• In liver, pristanic acid is degraded by peroxisomal beta oxidation to propionyl-CoA. (wikipedia.org)
  • They participate in important peroxisome-specific chemical reactions, such as beta-oxidation of very-long-chain fatty acids (VLCFAs) and detoxification of hydrogen peroxide. (medscape.com)
  • Whereas the mitochondria are responsible for the oxidation of the bulk of dietary fatty acids (palmitate, oleate and linolate), peroxisomes are responsible fully for the beta oxidation of VLCFAs (C24:0 and C26:0) in addition to pristanic acid (from dietary phytanic acid) and dihydroxycholestanoic acid (DHCA) or trihydroxycholestanoic acid (THCA). (medscape.com)
  • Another major function of the peroxisomal beta-oxidation system is related to the biosynthesis of polyunsaturated fatty acid (C22:6w3). (medscape.com)
  • Because of the presence of a 3-methyl group, phytanic acid cannot be degraded by beta-oxidation. (diseasesdic.com)
  • In the metabolism of phytanic acid it must first be converted to pristanic acid via alpha-oxidation, due to the presence of the methyl group at the 3-position, before undergoing beta-oxidation. (matreya.com)
  • 1 In diseases involving peroxisomal impairment the branched chain fatty acids phytanic acid and pristanic acid accumulate in high amounts due to deficiencies in alpha- and beta-oxidation. (matreya.com)
  • With a chain length from 2 to 6 (or 4) they are called short-chain, from 8 (or 6) to 10 they are called medium-chain and 12 up to 24 they are called long-chain fatty acids. (gerli.com)
  • Serum very long-chain fatty acids were significantly elevated. (bvsalud.org)
  • These enzymes overlap in function with those in mitochondria, with the exception that mitochondria lack enzymes to metabolize very long-chain fatty acids (VLCFA), those 20 to 26 carbons in length. (msdmanuals.com)
  • Refsum disease is associated with the accumulation of phytanic acid in plasma and tissues, which is an unusual branched-chain fatty acid (3,7,11,15-tetramethyl-hexadecanoic acid), derived from the chlorophyll and is present in the typical human diet (strictly exogenous source) consisting of dairy products, meats, and ruminant fats. (diseasesdic.com)
  • At physiological concentrations pristanic acid is a natural ligand for peroxisome proliferator-activated receptor alpha (PPARα). (wikipedia.org)
  • The gene code of PHYH for the Phytanoyl-CoA enzyme is used to break down phytanic acid in the peroxisome. (diseasesdic.com)
  • 2 Phytanic acid is a ligand and transcriptional activator of murine liver fatty acid binding protein thereby causing peroxisome proliferation. (matreya.com)
  • At first It must be recalled that if the majority of the fatty acids found in lipids are monocarboxylic acids, some of them are dicarboxylic and constitute important metabolic or oxidation products of the previous ones. (gerli.com)
  • Carboxylic acids occur in many molecular forms. (gerli.com)
  • Peroxisomes are also involved in the production of cholesterol, bile acids, and plasmalogens, which contribute to a big part of the phospholipid content of the brain white matter. (medscape.com)
  • Peroxisomes also work in conjunction with mitochondria to shorten fatty acid chains, which are in turn degraded to completion in the mitochondria. (medscape.com)
  • Mutant forms of phytanoyl-CoA 2-hydroxylase (PHYH) which plays a key role of phytanic acid alpha-oxidation in peroxisomes have been shown to be responsible for some, but not all, cases of Refsum's disease. (diseasesdic.com)
  • Refsum disease is a genetic disorder that affects the metabolism of the fatty acid phytanic acid. (diseasesdic.com)
  • Overview of Fatty Acid and Glycerol Metabolism Disorders Fatty acids are the preferred energy source for the heart and an important energy source for skeletal muscle during prolonged exertion. (msdmanuals.com)
  • In humans, pristanic acid is obtained from two sources: either directly from the diet or as the alpha oxidation product of phytanic acid. (wikipedia.org)
  • Phytanic acid will have alpha-oxidation, which shortens phytanic acid by one carbon atom yielding pristanic acid and carbon dioxide. (diseasesdic.com)
  • To describe precisely the structure of a fatty acid molecule, one must give the length of the carbon chain (number of carbon), the number of double bonds and also the exact position of these double bonds, this will define the biological reactivity of the fatty acid molecule and even of the lipid containing the fatty acids studied. (gerli.com)
  • These last 2 compounds lead to the formation of bile acids, cholic acid, and chenodeoxycholic acid from cholesterol in the liver. (medscape.com)
  • Diagnosis is suspected when elevated blood levels of VLCFA, phytanic acid, bile acid intermediates, and pipecolic acid are detected and is confirmed by genetic testing. (msdmanuals.com)
  • Because the patients are unable to metabolize phytanic acid derived from exogenous sources, highly raised plasma phytanic acid (PA) level in tissues and body fluids is the hallmark of RD. (diseasesdic.com)
  • Pristanic acid (2,6,10,14-tetramethylpentadecanoic acid) is a terpenoid acid present at micromolar concentrations in the blood plasma of healthy individuals. (wikipedia.org)
  • Mature adipocytes are known to play an important role controlling energy balance in mammals by storing fatty acids in the form of triglycerides in periods of excess of energy and by releasing fatty acids when are needed. (biomedcentral.com)
  • Most fatty acids are straight-chain compounds with the most frequently an even number of carbon atoms. (gerli.com)
  • Except fatty acyl-CoA, we have based our classification of fatty acids first on the type of carbon chain : either straight (or normal), or branched, or containing a carbon ring. (gerli.com)
  • The world production of fatty acids from the hydrolysis of natural fats and oils totaled about 4 million metric tons per year. (gerli.com)
  • The typical presence of phytanic acid is dairy, beef, lamb and other feed originating from ruminant animals as well as certain sea foods. (diseasesdic.com)
  • Phytanic acid in animals is derived from phytol, a breakdown product of chlorophyll which is caused by bacteria in the gut of ruminant animals. (matreya.com)
  • It is usually present in combination with phytanic acid. (wikipedia.org)
  • When double bonds are present, fatty acids are said unsaturated, monounsaturated (MUFA) if only one double bond is present and polyenoic (or polyunsaturated fatty acids = PUFA ) if they have two or more double bonds generally separated by a single methylene group (methylene-interrupted unsaturation). (gerli.com)
  • Some uncommon polyunsaturated fatty acids have two adjacent double bonds separated by more than one methylene group, they are named polymethylene-interrupted fatty acids. (gerli.com)
  • In humans, pristanic acid is obtained from two sources: either directly from the diet or as the alpha oxidation product of phytanic acid. (wikipedia.org)
  • Fatty acid alpha-oxidation is a strictly peroxisomal process. (medscape.com)
  • ARD is a rare peroxisomal autosomal recessive disease due to deficient alpha-oxidation of phytanic acid (PA), a branched-chain fatty acid. (nih.gov)
  • Among straight-chain fatty acids, the simplest are referred to as saturated fatty acids. (gerli.com)
  • After describing the enzyme machinery that degrades these nutrient fatty acids in the peroxisome, we show by the criteria of a mouse model and of a human cell culture model that they induce with high potency expression of enzymes responsible for beta-oxidation of straight-chain fatty acids in the peroxisome. (ocl-journal.org)
  • At physiological concentrations pristanic acid is a natural ligand for peroxisome proliferator-activated receptor alpha (PPARα). (wikipedia.org)
  • We summarize present mechanistic knowledge on fatty acid signaling to the nucleus, which involves protein/protein contacts between peroxisome proliferator activated receptor (PPAR) and fatty acid binding protein (FABP). (ocl-journal.org)
  • This hypothesis was confirmed when post-mortem biochemical analysis of brain, adrenal glands and serum of seven patients revealed the presence of cholesterol esters with high amounts of fatty acids longer than C22. (biomedcentral.com)
  • To describe precisely the structure of a fatty acid molecule, one must give the length of the carbon chain (number of carbon), the number of double bonds and also the exact position of these double bonds, this will define the biological reactivity of the fatty acid molecule and even of the lipid containing the fatty acids studied. (gerli.com)
  • Here we focus on chlorophyll-derived phytanic and pristanic acids that are minor isoprenoid branched-chain lipid constituents in food, but of unknown nutritional value. (ocl-journal.org)
  • Overview of Fatty Acid and Glycerol Metabolism Disorders Fatty acids are the preferred energy source for the heart and an important energy source for skeletal muscle during prolonged exertion. (msdmanuals.com)
  • Peroxisomes also work in conjunction with mitochondria to shorten fatty acid chains, which are in turn degraded to completion in the mitochondria. (medscape.com)
  • Les différents éléments ayant conduit à un développement significatif d'une activité biodiesel en France seront examinés : organisation de la filière oléagineuse, partenariat avec les pétroliers, accompagnement des pouvoirs publics en termes réglementaires et fiscaux. (ocl-journal.org)
  • The world production of fatty acids from the hydrolysis of natural fats and oils totaled about 4 million metric tons per year. (gerli.com)
  • 5. Studies on the oxidation of phytanic acid and pristanic acid in human fibroblasts by acylcarnitine analysis. (nih.gov)