Lipoprotein lipase

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Federal government websites often end in. Before sharing sensitive information, make sure you're on a federal government site. The site is secure. NCBI Bookshelf. Lipoprotein lipase LPL is an extracellular enzyme on the vascular endothelial surface that degrades circulating triglycerides in the bloodstream. These triglycerides are embedded in very low-density lipoproteins VLDL and chylomicrons traveling through the bloodstream.

Lipoprotein lipase

It is a water-soluble enzyme that hydrolyzes triglycerides in lipoproteins , such as those found in chylomicrons and very low-density lipoproteins VLDL , into two free fatty acids and one monoacylglycerol molecule:. It is also involved in promoting the cellular uptake of chylomicron remnants , cholesterol-rich lipoproteins, and free fatty acids. In brief, LPL is secreted from heart, muscle and adipose parenchymal cells as a glycosylated homodimer, after which it is translocated through the extracellular matrix and across endothelial cells to the capillary lumen. After translation, the newly synthesized protein is glycosylated in the endoplasmic reticulum. Homodimerization is required before LPL can be secreted from cells. These two regions are attached by a peptide linker. Importantly, LPL binding to the cell surface or receptors is not dependent on its catalytic activity. The LPL non-covalent homodimer has a head-to-tail arrangement of the monomers. The lipid interacts with both the lid region and the hydrophobic groove at the active site; this causes the lid to move, providing access to the active site. LPL gene encodes lipoprotein lipase, which is expressed in the heart, muscle, and adipose tissue. Severe mutations that cause LPL deficiency result in type I hyperlipoproteinemia, while less extreme mutations in LPL are linked to many disorders of lipoprotein metabolism.

After translation, the newly synthesized protein is glycosylated in the endoplasmic reticulum.

Lipoprotein lipase LPL catalyses the hydrolysis of the triacylglycerol component of circulating chylomicrons and very low density lipoproteins, thereby providing non-esterified fatty acids and 2-monoacylglycerol for tissue utilisation. Research carried out over the past two decades have not only established a central role for LPL in the overall lipid metabolism and transport but have also identified additional, non-catalytic functions of the enzyme. Furthermore, abnormalities in LPL function have been found to be associated with a number of pathophysiological conditions, including atherosclerosis, chylomicronaemia, obesity, Alzheimer's disease, and dyslipidaemia associated with diabetes, insulin resistance, and infection. Advances have also been made in relating the various domains in the protein to different functions, and in understanding the mechanisms that are responsible for the changes in LPL expression seen in response to nutritional and other physiological changes, and during disease. This review summarises recent findings in relation to the structure, function, and regulation of LPL along with its important role in disease.

Federal government websites often end in. The site is secure. Lipoprotein lipase LPL is one of the most important factors in systemic lipid partitioning and metabolism. It mediates intravascular hydrolysis of triglycerides packed in lipoproteins such as chylomicrons and VLDL. Since LPL was initially discovered in the s, its biology and pathophysiological significance have been well characterized and documented. LPL was first discovered in by Dr.

Lipoprotein lipase

Federal government websites often end in. Before sharing sensitive information, make sure you're on a federal government site. The site is secure. NCBI Bookshelf. Lipoprotein lipase deficiency is a rare autosomal recessive genetic disorder of lipid metabolism.

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It is also involved in promoting the cellular uptake of chylomicron remnants , cholesterol-rich lipoproteins, and free fatty acids. In both type one familial dyslipidemia or hyperchylomicronemia, there is severe LPL dysfunction; this is because of LPL deficiency and LPL co-factor deficiency, or apolipoprotein C2 deficiency, which is necessary for activation of lipoprotein lipase. Instead, this regulation occurs by managing the flux of LPL arriving at the lipolytic site and by regulating the activity of LPL present on the endothelium. Pathophysiology The pathophysiology of lipoprotein lipase is evident in familial dyslipidemias specifically type one or hyperchylomicronemia. In: StatPearls [Internet]. National Library of Medicine. The lipid interacts with both the lid region and the hydrophobic groove at the active site; this causes the lid to move, providing access to the active site. Lipoprotein lipase LPL catalyses the hydrolysis of the triacylglycerol component of circulating chylomicrons and very low density lipoproteins, thereby providing non-esterified fatty acids and 2-monoacylglycerol for tissue utilisation. Search term. Thus, fibrates are indicated for patients with highly elevated levels of triglycerides. Lipoprotein lipase LPL is an extracellular enzyme on the vascular endothelial surface that degrades circulating triglycerides in the bloodstream. Bulk Download. May Disclosure: Sandeep Sharma declares no relevant financial relationships with ineligible companies.

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It is also involved in promoting the cellular uptake of chylomicron remnants , cholesterol-rich lipoproteins, and free fatty acids. Portal : Biology. The model suggests a general framework for how triglyceride trafficking is regulated. Affiliations 1 California Northstate College Medicine. Lipoprotein lipase is an enzyme that degrades circulating triglycerides in the bloodstream. The disorder causes a large amount of fat to build up in the blood. Fibrates, such as fenofibrate, bezafibrate, and gemfibrozil, work by activating peroxisome proliferator-activated receptor alpha PPAR-alpha and upregulating lipoprotein lipase. LPL gene lipoprotein lipase. Risk factors include a family history of lipoprotein lipase deficiency. Benefits and risks of the treatment with fibrates--a comprehensive summary. Bulk Download. When to Contact a Medical Professional. Allosteric regulation Cooperativity Enzyme inhibitor Enzyme activator. Also reviewed by David C. Trends Endocrinol.