Triglyceride metabolism and classification of hypertriglyceridemias. 2021

Daiana Ibarretxe, and Lluís Masana
Unitat de Medicina Vascular i Metabolisme, Hospital Universitari Sant Joan, Reus, Tarragona, España; Unitat de Reçerca en Lípids i Arteriosclerosi, Universitat Rovira i Virgili, IISPV, Reus, Tarragona, España; Centro de Investigación Biomédica en Red de Diabetes y Enfermedades Metabólicas Asociadas (CIBERDEM), Madrid, España. Electronic address: daiana.ibarretxe@urv.cat.

Triglycerides (TG) are the most important molecules for the energy reserve of our body. After their hepatic or intestinal synthesis from fatty acids, they are carried by chylomicrons (QM (intestinal origin) or VLDL (hepatic origin) in plasma. Their catabolism is determined by the action of the lipoprotein lipase protein complex (LPL) and the hepatic receptors (RLDL and LRP-1) are responsible for their clearance are. Changes in the production or catabolism leads to hypertriglyceridaemia (HTG). The HTG are classified according to severity as, mild-moderate (150-885mg/dl), severe (>885mg/dl), or very severe (>1770mg/dl). They can be primary and secondary depending on origin. In the main primary form is highlighted Familial Chylomicronaemia Syndrome (CFS), a very severe form due to mutations in the LPL gene or associated proteins. Most HTG are due to a combination of genetic and environmental predisposing factors.

UI MeSH Term Description Entries
D008071 Lipoprotein Lipase An enzyme of the hydrolase class that catalyzes the reaction of triacylglycerol and water to yield diacylglycerol and a fatty acid anion. The enzyme hydrolyzes triacylglycerols in chylomicrons, very-low-density lipoproteins, low-density lipoproteins, and diacylglycerols. It occurs on capillary endothelial surfaces, especially in mammary, muscle, and adipose tissue. Genetic deficiency of the enzyme causes familial hyperlipoproteinemia Type I. (Dorland, 27th ed) EC 3.1.1.34. Heparin-Clearing Factor,Lipemia-Clearing Factor,Diacylglycerol Lipase,Diglyceride Lipase,Post-Heparin Lipase,Postheparin Lipase,Postheparin Lipoprotein Lipase,Factor, Heparin-Clearing,Factor, Lipemia-Clearing,Heparin Clearing Factor,Lipase, Diacylglycerol,Lipase, Diglyceride,Lipase, Lipoprotein,Lipase, Post-Heparin,Lipase, Postheparin,Lipase, Postheparin Lipoprotein,Lipemia Clearing Factor,Lipoprotein Lipase, Postheparin,Post Heparin Lipase
D008072 Hyperlipoproteinemia Type I An inherited condition due to a deficiency of either LIPOPROTEIN LIPASE or APOLIPOPROTEIN C-II (a lipase-activating protein). The lack of lipase activities results in inability to remove CHYLOMICRONS and TRIGLYCERIDES from the blood which has a creamy top layer after standing. Apolipoprotein C-II Deficiency,Hyperchylomicronemia, Familial,Lipoprotein Lipase Deficiency, Familial,Burger-Grutz Syndrome,C-II Anapolipoproteinemia,Chylomicronemia, Familial,Familial Fat-Induced Hypertriglyceridemia,Familial Hyperchylomicronemia,Familial Hyperlipoproteinemia Type 1,Familial LPL Deficiency,Familial Lipoprotein Lipase Deficiency,Hyperlipemia, Essential Familial,Hyperlipemia, Idiopathic, Burger-Grutz Type,Hyperlipoproteinemia Type Ia,Hyperlipoproteinemia Type Ib,Hyperlipoproteinemia, Type I,Hyperlipoproteinemia, Type Ia,Hyperlipoproteinemia, Type Ib,LIPD Deficiency,Lipase D Deficiency,Lipoprotein Lipase Deficiency,Anapolipoproteinemia, C-II,Anapolipoproteinemias, C-II,Apolipoprotein C II Deficiency,Apolipoprotein C-II Deficiencies,Burger Grutz Syndrome,Burger-Grutz Syndromes,C-II Anapolipoproteinemias,Chylomicronemias, Familial,Deficiencies, Apolipoprotein C-II,Deficiencies, Familial LPL,Deficiencies, LIPD,Deficiencies, Lipase D,Deficiencies, Lipoprotein Lipase,Deficiency, Apolipoprotein C-II,Deficiency, Familial LPL,Deficiency, LIPD,Deficiency, Lipase D,Deficiency, Lipoprotein Lipase,Essential Familial Hyperlipemia,Essential Familial Hyperlipemias,Familial Chylomicronemia,Familial Chylomicronemias,Familial Fat Induced Hypertriglyceridemia,Familial Fat-Induced Hypertriglyceridemias,Familial Hyperchylomicronemias,Familial Hyperlipemia, Essential,Familial Hyperlipemias, Essential,Familial LPL Deficiencies,Fat-Induced Hypertriglyceridemia, Familial,Fat-Induced Hypertriglyceridemias, Familial,Hyperchylomicronemias, Familial,Hyperlipemias, Essential Familial,Hyperlipoproteinemia Type Ias,Hyperlipoproteinemia Type Ibs,Hyperlipoproteinemia Type Is,Hyperlipoproteinemias, Type I,Hyperlipoproteinemias, Type Ia,Hyperlipoproteinemias, Type Ib,Hypertriglyceridemia, Familial Fat-Induced,Hypertriglyceridemias, Familial Fat-Induced,LIPD Deficiencies,LPL Deficiencies, Familial,LPL Deficiency, Familial,Lipase D Deficiencies,Lipase Deficiencies, Lipoprotein,Lipoprotein Lipase Deficiencies,Syndrome, Burger-Grutz,Syndromes, Burger-Grutz,Type I Hyperlipoproteinemia,Type I Hyperlipoproteinemias,Type Ia Hyperlipoproteinemia,Type Ia Hyperlipoproteinemias,Type Ib Hyperlipoproteinemia,Type Ib Hyperlipoproteinemias
D008079 Lipoproteins, VLDL A class of lipoproteins of very light (0.93-1.006 g/ml) large size (30-80 nm) particles with a core composed mainly of TRIGLYCERIDES and a surface monolayer of PHOSPHOLIPIDS and CHOLESTEROL into which are imbedded the apolipoproteins B, E, and C. VLDL facilitates the transport of endogenously made triglycerides to extrahepatic tissues. As triglycerides and Apo C are removed, VLDL is converted to INTERMEDIATE-DENSITY LIPOPROTEINS, then to LOW-DENSITY LIPOPROTEINS from which cholesterol is delivered to the extrahepatic tissues. Pre-beta-Lipoprotein,Prebeta-Lipoprotein,Prebeta-Lipoproteins,Very Low Density Lipoprotein,Very-Low-Density Lipoprotein,Very-Low-Density Lipoproteins,Lipoprotein VLDL II,Lipoproteins, VLDL I,Lipoproteins, VLDL III,Lipoproteins, VLDL1,Lipoproteins, VLDL2,Lipoproteins, VLDL3,Pre-beta-Lipoproteins,Lipoprotein, Very-Low-Density,Lipoproteins, Very-Low-Density,Pre beta Lipoprotein,Pre beta Lipoproteins,Prebeta Lipoprotein,Prebeta Lipoproteins,VLDL Lipoproteins,VLDL1 Lipoproteins,VLDL2 Lipoproteins,VLDL3 Lipoproteins,Very Low Density Lipoproteins
D009154 Mutation Any detectable and heritable change in the genetic material that causes a change in the GENOTYPE and which is transmitted to daughter cells and to succeeding generations. Mutations
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D014280 Triglycerides An ester formed from GLYCEROL and three fatty acid groups. Triacylglycerol,Triacylglycerols,Triglyceride
D015228 Hypertriglyceridemia A condition of elevated levels of TRIGLYCERIDES in the blood. Hypertriglyceridemias
D050356 Lipid Metabolism Physiological processes in biosynthesis (anabolism) and degradation (catabolism) of LIPIDS. Metabolism, Lipid

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