acid lipase deficiency (LAL deficiency or LAL-D) is an autosomal recessive inborn error of metabolism that results in the body not producing enough active lysosomal acid lipase (LAL) enzyme. This enzyme plays an important role in breakin ...

Wolman disease Lysosomal acid lipase deficiency (LAL deficiency or LAL-D) is an autosomal recessive inborn error of metabolism that results in the body not producing enough active Lysosomal lipase, lysosomal acid lipase (LAL) enzyme. This enzyme plays an importan ...

, and

cholesteryl ester storage disease Lysosomal acid lipase deficiency (LAL deficiency or LAL-D) is an autosomal recessive inborn error of metabolism that results in the body not producing enough active lysosomal acid lipase (LAL) enzyme. This enzyme plays an important role in breakin ...

Chlorpromazine Chlorpromazine (CPZ), marketed under the brand names Thorazine and Largactil among others, is an antipsychotic medication. It is primarily used to treat psychotic disorders such as schizophrenia. Other uses include the treatment of bipolar dis ...

is an inhibitor of lysosomal lipase. A genome wide survey suggests that lysosomal lipase A (located at chromosome 10q23.31) is associated with coronary artery disease in humans.

References

External links

* {{biochem-stub