Lysosomal Acid Lipase (LAL)

Lysosomal acid lipase (LAL) is a serine hydrolase enzyme located within lysosomes, where it plays a central role in intracellular lipid metabolism. Its primary function is to hydrolyze cholesteryl esters (CEs) and triglycerides (TGs) into free cholesterol and fatty acids, which are then used for membrane assembly, energy production, and various cellular signaling pathways. Deficiency or loss-of-function mutations in *LIPA* cause Wolman disease and Cholesteryl Ester Storage Disease (CESD), characterized by accumulation of CEs/TGs primarily in liver, spleen, and adrenal glands.

Other names

Lipase AAcid cholesteryl ester hydrolaseAcid cholesterol esterase

Enzyme replacement therapy (ERT) with sebelipase alfa provides exogenous LAL to hydrolyze accumulated cholesteryl esters and triglycerides within lysosomes.