Carnitine: A constituent of STRIATED MUSCLE and LIVER. It is an amino acid derivative and an essential cofactor for fatty acid metabolism.Acyltransferases: Enzymes from the transferase class that catalyze the transfer of acyl groups from donor to acceptor, forming either esters or amides. (From Enzyme Nomenclature 1992) EC 2.3.Carnitine O-Palmitoyltransferase: An enzyme that catalyzes reversibly the conversion of palmitoyl-CoA to palmitoylcarnitine in the inner mitochondrial membrane. EC 18.104.22.168.Carnitine O-Acetyltransferase: An enzyme that catalyzes the formation of O-acetylcarnitine from acetyl-CoA plus carnitine. EC 22.214.171.124.Carnitine Acyltransferases: Acyltransferases in the inner mitochondrial membrane that catalyze the reversible transfer of acyl groups from acyl-CoA to L-carnitine and thereby mediate the transport of activated fatty acids through that membrane. EC 2.3.1.Glycerol-3-Phosphate O-Acyltransferase: An enzyme that transfers acyl groups from acyl-CoA to glycerol-3-phosphate to form monoglyceride phosphates. It acts only with CoA derivatives of fatty acids of chain length above C-10. Also forms diglyceride phosphates. EC 126.96.36.199.Phosphatidylcholine-Sterol O-Acyltransferase: An enzyme secreted from the liver into the plasma of many mammalian species. It catalyzes the esterification of the hydroxyl group of lipoprotein cholesterol by the transfer of a fatty acid from the C-2 position of lecithin. In familial lecithin:cholesterol acyltransferase deficiency disease, the absence of the enzyme results in an excess of unesterified cholesterol in plasma. EC 188.8.131.52.Diacylglycerol O-Acyltransferase: An enzyme that catalyses the last step of the TRIACYLGLYCEROL synthesis reaction in which diacylglycerol is covalently joined to LONG-CHAIN ACYL COA to form triglyceride. It was formerly categorized as EC 184.108.40.206.Sterol O-Acyltransferase: An enzyme that catalyzes the formation of cholesterol esters by the direct transfer of the fatty acid group from a fatty acyl CoA derivative. This enzyme has been found in the adrenal gland, gonads, liver, intestinal mucosa, and aorta of many mammalian species. EC 220.127.116.11.Lecithin Acyltransferase Deficiency: An autosomal recessively inherited disorder caused by mutation of LECITHIN CHOLESTEROL ACYLTRANSFERASE that facilitates the esterification of lipoprotein cholesterol and subsequent removal from peripheral tissues to the liver. This defect results in low HDL-cholesterol level in blood and accumulation of free cholesterol in tissue leading to a triad of CORNEAL OPACITY, hemolytic anemia (ANEMIA, HEMOLYTIC), and PROTEINURIA.1-Acylglycerophosphocholine O-Acyltransferase: An enzyme localized predominantly within the plasma membrane of lymphocytes. It catalyzes the transfer of long-chain fatty acids, preferentially unsaturated fatty acids, to lysophosphatides with the formation of 1,2-diacylglycero-3-phosphocholine and CoA. EC 18.104.22.168.Acyl Coenzyme A: S-Acyl coenzyme A. Fatty acid coenzyme A derivatives that are involved in the biosynthesis and oxidation of fatty acids as well as in ceramide formation.Acetylcarnitine: An acetic acid ester of CARNITINE that facilitates movement of ACETYL COA into the matrices of mammalian MITOCHONDRIA during the oxidation of FATTY ACIDS.Palmitoyl Coenzyme A: A fatty acid coenzyme derivative which plays a key role in fatty acid oxidation and biosynthesis.Malonyl Coenzyme A: A coenzyme A derivative which plays a key role in the fatty acid synthesis in the cytoplasmic and microsomal systems.gamma-Butyrobetaine Dioxygenase: An enzyme that catalyzes the HYDROXYLATION of gamma-butyrobetaine to L-CARNITINE. It is the last enzyme in the biosynthetic pathway of L-CARNITINE and is dependent on alpha-ketoglutarate; IRON; ASCORBIC ACID; and OXYGEN.1-Acylglycerol-3-Phosphate O-Acyltransferase: An enzyme that catalyzes the acyl group transfer of ACYL COA to 1-acyl-sn-glycerol 3-phosphate to generate 1,2-diacyl-sn-glycerol 3-phosphate. This enzyme has alpha, beta, gamma, delta and epsilon subunits.Organic Cation Transport Proteins: A family of proteins involved in the transport of organic cations. They play an important role in the elimination of a variety of endogenous substances, xenobiotics, and their metabolites from the body.Esterification: The process of converting an acid into an alkyl or aryl derivative. Most frequently the process consists of the reaction of an acid with an alcohol in the presence of a trace of mineral acid as catalyst or the reaction of an acyl chloride with an alcohol. Esterification can also be accomplished by enzymatic processes.Fatty Acids: Organic, monobasic acids derived from hydrocarbons by the equivalent of oxidation of a methyl group to an alcohol, aldehyde, and then acid. Fatty acids are saturated and unsaturated (FATTY ACIDS, UNSATURATED). (Grant & Hackh's Chemical Dictionary, 5th ed)Palmitoylcarnitine: A long-chain fatty acid ester of carnitine which facilitates the transfer of long-chain fatty acids from cytoplasm into mitochondria during the oxidation of fatty acids.Acylation: The addition of an organic acid radical into a molecule.Coenzyme ACholesterol Esters: Fatty acid esters of cholesterol which constitute about two-thirds of the cholesterol in the plasma. The accumulation of cholesterol esters in the arterial intima is a characteristic feature of atherosclerosis.Liver: A large lobed glandular organ in the abdomen of vertebrates that is responsible for detoxification, metabolism, synthesis and storage of various substances.TriglyceridesMitochondria, Liver: Mitochondria in hepatocytes. As in all mitochondria, there are an outer membrane and an inner membrane, together creating two separate mitochondrial compartments: the internal matrix space and a much narrower intermembrane space. In the liver mitochondrion, an estimated 67% of the total mitochondrial proteins is located in the matrix. (From Alberts et al., Molecular Biology of the Cell, 2d ed, p343-4)Cholesterol: The principal sterol of all higher animals, distributed in body tissues, especially the brain and spinal cord, and in animal fats and oils.Glycerophosphates: Any salt or ester of glycerophosphoric acid.Apolipoprotein A-I: The most abundant protein component of HIGH DENSITY LIPOPROTEINS or HDL. This protein serves as an acceptor for CHOLESTEROL released from cells thus promoting efflux of cholesterol to HDL then to the LIVER for excretion from the body (reverse cholesterol transport). It also acts as a cofactor for LECITHIN CHOLESTEROL ACYLTRANSFERASE that forms CHOLESTEROL ESTERS on the HDL particles. Mutations of this gene APOA1 cause HDL deficiency, such as in FAMILIAL ALPHA LIPOPROTEIN DEFICIENCY DISEASE and in some patients with TANGIER DISEASE.Betaine: A naturally occurring compound that has been of interest for its role in osmoregulation. As a drug, betaine hydrochloride has been used as a source of hydrochloric acid in the treatment of hypochlorhydria. Betaine has also been used in the treatment of liver disorders, for hyperkalemia, for homocystinuria, and for gastrointestinal disturbances. (From Martindale, The Extra Pharmacopoeia, 30th ed, p1341)Kinetics: The rate dynamics in chemical or physical systems.Microsomes: Artifactual vesicles formed from the endoplasmic reticulum when cells are disrupted. They are isolated by differential centrifugation and are composed of three structural features: rough vesicles, smooth vesicles, and ribosomes. Numerous enzyme activities are associated with the microsomal fraction. (Glick, Glossary of Biochemistry and Molecular Biology, 1990; from Rieger et al., Glossary of Genetics: Classical and Molecular, 5th ed)Retinol O-Fatty-Acyltransferase: An enzyme that catalyzes the acyl group transfer of acyl COENZYME A to RETINOL to generate COENZYME A and a retinyl ester.Pentanoic AcidsEstersSubstrate Specificity: A characteristic feature of enzyme activity in relation to the kind of substrate on which the enzyme or catalytic molecule reacts.Phosphatidylcholines: Derivatives of phosphatidic acids in which the phosphoric acid is bound in ester linkage to a choline moiety. Complete hydrolysis yields 1 mole of glycerol, phosphoric acid and choline and 2 moles of fatty acids.Phospholipids: Lipids containing one or more phosphate groups, particularly those derived from either glycerol (phosphoglycerides see GLYCEROPHOSPHOLIPIDS) or sphingosine (SPHINGOLIPIDS). They are polar lipids that are of great importance for the structure and function of cell membranes and are the most abundant of membrane lipids, although not stored in large amounts in the system.Lipid Metabolism: Physiological processes in biosynthesis (anabolism) and degradation (catabolism) of LIPIDS.Palmitic Acids: A group of 16-carbon fatty acids that contain no double bonds.Organosilicon Compounds: Organic compounds that contain silicon as an integral part of the molecule.Lipoproteins, HDL: A class of lipoproteins of small size (4-13 nm) and dense (greater than 1.063 g/ml) particles. HDL lipoproteins, synthesized in the liver without a lipid core, accumulate cholesterol esters from peripheral tissues and transport them to the liver for re-utilization or elimination from the body (the reverse cholesterol transport). Their major protein component is APOLIPOPROTEIN A-I. HDL also shuttle APOLIPOPROTEINS C and APOLIPOPROTEINS E to and from triglyceride-rich lipoproteins during their catabolism. HDL plasma level has been inversely correlated with the risk of cardiovascular diseases.Dihydroxyacetone Phosphate: An important intermediate in lipid biosynthesis and in glycolysis.Penicillium chrysogenum: A mitosporic fungal species used in the production of penicillin.Microbodies: Electron-dense cytoplasmic particles bounded by a single membrane, such as PEROXISOMES; GLYOXYSOMES; and glycosomes.Rats, Inbred Strains: Genetically identical individuals developed from brother and sister matings which have been carried out for twenty or more generations or by parent x offspring matings carried out with certain restrictions. This also includes animals with a long history of closed colony breeding.Lipid Metabolism, Inborn Errors: Errors in the metabolism of LIPIDS resulting from inborn genetic MUTATIONS that are heritable.Oleic Acids: A group of fatty acids that contain 18 carbon atoms and a double bond at the omega 9 carbon.Molecular Sequence Data: Descriptions of specific amino acid, carbohydrate, or nucleotide sequences which have appeared in the published literature and/or are deposited in and maintained by databanks such as GENBANK, European Molecular Biology Laboratory (EMBL), National Biomedical Research Foundation (NBRF), or other sequence repositories.Epoxy Compounds: Organic compounds that include a cyclic ether with three ring atoms in their structure. They are commonly used as precursors for POLYMERS such as EPOXY RESINS.Microsomes, Liver: Closed vesicles of fragmented endoplasmic reticulum created when liver cells or tissue are disrupted by homogenization. They may be smooth or rough.Lysophosphatidylcholines: Derivatives of PHOSPHATIDYLCHOLINES obtained by their partial hydrolysis which removes one of the fatty acid moieties.Ketone Bodies: The metabolic substances ACETONE; 3-HYDROXYBUTYRIC ACID; and acetoacetic acid (ACETOACETATES). They are produced in the liver and kidney during FATTY ACIDS oxidation and used as a source of energy by the heart, muscle and brain.Caprylates: Derivatives of caprylic acid. Included under this heading are a broad variety of acid forms, salts, esters, and amides that contain a carboxy terminated eight carbon aliphatic structure.Palmitates: Salts and esters of the 16-carbon saturated monocarboxylic acid--palmitic acid.Vitamin B Deficiency: A condition due to deficiency in any member of the VITAMIN B COMPLEX. These B vitamins are water-soluble and must be obtained from the diet because they are easily lost in the urine. Unlike the lipid-soluble vitamins, they cannot be stored in the body fat.Amino Acid Sequence: The order of amino acids as they occur in a polypeptide chain. This is referred to as the primary structure of proteins. It is of fundamental importance in determining PROTEIN CONFORMATION.Starvation: Lengthy and continuous deprivation of food. (Stedman, 25th ed)Oleic Acid: An unsaturated fatty acid that is the most widely distributed and abundant fatty acid in nature. It is used commercially in the preparation of oleates and lotions, and as a pharmaceutical solvent. (Stedman, 26th ed)Lipids: A generic term for fats and lipoids, the alcohol-ether-soluble constituents of protoplasm, which are insoluble in water. They comprise the fats, fatty oils, essential oils, waxes, phospholipids, glycolipids, sulfolipids, aminolipids, chromolipids (lipochromes), and fatty acids. (Grant & Hackh's Chemical Dictionary, 5th ed)Dithionitrobenzoic Acid: A standard reagent for the determination of reactive sulfhydryl groups by absorbance measurements. It is used primarily for the determination of sulfhydryl and disulfide groups in proteins. The color produced is due to the formation of a thio anion, 3-carboxyl-4-nitrothiophenolate.Waxes: A plastic substance deposited by insects or obtained from plants. Waxes are esters of various fatty acids with higher, usually monohydric alcohols. The wax of pharmacy is principally yellow wax (beeswax), the material of which honeycomb is made. It consists chiefly of cerotic acid and myricin and is used in making ointments, cerates, etc. (Dorland, 27th ed)Coenzyme A Ligases: Enzymes that catalyze the formation of acyl-CoA derivatives. EC 6.2.1.Lipoproteins: Lipid-protein complexes involved in the transportation and metabolism of lipids in the body. They are spherical particles consisting of a hydrophobic core of TRIGLYCERIDES and CHOLESTEROL ESTERS surrounded by a layer of hydrophilic free CHOLESTEROL; PHOSPHOLIPIDS; and APOLIPOPROTEINS. Lipoproteins are classified by their varying buoyant density and sizes.Hypolipoproteinemias: Conditions with abnormally low levels of LIPOPROTEINS in the blood. This may involve any of the lipoprotein subclasses, including ALPHA-LIPOPROTEINS (high-density lipoproteins); BETA-LIPOPROTEINS (low-density lipoproteins); and PREBETA-LIPOPROTEINS (very-low-density lipoproteins).Lysophospholipase: An enzyme that catalyzes the hydrolysis of a single fatty acid ester bond in lysoglycerophosphatidates with the formation of glyceryl phosphatidates and a fatty acid. EC 22.214.171.124.Corneal Opacity: Disorder occurring in the central or peripheral area of the cornea. The usual degree of transparency becomes relatively opaque.Oxidation-Reduction: A chemical reaction in which an electron is transferred from one molecule to another. The electron-donating molecule is the reducing agent or reductant; the electron-accepting molecule is the oxidizing agent or oxidant. Reducing and oxidizing agents function as conjugate reductant-oxidant pairs or redox pairs (Lehninger, Principles of Biochemistry, 1982, p471).Palmitic Acid: A common saturated fatty acid found in fats and waxes including olive oil, palm oil, and body lipids.Clofibrate: A fibric acid derivative used in the treatment of HYPERLIPOPROTEINEMIA TYPE III and severe HYPERTRIGLYCERIDEMIA. (From Martindale, The Extra Pharmacopoeia, 30th ed, p986)Apolipoproteins: Protein components on the surface of LIPOPROTEINS. They form a layer surrounding the hydrophobic lipid core. There are several classes of apolipoproteins with each playing a different role in lipid transport and LIPID METABOLISM. These proteins are synthesized mainly in the LIVER and the INTESTINES.Monoglycerides: GLYCEROL esterified with a single acyl (FATTY ACIDS) chain.Apolipoproteins A: Structural proteins of the alpha-lipoproteins (HIGH DENSITY LIPOPROTEINS), including APOLIPOPROTEIN A-I and APOLIPOPROTEIN A-II. They can modulate the activity of LECITHIN CHOLESTEROL ACYLTRANSFERASE. These apolipoproteins are low in atherosclerotic patients. They are either absent or present in extremely low plasma concentration in TANGIER DISEASE.DiglyceridesErgothioneine: A naturally occurring metabolite of HISTIDINE that has antioxidant properties.Lipoylation: Covalent attachment of LIPIDS and FATTY ACIDS to other compounds and PROTEINS.Acetyl Coenzyme A: Acetyl CoA participates in the biosynthesis of fatty acids and sterols, in the oxidation of fatty acids and in the metabolism of many amino acids. It also acts as a biological acetylating agent.3-Hydroxybutyric Acid: BUTYRIC ACID substituted in the beta or 3 position. It is one of the ketone bodies produced in the liver.Palmitoyl-CoA Hydrolase: Enzyme catalyzing reversibly the hydrolysis of palmitoyl-CoA or other long-chain acyl coenzyme A compounds to yield CoA and palmitate or other acyl esters. The enzyme is involved in the esterification of fatty acids to form triglycerides. EC 126.96.36.199.Biological Transport: The movement of materials (including biochemical substances and drugs) through a biological system at the cellular level. The transport can be across cell membranes and epithelial layers. It also can occur within intracellular compartments and extracellular compartments.Acetyltransferases: Enzymes catalyzing the transfer of an acetyl group, usually from acetyl coenzyme A, to another compound. EC 2.3.1.Carrier Proteins: Transport proteins that carry specific substances in the blood or across cell membranes.Reye Syndrome: A form of encephalopathy with fatty infiltration of the LIVER, characterized by brain EDEMA and VOMITING that may rapidly progress to SEIZURES; COMA; and DEATH. It is caused by a generalized loss of mitochondrial function leading to disturbances in fatty acid and CARNITINE metabolism.Cloning, Molecular: The insertion of recombinant DNA molecules from prokaryotic and/or eukaryotic sources into a replicating vehicle, such as a plasmid or virus vector, and the introduction of the resultant hybrid molecules into recipient cells without altering the viability of those cells.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.Gene Expression Regulation, Enzymologic: Any of the processes by which nuclear, cytoplasmic, or intercellular factors influence the differential control of gene action in enzyme synthesis.Vitamin A: Retinol and derivatives of retinol that play an essential role in metabolic functioning of the retina, the growth of and differentiation of epithelial tissue, the growth of bone, reproduction, and the immune response. Dietary vitamin A is derived from a variety of CAROTENOIDS found in plants. It is enriched in the liver, egg yolks, and the fat component of dairy products.Sequence Homology, Amino Acid: The degree of similarity between sequences of amino acids. This information is useful for the analyzing genetic relatedness of proteins and species.Octoxynol: Nonionic surfactant mixtures varying in the number of repeating ethoxy (oxy-1,2-ethanediyl) groups. They are used as detergents, emulsifiers, wetting agents, defoaming agents, etc. Octoxynol-9, the compound with 9 repeating ethoxy groups, is a spermatocide.Lysophospholipids: Derivatives of PHOSPHATIDIC ACIDS that lack one of its fatty acyl chains due to its hydrolytic removal.Acyl Carrier Protein: Consists of a polypeptide chain and 4'-phosphopantetheine linked to a serine residue by a phosphodiester bond. Acyl groups are bound as thiol esters to the pantothenyl group. Acyl carrier protein is involved in every step of fatty acid synthesis by the cytoplasmic system.Base Sequence: The sequence of PURINES and PYRIMIDINES in nucleic acids and polynucleotides. It is also called nucleotide sequence.Chromatography, Thin Layer: Chromatography on thin layers of adsorbents rather than in columns. The adsorbent can be alumina, silica gel, silicates, charcoals, or cellulose. (McGraw-Hill Dictionary of Scientific and Technical Terms, 4th ed)Escherichia coli: A species of gram-negative, facultatively anaerobic, rod-shaped bacteria (GRAM-NEGATIVE FACULTATIVELY ANAEROBIC RODS) commonly found in the lower part of the intestine of warm-blooded animals. It is usually nonpathogenic, but some strains are known to produce DIARRHEA and pyogenic infections. Pathogenic strains (virotypes) are classified by their specific pathogenic mechanisms such as toxins (ENTEROTOXIGENIC ESCHERICHIA COLI), etc.RNA, Messenger: RNA sequences that serve as templates for protein synthesis. Bacterial mRNAs are generally primary transcripts in that they do not require post-transcriptional processing. Eukaryotic mRNA is synthesized in the nucleus and must be exported to the cytoplasm for translation. Most eukaryotic mRNAs have a sequence of polyadenylic acid at the 3' end, referred to as the poly(A) tail. The function of this tail is not known for certain, but it may play a role in the export of mature mRNA from the nucleus as well as in helping stabilize some mRNA molecules by retarding their degradation in the cytoplasm.DNA, Complementary: Single-stranded complementary DNA synthesized from an RNA template by the action of RNA-dependent DNA polymerase. cDNA (i.e., complementary DNA, not circular DNA, not C-DNA) is used in a variety of molecular cloning experiments as well as serving as a specific hybridization probe.Tissue Distribution: Accumulation of a drug or chemical substance in various organs (including those not relevant to its pharmacologic or therapeutic action). This distribution depends on the blood flow or perfusion rate of the organ, the ability of the drug to penetrate organ membranes, tissue specificity, protein binding. The distribution is usually expressed as tissue to plasma ratios.Phosphatidic Acids: Fatty acid derivatives of glycerophosphates. They are composed of glycerol bound in ester linkage with 1 mole of phosphoric acid at the terminal 3-hydroxyl group and with 2 moles of fatty acids at the other two hydroxyl groups.Fatty Acids, Nonesterified: FATTY ACIDS found in the plasma that are complexed with SERUM ALBUMIN for transport. These fatty acids are not in glycerol ester form.Ethylmaleimide: A sulfhydryl reagent that is widely used in experimental biochemical studies.Carbon Radioisotopes: Unstable isotopes of carbon that decay or disintegrate emitting radiation. C atoms with atomic weights 10, 11, and 14-16 are radioactive carbon isotopes.Recombinant Proteins: Proteins prepared by recombinant DNA technology.Polyketide Synthases: Large enzyme complexes composed of a number of component enzymes that are found in STREPTOMYCES which biosynthesize MACROLIDES and other polyketides.Mitochondria: Semiautonomous, self-reproducing organelles that occur in the cytoplasm of all cells of most, but not all, eukaryotes. Each mitochondrion is surrounded by a double limiting membrane. The inner membrane is highly invaginated, and its projections are called cristae. Mitochondria are the sites of the reactions of oxidative phosphorylation, which result in the formation of ATP. They contain distinctive RIBOSOMES, transfer RNAs (RNA, TRANSFER); AMINO ACYL T RNA SYNTHETASES; and elongation and termination factors. Mitochondria depend upon genes within the nucleus of the cells in which they reside for many essential messenger RNAs (RNA, MESSENGER). Mitochondria are believed to have arisen from aerobic bacteria that established a symbiotic relationship with primitive protoeukaryotes. (King & Stansfield, A Dictionary of Genetics, 4th ed)Membrane Lipids: Lipids, predominantly phospholipids, cholesterol and small amounts of glycolipids found in membranes including cellular and intracellular membranes. These lipids may be arranged in bilayers in the membranes with integral proteins between the layers and peripheral proteins attached to the outside. Membrane lipids are required for active transport, several enzymatic activities and membrane formation.Lipoprotein-X: An abnormal lipoprotein present in large amounts in patients with obstructive liver diseases such as INTRAHEPATIC CHOLESTASIS. LP-X derives from the reflux of BILE lipoproteins into the bloodstream. LP-X is a low-density lipoprotein rich in free CHOLESTEROL and PHOSPHOLIPIDS but poor in TRIGLYCERIDES; CHOLESTEROL ESTERS; and protein.Phosphatidate Phosphatase: A phosphomonoesterase involved in the synthesis of triacylglycerols. It catalyzes the hydrolysis of phosphatidates with the formation of diacylglycerols and orthophosphate. EC 188.8.131.52.Subcellular Fractions: Components of a cell produced by various separation techniques which, though they disrupt the delicate anatomy of a cell, preserve the structure and physiology of its functioning constituents for biochemical and ultrastructural analysis. (From Alberts et al., Molecular Biology of the Cell, 2d ed, p163)Sterol Esterase: An enzyme that catalyzes the hydrolysis of CHOLESTEROL ESTERS and some other sterol esters, to liberate cholesterol plus a fatty acid anion.Seeds: The encapsulated embryos of flowering plants. They are used as is or for animal feed because of the high content of concentrated nutrients like starches, proteins, and fats. Rapeseed, cottonseed, and sunflower seed are also produced for the oils (fats) they yield.Hydroxymethylglutaryl CoA Reductases: Enzymes that catalyze the reversible reduction of alpha-carboxyl group of 3-hydroxy-3-methylglutaryl-coenzyme A to yield MEVALONIC ACID.Hyperammonemia: Elevated level of AMMONIA in the blood. It is a sign of defective CATABOLISM of AMINO ACIDS or ammonia to UREA.Myocardium: The muscle tissue of the HEART. It is composed of striated, involuntary muscle cells (MYOCYTES, CARDIAC) connected to form the contractile pump to generate blood flow.Lipase: An enzyme of the hydrolase class that catalyzes the reaction of triacylglycerol and water to yield diacylglycerol and a fatty acid anion. It is produced by glands on the tongue and by the pancreas and initiates the digestion of dietary fats. (From Dorland, 27th ed) EC 184.108.40.206.Isoenzymes: Structurally related forms of an enzyme. Each isoenzyme has the same mechanism and classification, but differs in its chemical, physical, or immunological characteristics.Lipid A: Lipid A is the biologically active component of lipopolysaccharides. It shows strong endotoxic activity and exhibits immunogenic properties.Lipoproteins, LDL: A class of lipoproteins of small size (18-25 nm) and light (1.019-1.063 g/ml) particles with a core composed mainly of CHOLESTEROL ESTERS and smaller amounts of TRIGLYCERIDES. The surface monolayer consists mostly of PHOSPHOLIPIDS, a single copy of APOLIPOPROTEIN B-100, and free cholesterol molecules. The main LDL function is to transport cholesterol and cholesterol esters to extrahepatic tissues.Penicillium: A mitosporic Trichocomaceae fungal genus that develops fruiting organs resembling a broom. When identified, teleomorphs include EUPENICILLIUM and TALAROMYCES. Several species (but especially PENICILLIUM CHRYSOGENUM) are sources of the antibiotic penicillin.Apolipoprotein A-II: The second most abundant protein component of HIGH DENSITY LIPOPROTEINS or HDL. It has a high lipid affinity and is known to displace APOLIPOPROTEIN A-I from HDL particles and generates a stable HDL complex. ApoA-II can modulate the activation of LECITHIN CHOLESTEROL ACYLTRANSFERASE in the presence of APOLIPOPROTEIN A-I, thus affecting HDL metabolism.Clofibric Acid: An antilipemic agent that is the biologically active metabolite of CLOFIBRATE.Methylhydrazines: Hydrazines substituted by one or more methyl groups in any position.Phospholipases: A class of enzymes that catalyze the hydrolysis of phosphoglycerides or glycerophosphatidates. EC 3.1.-.Dietary Fats: Fats present in food, especially in animal products such as meat, meat products, butter, ghee. They are present in lower amounts in nuts, seeds, and avocados.Mersalyl: A toxic thiol mercury salt formerly used as a diuretic. It inhibits various biochemical functions, especially in mitochondria, and is used to study those functions.Glycerides: GLYCEROL esterified with FATTY ACIDS.Cardiolipins: Acidic phospholipids composed of two molecules of phosphatidic acid covalently linked to a molecule of glycerol. They occur primarily in mitochondrial inner membranes and in bacterial plasma membranes. They are the main antigenic components of the Wassermann-type antigen that is used in nontreponemal SYPHILIS SERODIAGNOSIS.Mitochondria, Muscle: Mitochondria of skeletal and smooth muscle. It does not include myocardial mitochondria for which MITOCHONDRIA, HEART is available.Cholesterol Ester Transfer Proteins: Proteins that bind to and transfer CHOLESTEROL ESTERS between LIPOPROTEINS such as LOW-DENSITY LIPOPROTEINS and HIGH-DENSITY LIPOPROTEINS.Enzyme Inhibitors: Compounds or agents that combine with an enzyme in such a manner as to prevent the normal substrate-enzyme combination and the catalytic reaction.Arachis hypogaea: A plant species of the family FABACEAE that yields edible seeds, the familiar peanuts, which contain protein, oil and lectins.Intracellular Membranes: Thin structures that encapsulate subcellular structures or ORGANELLES in EUKARYOTIC CELLS. They include a variety of membranes associated with the CELL NUCLEUS; the MITOCHONDRIA; the GOLGI APPARATUS; the ENDOPLASMIC RETICULUM; LYSOSOMES; PLASTIDS; and VACUOLES.Cyclohexanes: Six-carbon alicyclic hydrocarbons.Acetyl-CoA C-Acetyltransferase: An enzyme that catalyzes the formation of acetoacetyl-CoA from two molecules of ACETYL COA. Some enzymes called thiolase or thiolase-I have referred to this activity or to the activity of ACETYL-COA C-ACYLTRANSFERASE.Hydroxybutyrates: Salts and esters of hydroxybutyric acid.Multienzyme Complexes: Systems of enzymes which function sequentially by catalyzing consecutive reactions linked by common metabolic intermediates. They may involve simply a transfer of water molecules or hydrogen atoms and may be associated with large supramolecular structures such as MITOCHONDRIA or RIBOSOMES.Detergents: Purifying or cleansing agents, usually salts of long-chain aliphatic bases or acids, that exert cleansing (oil-dissolving) and antimicrobial effects through a surface action that depends on possessing both hydrophilic and hydrophobic properties.Cricetinae: A subfamily in the family MURIDAE, comprising the hamsters. Four of the more common genera are Cricetus, CRICETULUS; MESOCRICETUS; and PHODOPUS.Muscles: Contractile tissue that produces movement in animals.Polyethylene Glycols: Polymers of ETHYLENE OXIDE and water, and their ethers. They vary in consistency from liquid to solid depending on the molecular weight indicated by a number following the name. They are used as SURFACTANTS, dispersing agents, solvents, ointment and suppository bases, vehicles, and tablet excipients. Some specific groups are NONOXYNOLS, OCTOXYNOLS, and POLOXAMERS.Plasmalogens: GLYCEROPHOSPHOLIPIDS in which one of the two acyl chains is attached to glycerol with an ether alkenyl linkage instead of an ester as with the other glycerophospholipids.Cells, Cultured: Cells propagated in vitro in special media conducive to their growth. Cultured cells are used to study developmental, morphologic, metabolic, physiologic, and genetic processes, among others.Rats, Sprague-Dawley: A strain of albino rat used widely for experimental purposes because of its calmness and ease of handling. It was developed by the Sprague-Dawley Animal Company.TritiumStructure-Activity Relationship: The relationship between the chemical structure of a compound and its biological or pharmacological activity. Compounds are often classed together because they have structural characteristics in common including shape, size, stereochemical arrangement, and distribution of functional groups.Cell Membrane: The lipid- and protein-containing, selectively permeable membrane that surrounds the cytoplasm in prokaryotic and eukaryotic cells.Cholesterol, Dietary: Cholesterol present in food, especially in animal products.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.Mortierella: A genus of zygomycetous fungi of the family Mortierellaceae, order MUCORALES. Its species are abundant in soil and can cause rare infections in humans and animals. Mortierella alpinais is used for production of arachidonic acid.Diet: Regular course of eating and drinking adopted by a person or animal.Sterols: Steroids with a hydroxyl group at C-3 and most of the skeleton of cholestane. Additional carbon atoms may be present in the side chain. (IUPAC Steroid Nomenclature, 1987)Chondrodysplasia Punctata, Rhizomelic: An autosomal recessive form of CHONDRODYSPLASIA PUNCTATA characterized by defective plasmalogen biosynthesis and impaired peroxisomes. Patients have shortened proximal limbs and severely disturbed endochondral bone formation. The metabolic defects associated with the impaired peroxisomes are present only in the rhizomelic form of chondrodysplasia punctata. (From Scriver et al, Metabolic Basis of Inherited Disease, 6th ed, p1497)Acyl-CoA Dehydrogenase: A flavoprotein oxidoreductase that has specificity for medium-chain fatty acids. It forms a complex with ELECTRON TRANSFERRING FLAVOPROTEINS and conveys reducing equivalents to UBIQUINONE.Deoxycholic Acid: A bile acid formed by bacterial action from cholate. It is usually conjugated with glycine or taurine. Deoxycholic acid acts as a detergent to solubilize fats for intestinal absorption, is reabsorbed itself, and is used as a choleretic and detergent.Molecular Structure: The location of the atoms, groups or ions relative to one another in a molecule, as well as the number, type and location of covalent bonds.
Carnitine octanoyltransferase (EC 220.127.116.11) is a carnitine acyltransferase that catalyzes the reversible transfer of fatty ... 2000). "Genomics of the human carnitine acyltransferase genes". Mol. Genet. Metab. 71 (1-2): 139-53. doi:10.1006/mgme.2000.3055 ... 2000) reviewed the function, structural features, and phylogenetics of human carnitine acyltransferase genes, including CROT.[ ... 2002). "Structural model of a malonyl-CoA-binding site of carnitine octanoyltransferase and carnitine palmitoyltransferase I: ...
Fatty acyl-CoA esters
There carnitine acyltransferase II reverses the process, producing fatty acyl-CoA and carnitine. This shuttle mechanism is ... They are converted to fatty acyl carnitine by carnitine acyltransferase I, an enzyme of the inner leaflet of the outer ... Fatty acyl carnitine is then transported by an antiport in exchange for free carnitine to the inner surface of the inner ...
Fatty acid degradation
The liberated carnitine returns to the cytosol. It is important to note that carnitine acyltransferase I undergoes allosteric ... This occurs via a series of similar steps: Acyl CoA is conjugated to carnitine by carnitine acyltransferase I ( ... is converted to acyl CoA by carnitine acyltransferase (palmitoyltransferase) II located on the inner mitochondrial membrane. ... I located on the outer mitochondrial membrane Acyl carnitine is shuttled inside by a translocase Acyl carnitine (such as ...
Chromosome 11 (human)
Glycine-N-acyltransferase GLYATL2 encoding protein Glycine-N-acyltransferase like 2 GPHA2: Glycoprotein hormone alpha-2 GYLTL1B ... carnitine palmitoyltransferase 1A (liver) CREBZF encoding protein CREB/ATF bZIP transcription factor DAK: Triokinase/FMN ... monoacylglycerol O-acyltransferase 2 MTRNR2L8: encoding protein MT-RNR2-like 8 NADSYN1: NAD synthetase 1 NAP1L4: nucleosome ... DGAT2 encoding protein Diacylglycerol O-acyltransferase 2 DHCR7: 7-dehydrocholesterol reductase DKK3: Dickkopf-related protein ...
Transporter Classification Database
List of EC numbers (EC 2)
... diacylglycerol O-acyltransferase EC 18.104.22.168: carnitine O-palmitoyltransferase EC 22.214.171.124: 2-acylglycerol O-acyltransferase EC ... phosphatidylcholine-retinol O-acyltransferase EC 126.96.36.199: polysialic-acid O-acetyltransferase EC 188.8.131.52: carnitine O- ... sphingosine N-acyltransferase EC 184.108.40.206: plasmalogen synthase EC 220.127.116.11: sterol O-acyltransferase EC 18.104.22.168: cortisol O- ... retinol O-fatty-acyltransferase EC 22.214.171.124: triacylglycerol-sterol O-acyltransferase EC 126.96.36.199: heparan-a-glucosaminide N- ...
List of MeSH codes (D08)
... carnitine acyltransferases MeSH D08.811.913.050.350.170 --- carnitine O-acetyltransferase MeSH D08.811.913.050.350.200 --- ... phosphatidylcholine-sterol O-acyltransferase MeSH D08.811.913.050.646 --- retinol O-fatty-acyltransferase MeSH D08.811.913.050. ... diacylglycerol o-acyltransferase MeSH D08.811.913.050.425 --- glycerol-3-phosphate O-acyltransferase MeSH D08.811.913.050.600 ... 1-acylglycerol-3-phosphate O-acyltransferase MeSH D08.811.913.050.175 --- 1-acylglycerophosphocholine O-acyltransferase MeSH ...
Other names in common use include medium-chain/long-chain carnitine acyltransferase, carnitine medium-chain acyltransferase, ... Farrell SO, Fiol CJ, Reddy JK, Bieber LL (1984). "Properties of purified carnitine acyltransferases of mouse liver peroxisomes ... Is overt carnitine palmitoyltransferase of liver peroxisomal carnitine octanoyltransferase?". Biochem. J. 249 (1): 231-7. PMC ... easily solubilized mitochondrial carnitine palmitoyltransferase, and overt mitochondrial carnitine palmitoyltransferase. As of ...
PDOC00402 - Acyltransferases ChoActase / COT / CPT family in PROSITE Choline/Carnitine o-acyltransferase family[permanent dead ... A related transferase is carnitine acyltransferase. Palmitoylcarnitine Palmitoyl CoA There are four different forms of CPT in ... Carnitine O-palmitoyltransferase (also called carnitine palmitoyltransferase) is a mitochondrial transferase enzyme (EC 2.3. ... humans: CPT1A - associated with Carnitine palmitoyltransferase I deficiency CPT1B CPT1C CPT2 - associated with carnitine ...
Cronin CN (Sep 1997). "The conserved serine-threonine-serine motif of the carnitine acyltransferases is involved in carnitine ... carnitine acetyl coenzyme A transferase, carnitine acetylase, carnitine acetyltransferase, carnitine-acetyl-CoA transferase, ... "Carnitine acyltransferase enzymic catalysis requires a positive charge on the carnitine cofactor". Archives of Biochemistry and ... Jogl G, Hsiao YS, Tong L (Nov 2004). "Structure and function of carnitine acyltransferases". Annals of the New York Academy of ...
Carnitine palmitoyltransferase I
... (CPT1) also known as carnitine acyltransferase I, CPTI, CAT1, CoA:carnitine acyl transferase ( ... It is part of a family of enzymes called carnitine acyltransferases. This "preparation" allows for subsequent movement of the ... Carnitine palmitoyltransferase I is the first component and rate-limiting step of the carnitine palmitoyltransferase system, ... One such mechanism based upon a carnitine acetyltransferase model is shown below in which the His 473 deprotonates carnitine ...
Carnitine palmitoyltransferase II deficiency
CPT II shares structural elements with other members of the carnitine acyltransferase protein family. The crystal structure of ... carnitine Carnitine O-palmitoyltransferase Carnitine palmitoyltransferase I deficiency Fasciculation Myokymia Primary carnitine ... 1993). "Comparison of the active sites of the purified carnitine acyltransferases from peroxisomes and mitochondria by using a ... The "carnitine shuttle" is composed of three enzymes that utilize carnitine to facilitate the import of hydrophobic long-chain ...
2004). "Downregulation of carnitine acyltransferases and organic cation transporter OCTN2 in mononuclear cells in healthy ... 1999). "Primary systemic carnitine deficiency is caused by mutations in a gene encoding sodium ion-dependent carnitine ... 1999). "Carnitine transporter OCTN2 mutations in systemic primary carnitine deficiency: a novel Arg169Gln mutation and a ... 1999). "Mutations of OCTN2, an organic cation/carnitine transporter, lead to deficient cellular carnitine uptake in primary ...
... (or O-acyltransferase), DGAT, catalyzes the formation of triglycerides from diacylglycerol and Acyl ... cholesterol acyltransferase-related enzymes". The Journal of Biological Chemistry. 273 (41): 26765-71. doi:10.1074/jbc.273.41. ... a second mammalian diacylglycerol acyltransferase, and related family members". The Journal of Biological Chemistry. 276 (42): ... "Acylation of acylglycerols by acyl coenzyme A:diacylglycerol acyltransferase 1 (DGAT1). Functional importance of DGAT1 in the ...
β-oxidation in the peroxisome requires the use of a peroxisomal carnitine acyltransferase (instead of carnitine acyltransferase ... Acyl-carnitine is shuttled inside by a carnitine-acylcarnitine translocase, as a carnitine is shuttled outside. Acyl-carnitine ... The liberated carnitine is shuttled back to the cytosol, as an acyl-carnitine is shuttled into the matrix. If the fatty acyl- ... Acyl-CoA is transferred to the hydroxyl group of carnitine by carnitine palmitoyltransferase I, located on the cytosolic faces ...
Carnitine has the ability to lower blood triglyceride levels. In some cases, fibrates have been used to bring down ... Diglyceride acyltransferase, enzyme responsible for triglyceride biosynthesis Medium-chain triglycerides Lipid profile Lipids ... ISBN 978-1-58829-551-4. Balch, Phyllis A. (2006). "Carnitine". Prescription for nutritional healing (4th ed.). New York: Avery ...
acetyl-CoA C-acyltransferase activity. • long-chain-enoyl-CoA hydratase activity. Cellular component. • membrane. • ... Trifunctional enzyme subunit beta, mitochondrial (TP-beta) also known as 3-ketoacyl-CoA thiolase, acetyl-CoA acyltransferase, ... Carnitine-acylcarnitine translocase. *Carnitine palmitoyltransferase II. Beta oxidation. General. *Acyl CoA dehydrogenase ( ...
Fatty acid synthase
SLC22A3 is an extraneuronal monoamine transporter that is present in astrocytes, and SLC22A5 is a high-affinity carnitine ... CYP2D6, dopamine β-hydroxylase, flavin-containing monooxygenase 3, butyrate-CoA ligase, and glycine N-acyltransferase are the ... "Substrate/Product". glycine N-acyltransferase. BRENDA. Technische Universität Braunschweig. Retrieved 7 May 2014. Haiser HJ, ...
Chromosome 5 (human)
ICD-10 Chapter IV: Endocrine, nutritional and metabolic diseases
Rytting E, Audus KL (January 2005). "Novel organic cation transporter 2-mediated carnitine uptake in placental choriocarcinoma ... and glycine N-acyltransferase (GLYAT) are the enzymes known to metabolize amphetamine or its metabolites in humans. Amphetamine ... and SLC22A5 is a high-affinity carnitine transporter. Amphetamine is known to strongly induce cocaine- and amphetamine- ... the role of glycine N-acyltransferase, and factors that influence interindividual variation". Expert Opin. Drug Metab. Toxicol ...
Retrospective diagnosis of carnitine-acylcarnitine translocase deficiency by acylcarnitine analysis in the proband Guthrie card...
Retrospective diagnosis of carnitine-acylcarnitine translocase deficiency by acylcarnitine analysis in the proband Guthrie card ... Retrospective diagnosis of carnitine-acylcarnitine translocase deficiency by acylcarnitine analysis in the proband Guthrie card ... Carnitine Acyltransferases * Carnitine O-Palmitoyltransferase * Female * Fibroblasts * Humans * Infant * Intracellular ...
l-carnitine acyltransferase in intact peroxisomes is inhibited by malonyl-CoA | Biochemical Journal
l-carnitine acyltransferase in intact peroxisomes is inhibited by malonyl-CoA. J P Derrick, R R Ramsay ... l-carnitine acyltransferase in intact peroxisomes is inhibited by malonyl-CoA Message Subject (Your Name) has forwarded a page ... In the past, the contribution of peroxisomal carnitine acyltransferase activity to the generation of medium- and long-chain ... Thus the overt carnitine acyltransferase activities in peroxisomes and mitochondria have the same properties. Further, the ...
Enzymes That Break Down Fat Cells | LIVESTRONG.COM
Structure-guided enzymology of the lipid A acyltransferase LpxM reveals a dual activity mechanism | PNAS
Acetyl-L-carnitine deficiency and major depressive disorder. A study finds that individuals with major depressive disorder had ... monoacylglycerol acyltransferases (MGATs), and acylglycerolphosphate acyltransferases (AGPATs), which are each necessary for ... 2015) Acyltransferase inhibitors: A patent review (2010-present). Expert Opin Ther Pat 25(2):145-158. ... AbLpxM Possesses Acyltransferase and Acylprotein Thioesterase Activities.. To measure activity of AbLpxM, we measured the ...
JCI - Insulin sensitivity: modulation by nutrients and inflammation
DAG acyltransferase; GPAT, glycerol-3-phosphate acyltransferase; HFD, high-fat diet; IKK, IκB kinase; IMTG, intramuscular TG; ... Nonstandard abbreviations used: ATM, adipose tissue macrophage; cPKC, conventional PKC; CPT-I, carnitine palmitoyltransferase I ... The final step in TG synthesis is the addition of a fatty acyl-CoA to DAG by DAG acyltransferase (DGAT). Another important ... The rate-limiting step for β-oxidation of long-chain fatty acids is their transport into the mitochondria via carnitine ...
Search: All Pfam signatures | InterPro | EMBL-EBI
Choline/carnitine acyltransferase domain (IPR039551) Pfam signature: PF00755 Putative esterase (IPR000801) Pfam signature: ... 2-oxoacid dehydrogenase acyltransferase, catalytic domain (IPR001078) Pfam signature: PF00198 Catalase core domain (IPR011614) ... Phospholipid/glycerol acyltransferase (IPR002123) Pfam signature: PF01553 Multi antimicrobial extrusion protein (IPR002528) ...
Carnitine palmitoyltransferase 1B isoform a variant - Homo sapiens (Human)
AcyltransferaseUniRule annotation. ,p>Information which has been generated by the UniProtKB automatic annotation system, ... Carnitine palmitoyltransferase 1B isoform a variantImported. ,p>Information which has been imported from another database using ... Belongs to the carnitine/choline acetyltransferase family.UniRule annotation. ,p>Information which has been generated by the ... tr,Q53FV7,Q53FV7_HUMAN Carnitine palmitoyltransferase 1B isoform a variant (Fragment) OS=Homo sapiens OX=9606 PE=2 SV=1 ...
Carnitine O-palmitoyltransferase - Wikipedia
PDOC00402 - Acyltransferases ChoActase / COT / CPT family in PROSITE Choline/Carnitine o-acyltransferase family[permanent dead ... A related transferase is carnitine acyltransferase. Palmitoylcarnitine Palmitoyl CoA There are four different forms of CPT in ... Carnitine O-palmitoyltransferase (also called carnitine palmitoyltransferase) is a mitochondrial transferase enzyme (EC 2.3. ... humans: CPT1A - associated with Carnitine palmitoyltransferase I deficiency CPT1B CPT1C CPT2 - associated with carnitine ...
JCI - Contribution of de novo fatty acid synthesis to hepatic steatosis and insulin resistance: lessons from genetically...
... mitochondrial glycerol 3-phosphate acyltransferase (GPAT) and diacylglycerol acyltransferase (DGAT) for TG synthesis (26) ( ... The mitochondrial carnitine palmitoyltransferase system. From concept to molecular analysis. Eur. J. Biochem. 244:1-14. View ... Hepatic overexpression of glycerol-sn-3-phosphate acyltransferase 1 in rats causes insulin resistance. J. Biol. Chem. 282:14807 ... Characterization of the murine mitochondrial glycerol-3-phosphate acyltransferase promoter. J. Biol. Chem. 270:1416-1421. View ...
Assimacopoulos-Jeannet F, Thumelin S, Roche E, Esser V, McGarry JD, Prentki M: Fatty acids rapidly induce the carnitine ... glycerol-palmitate acyltransferase; GSIS, glucose-stimulated insulin secretion; KATP channel, ATP-sensitive potassium channel; ... Colquhoun A: Induction of apoptosis by polyunsaturated fatty acids and its relationship to fatty acid inhibition of carnitine ... ACC, acetyl-CoA carboxylase; CPT-1, carnitine palmitoyltransferase-1; FACoA, FFA-derived long chain acyl-CoA esters; FAS, fatty ...
Carnitine palmitoyltransferase I - Wikipedia
Carnitine palmitoyltransferase I (CPT1) also known as carnitine acyltransferase I, CPTI, CAT1, CoA:carnitine acyl transferase ( ... It is part of a family of enzymes called carnitine acyltransferases. This "preparation" allows for subsequent movement of the ... Carnitine palmitoyltransferase I is the first component and rate-limiting step of the carnitine palmitoyltransferase system, ... One such mechanism based upon a carnitine acetyltransferase model is shown below in which the His 473 deprotonates carnitine ...
Trimethylamine oxide accumulation in marine animals: relationship to acylglycerol storagej | Journal of Experimental Biology
... diacylglycerol acyltransferase; (5,6) carnitine palmitoyl transferases I and II; (7) trimethylamine oxygenase; (8) choline ... Marzo, A. and Curti, S. (1997). l-Carnitine moiety assay: an up-to-date reappraisal covering the commonest methods for various ... One possible cause is competition for carnitine which, like choline, may be oxidized to TMAO (Marzo and Curti, 1997). ... Minana, M., Hermenegildo, C., Llansola, M., Montoliu, C., Grisolia, S. and Felip, V. (1996). Carnitine and choline derivatives ...
Cardiac Muscle | SpringerLink
Different carnitine acyl transferases in calfliver. Biochim. Biophys. Acta 280:422-433.PubMedGoogle Scholar ... Effects of chronic ethanol ingestion upon acyl-CoA carnitine acyltransferase in liver and heart. Lipids 9:520-525.PubMed ... Long-chain carnitine acyltransferase and the role of acylcarnitine derivatives in the catalytic increase of fatty acid ... Carnitine in intermediary metabolism. Reversible acetylation of carnitine by mitochondria.J. Biol. Chem. 237:2228-2231.PubMed ...
Cpt1c MGI Mouse Gene Detail - MGI:2446526 - carnitine palmitoyltransferase 1c
IPR000542 Acyltransferase ChoActase/COT/CPT. IPR042572 Carnitine o-acyltransferase, N-terminal. IPR032476 Carnitine O- ... J:79496 Price N, et al., A novel brain-expressed protein related to carnitine palmitoyltransferase I. Genomics. 2002 Oct;80(4): ... IPR042232 Choline/Carnitine o-acyltransferase, domain 1. IPR042231 Choline/Carnitine o-acyltransferase, domain 2 ...
Set 16 Enzymes and Hormones Flashcards by Languages 247365 | Brainscape
Insulin Resistance: Metabolic Mechanisms and Consequences in the Heart | Arteriosclerosis, Thrombosis, and Vascular Biology
CAT indicates carnitine acyltransferase; CPT, carnitine palmitoyltransferase; DAG, diacylglycerol; FAT, fatty acid translocase ... FFAs are transported across the outer and inner mitochondrial membrane by carnitine palmitoyltransferase 1, which is the rate- ... limiting step of FA oxidation and carnitine palmitoyltransferase 2. The acetyl-CoA resulting from β-oxidation enters the ... a potent inhibitor of carnitine palmitoyltransferase 1. AMPK directly enhances insulin signaling in endothelial cells, ...
MEDLINE - Resultado p gina 1
Carnitine Acyltransferases); EC 188.8.131.52 (Carnitine O-Palmitoyltransferase); S7UI8SM58A (Carnitine). [Em] M s de entrada:. 1705 ... 0 (Membrane Glycoproteins); 0 (MicroRNAs); EC 2.3.1.- (Carnitine Acyltransferases); EC 2.3.1.- (carnitine octanoyltransferase ... Carnitine Acyltransferases); EC 2.3.1.- (carnitine octanoyltransferase); EC 184.108.40.206 (Hadhb protein, mouse); EC 220.127.116.11 ( ... A group of genes, namely CPT1A, CPT1B, CPT1C, CPT2, CRAT and CROT, encode for three carnitine acyltransferases that are ...
MEDLINE - Resultado p gina 1
Carnitine Acyltransferases); EC 2.3.1.- (Slc25a20 protein, rat); K848JZ4886 (Cysteine); N762921K75 (Nitrogen); S7UI8SM58A ( ... The inhibition resulted to be competitive with respect the substrate, carnitine. NO competed also with NEM, correlating well ... S-nitrosylation of the mitochondrial carnitine/acylcarnitine transporter (CACT) has been investigated on the native and the ... Nitric oxide inhibits the mitochondrial carnitine/acylcarnitine carrier through reversible S-nitrosylation of cysteine 136.. ...
Acetate trafficking in the heart : carnitine acyltransferases matter. Physiological Reports, 4 (19) ... Diacylglycerol acyltransferase 2 acts upstream of diacylglycerol acyltransferase 1 and utilizes nascent diglycerides andde ... Diacylglycerol acyltransferase 2 links glucose utilization to fatty acid oxidation in the brown adipocytes. Journal of Lipid ... Evidence that diacylglycerol acyltransferase 1 (DGAT1) has dual membrane topology in the endoplasmic reticulum of HepG2 cells. ...
The Cellular Stress Response Interactome and Extracellular Matrix Cross-Talk during Fibrosis: A Stressed Extra-Matrix Affair |...
Carnitine Infusion and Insulin Resistance - Full Text View - ClinicalTrials.gov
Carnitine acyltransferase (CRaT) enzyme activity (physiological parameter) [ Time Frame: 6 hours ]. CRaT activity will be ... The first trial includes lipid infusion combined with L-Carnitine infusion (=LIPID + CAR). In the second trial, L-carnitine ... intravenous Lipid infusion (IntraLipid) combined with carnitor (L-carnitine) infusion. L-Carnitine will be administrated ... Carnitine Infusion and Insulin Resistance. The safety and scientific validity of this study is the responsibility of the study ...
Human Metabolome Database: Showing Protein Carnitine O-palmitoyltransferase 1, liver isoform (HMDBP00309)
Involved in acyltransferase activity. Specific Function. Catalyzes the transfer of the acyl group of long-chain fatty acid-CoA ... Structural model of carnitine palmitoyltransferase I based on the carnitine acetyltransferase crystal. Biochem J. 2004 May 1; ... Showing Protein Carnitine O-palmitoyltransferase 1, liver isoform (HMDBP00309). IdentificationBiological propertiesGene ... IJlst L, Mandel H, Oostheim W, Ruiter JP, Gutman A, Wanders RJ: Molecular basis of hepatic carnitine palmitoyltransferase I ...
... acyltransferase activity,IEA; GO:0009437,carnitine metabolism,IMP; GO:0016740,transferase activity,IEA. ... Putative mitochondrial carnitine O-acetyltransferase related cluster. 3395. 0.0. 93% (646/688). 18.104.22.168. Alanine and aspartate ... R] COG0596 Predicted hydrolases or acyltransferases (alpha/beta hydrolase superfamily) 1967. 0.0. 95% (381/400). General ... GO:0004092,carnitine O-acetyltransferase activity,IEA; GO:0005739,mitochondrion,IDA; GO:0005739,mitochondrion,IEA; GO:0006066, ...
Frontiers | First Description of Reduced Pyruvate Dehydrogenase Enzyme Activity Following Subarachnoid Hemorrhage (SAH) |...
Bresolin, N., Freddo, L., Vergani, L., and Angelini, C. (1982). Carnitine, carnitine acyltransferases, and rat brain function. ... Prevention of postischemic canine neurological injury through potentiation of brain energy metabolism by acetyl-L-carnitine. ... that intravenous administration of acetyl-L-carnitine (ALCAR) reduced brain lactate levels and improved neurological outcome, ( ...
BRENDA - Reference to 22.214.171.124; Id = 664215
Chapter 16 Flashcards by Margeaux Oliva | Brainscape
fatty acyl portion transferred onto carnitine by carnitine acyltransferase-1 (inhibited by malonyl CoA) ... carnitine acyltransferase 2 transfers fatty acyl group back to CoA (mitochondrial matrix) ... symptoms: profound fasting hypoglycermia, low to bsent ketons, lethargy, coma, death if untreated, C8-C10 acyl carnitines in ... long chain fatty acids (14-20 C) transported into mitochondria via carnitine shuttle ...
Human Metabolome Database: Showing metabocard for Propionyl-CoA (HMDB0001275)
4. Carnitine O-acetyltransferase. General function:. Involved in acyltransferase activity. Specific function:. Carnitine ... Glycine N-acyltransferase-like protein 1. General function:. Involved in glycine N-acyltransferase activity. Specific function: ... Glycerol-3-phosphate acyltransferase 4. General function:. Involved in acyltransferase activity. Specific function:. Esterifies ... Lysocardiolipin acyltransferase 1. General function:. Involved in acyltransferase activity. Specific function:. Acyl-CoA: ...
Metabolic Signatures of Kidney Yang Deficiency Syndrome and Protective Effects of Two Herbal Extracts in Rats Using GC/TOF MS
Acylcarnitines are synthesized by carnitine acyl transferases from acylCoA and carnitine. Carnitine induces fatty acid β- ... J. Bremer, "Carnitine in intermediary metabolism. The metabolism of fatty acid esters of carnitine by mitochondria," The ... I. B. Fritz, "Action of carnitine on long chain fatty acid oxidation by liver," The American Journal of Physiology, vol. 197, ... I. B. Fritz and K. T. Yue, "Effects of carnitine on acetyl-CoA oxidation by heart muscle mitochondria," The American Journal of ...
Human Metabolome Database: Showing metabocard for TG(16:1(9Z)/18:1(9Z)/20:4(5Z,8Z,11Z,14Z)) (HMDB0005441)
Carnitine O-palmitoyltransferase 1, muscle isoform. General function:. Involved in acyltransferase activity. Specific function: ... Carnitine O-palmitoyltransferase 1, liver isoform. General function:. Involved in acyltransferase activity. Specific function: ... Carnitine O-palmitoyltransferase 2, mitochondrial. General function:. Involved in acyltransferase activity. Specific function: ... Glycerol-3-phosphate acyltransferase 3. General function:. Involved in acyltransferase activity. Specific function:. Esterifies ...
Absence of Perilipin 2 Prevents Hepatic Steatosis, Glucose Intolerance and Ceramide Accumulation in Alcohol-Fed Mice - PubMed
Feeding acetyl-l-carnitine and lipoic acid to old rats significantly improves metabolic function while decreasing oxidative...
MDA and 4-hydroxy-nonenal also inactivate carnitine acyltransferase and decrease the binding affinity for substrates (35). Thus ... l-carnitine (ALCAR), a mitochondrial metabolite, reverses the age-related decline in tissue carnitine levels and improves ... acetyl-l-carnitine;. LA,. lipoic acid;. MDA,. malondialdehyde;. ROS,. reactive oxygen species. ... To address whether the dietary addition of acetyl-l-carnitine [ALCAR, 1.5% (wt/vol) in the drinking water] and/or (R)-α-lipoic ...
DeficiencyMitochondriaMetabolismInhibitionSupplementationOxidationCoenzymeMetabolicAcylcarnitinesLipidGlycerol-3-phosphate acyltransferaHepaticHormone sensitGroups other than aminoacyl groupsTranslocaseAdministration of L-CarnitineGeneIsoformInhibitor of carnitineAcetylcarnitineBiosynthesisFree carnitineAcyl carnitinesCholineEstersMembraneDietaryActivityFatty acyl groupsSerum
- Thus the overt carnitine acyltransferase activities in peroxisomes and mitochondria have the same properties. (biochemj.org)
- This "preparation" allows for subsequent movement of the acyl carnitine from the cytosol into the intermembrane space of mitochondria. (wikipedia.org)
- Reversible acetylation of carnitine by mitochondria. (springer.com)
- The metabolism of fatty acid esters of carnitine by mitochondria. (springer.com)
- S-nitrosylation of the mitochondrial carnitine/acylcarnitine transporter (CACT) has been investigated on the native and the recombinant proteins reconstituted in proteoliposomes, and on intact mitochondria. (bireme.br)
- A group of fats called long-chain fatty acids cannot enter mitochondria unless they are attached to a substance known as carnitine. (medlineplus.gov)
- Carnitine palmitoyltransferase 1A connects carnitine to long-chain fatty acids so they can cross the inner membrane of mitochondria. (medlineplus.gov)
- Once these fatty acids are inside mitochondria, carnitine is removed and they can be metabolized to produce energy. (medlineplus.gov)
- Once these fatty acids are inside mitochondria, carnitine palmitoyltransferase 2 removes the carnitine and adds a substance called coenzyme A. Long-chain fatty acids must be joined to coenzyme A before they can be metabolized to produce energy. (medlineplus.gov)
- Without enough functioning carnitine palmitoyltransferase 2, long-chain fatty acids are not properly processed after they enter mitochondria and cannot be metabolized to produce energy. (medlineplus.gov)
- Is carnitine palmitoyltransferase inhibited by a malonyl-CoA-binding unit in the mitochondria? (biochemsoctrans.org)
- It has been postulated that adequate glucose supply, conversion of acetyl-CoA to malonyl-CoA by acetyl-CoA carboxylase, and the resultant inhibition of carnitine palmitoyltransferase I (CPT I) ( 26 ) inhibit fatty acid uptake and oxidation by placental mitochondria in utero ( 20 ). (physiology.org)
- Gerbling, H., Gerhardt, B. (1988) Carnitine acyltransferase activity of mitochondria from mung bean hypocotyls. (springer.com)
- Briefly, L-carnitine is needed to transport long-chain FA from the cytosol to sites of beta-oxidation in the mitochondria. (plos.org)
- As mitochondrial membranes are impermeable to coenzyme A (CoA) esters and long-chain fatty acids, binding of l -carnitine to acetyl groups via carnitine acyltransferase is essential for the shuttle of the acetylated fatty acids into the mitochondria and for their subsequent β-oxidation in the matrix ( Figure 1 ) [ 4 ]. (mdpi.com)
- Low free L-carnitine levels have been shown to result in a 2- to 3-fold downregulation of the expression of carnitine acyltransferases, the enzymes involved in fatty acid oxidation in the mitochondria. (nutraceuticalsworld.com)
- L-carnitine is essential for the transfer of long-chain fatty acids from the cytosol to mitochondria for subsequent beta oxidation. (biomedcentral.com)
- Carnitine in intermediary metabolism. (springer.com)
- Carnitine--metabolism and functions. (semanticscholar.org)
- Given its pivotal role in fatty acid oxidation and energy metabolism, l -carnitine has been investigated as ergogenic aid for enhancing exercise capacity in the healthy athletic population. (mdpi.com)
- Apparently the extra carnitine allows more rapid transport of fatty acids and a more efficient energy metabolism. (cliffsnotes.com)
- ENGLISH ABSTRACT: Carnitine plays an essential role in eukaryotic metabolism by mediating the shuttling of activated acyl residues between intracellular compartments. (sun.ac.za)
- Carnitine plays a major role in energy metabolism. (bioportfolio.com)
- We will evaluate whether carnitine, a dietary supplement, reduces liver fat and improves metabolism in individuals who have a high concentration of fat within their liver. (bioportfolio.com)
- therefore it is not surprising that carnitine metabolism is impaired in patients and experimental animals with certain types of chronic liver disease. (biomedcentral.com)
- Inhibition of the overt mitochondrial carnitine palmitoyltransferase by malonyl-CoA is important in the regulation of fatty acid oxidation. (biochemj.org)
- The mechanism of substrate inhibition of palmityl coenzyme A: Carnitine palmityltransferase by palmityl coenzyme A. (springer.com)
- The inhibition resulted to be competitive with respect the substrate, carnitine. (bireme.br)
- Sekas, Paul: Inhibition of carnitine acyltransferase activities by bile acids in rat liver peroxisomes. (antikoerper-online.de)
- and 4) inhibition of gluconeogenesis, including inhibition of the long-chain acyl-CoA-carnitine acyltransferase I, the long-chain acylcarnitine translocase, and pyruvate carboxylase. (diabetesjournals.org)
- A primary biochemical action of malonyl-CoA is the inhibition of the acyltransferase activity of carnitine palmitoyltransferase-1 (CPT-1). (physiology.org)
- This condition demands supplementation of free carnitine above the normal dietary intake to continue to remove (detoxify) the accumulating organic acids. (hmdb.ca)
- Carnitine in Human Muscle Bioenergetics: Can Carnitine Supplementation Improve Physical Exercise? (nih.gov)
- 7 years) vs. mature adult (≤7 years) dogs, and supplementation with L-carnitine attenuated the effects of aging. (plos.org)
- Later studies point to the positive impact of dietary supplementation with l -carnitine on the recovery process after exercise. (mdpi.com)
- The supplementation-based increase in serum and muscle l -carnitine contents is suggested to enhance blood flow and oxygen supply to the muscle tissue via improved endothelial function thereby reducing hypoxia-induced cellular and biochemical disruptions. (mdpi.com)
- Samimi M, Jamilian M, Ebrahimi FA, Rahimi M, Tajbakhsh B, Asemi Z. Oral carnitine supplementation reduces body weight and insulin resistance in women with polycystic ovary syndrome: a randomized, double-blind, placebo-controlled trial. (springermedizin.de)
- A new study confirms previous research that daily supplementation with 2 grams of L-carnitine in the form of Lonza's Carnipure tartrate during pregnancy can significantly reduce the increase of plasma free fatty acid levels frequently observed during pregnancy. (nutraceuticalsworld.com)
- Additionally, the plasma levels of L-carnitine were found to be higher after supplementation. (nutraceuticalsworld.com)
- Symptoms of fatigue and functional status improved significantly in those patients who received oral L-carnitine supplementation. (bioportfolio.com)
- The carnitine palmitoyltransferase system is an essential step in the beta-oxidation of long chain fatty acids. (wikipedia.org)
- Catalyzes the transfer of the acyl group of long-chain fatty acid-CoA conjugates onto carnitine, an essential step for the mitochondrial uptake of long-chain fatty acids and their subsequent beta-oxidation in the mitochondrion. (hmdb.ca)
- Feeding old rats acetyl- l -carnitine (ALCAR), a mitochondrial metabolite, reverses the age-related decline in tissue carnitine levels and improves mitochondrial fatty acid β-oxidation in the tissues studied ( 15 - 18 ). (pnas.org)
- Akkaoui M, Cohen I, Esnous C, Lenoir V, Sournac M, Girard J, Prip-Buus C. Modulation of the hepatic malonyl-CoA-carnitine palmitoyltransferase 1A partnership creates a metabolic switch allowing oxidation of de novo fatty acids. (medlineplus.gov)
- Carnitine is an essential factor in long-chain fatty acid oxidation. (nih.gov)
- Subcellular localization of monoglyceride acyltransferase, xanthine oxidation, NADP: isocitrate dehydrogenase and alkaline phosphatase in the mucosa of the guinea-pig small intestine. (athabascau.ca)
- GPAT1 and GPAT2 on the outer mitochondrial membrane compete with carnitine palmitoyl transferase-1 for acyl-CoAs that may be used for glycerolipid biosynthesis or for β-oxidation. (nih.gov)
- Carnitine performs an obligate role in mitochondrial oxidation of long-chain fatty acids (FA) through the action of specialized acyltransferases . (plos.org)
- Under normal conditions, the availability of carnitine is not a limiting step in beta-oxidation, although an increase in carnitine content may increase the rate of FA oxidation, permitting a reduction of glucose utilization, preserving muscle glycogen content, and ensuring maximal rates of oxidative ATP production . (plos.org)
- Long-chain Carnitine Acyltransferase and the Role of Acylcarnitine Derivatives in the Catalytic Increase of Fatty Acid Oxidation Induced by Carnitine. (semanticscholar.org)
- Etomoxir is an inhibitor of carnitine palmitoyltransferase A (CPT1), which is required for the oxidation of long-chain acyl CoA esters. (adooq.com)
- Carnitine palmitoyltransferase I is the first component and rate-limiting step of the carnitine palmitoyltransferase system, catalyzing the transfer of the acyl group from coenzyme A to carnitine to form palmitoylcarnitine. (wikipedia.org)
- Carnitine acyltransferases catalyze the exchange of acyl groups between coenzyme A (CoA) and carnitine. (rcsb.org)
- Carnitine palmitoyltransferase-1 is in turn regulated by two important cytoplasmic enzymes, 5′-adenosine monophosphate (AMP)-activated protein kinase (AMPK) and acetyl coenzyme A (CoA) carboxylase (ACC) (2) . (onlinejacc.org)
- Recent evidence suggests that a low carnitine availability may limit acetylcarnitine formation, thereby reducing metabolic flexibility. (clinicaltrials.gov)
- To provide more direct insight into the effect of carnitine in preventing metabolic inflexibility and insulin resistance and to further explore the mechanism of action is the focus of this research. (clinicaltrials.gov)
- Objective: The primary objectives are to investigate whether L-carnitine infusion may rescue lipid-induced insulin resistance and whether L-carnitine infusion is improving metabolic flexibility in the state of lipid-induced insulin resistance. (clinicaltrials.gov)
- To investigate whether L-Carnitine infusion may rescue lipid induced insulin resistance and improve metabolic flexibility three intervention trials are included. (clinicaltrials.gov)
- L-carnitine (LC), and its acetylated form, acetyl L-carnitine (ALC), have immense functional capabilities to regulate the oxidative and metabolic status of the female reproductive system. (springermedizin.de)
- Carnitine is used as a dietary supplement by endurance athletes and in the treatment of certain metabolic diseases. (cliffsnotes.com)
- To date, the only reported metabolic and physiological roles for carnitine in Saccharomyces cerevisiae are related to the activity of the carnitine shuttle. (sun.ac.za)
- Treatment with L-carnitine, or HMF extract improved obesity and its associated metabolic problems in different degrees. (biomedcentral.com)
- In the past, the contribution of peroxisomal carnitine acyltransferase activity to the generation of medium- and long-chain acylcarnitines in the cytoplasm has been ignored. (biochemj.org)
- SLC25A20) mediates an antiport reaction allowing entry of acyl moieties in the form of acylcarnitines into the mitochondrial matrix and exit of free carnitine. (bireme.br)
- Fatty acids and long-chain acylcarnitines (fatty acids still attached to carnitine) may also build up in cells and damage the liver, heart, and muscles. (medlineplus.gov)
- To this end, insulin resistance will be induced by lipid infusion in healthy volunteers and it will be tested whether carnitine co-infusion can alleviate insulin resistance. (clinicaltrials.gov)
- The first trial includes lipid infusion combined with L-Carnitine infusion (=LIPID + CAR). (clinicaltrials.gov)
- In the second trial, L-carnitine infusion will be replaced by placebo infusion in the form of saline (= LIPID + PLAC) in order to investigate the effect of L-Carnitine. (clinicaltrials.gov)
- Import of acylcarnitine into mitochondrial matrix through carnitine/acylcarnitine-translocase (CACT) is fundamental for lipid catabolism. (nih.gov)
- Promoting lipid utilization with l-carnitine to improve oocyte quality. (springermedizin.de)
- meanwhile treatment with L-carnitine, or HMF significantly normalized the lipid profile. (biomedcentral.com)
- Glycerol-3-phosphate acyltransferase then esterifies a fatty acid to glycerol-3-phosphate thereby generating lysophosphatidic acid. (hmdb.ca)
- Four homologous isoforms of glycerol-3-phosphate acyltransferase (GPAT), each the product of a separate gene, catalyze the synthesis of lysophosphatidic acid from glycerol-3-phosphate and long-chain acyl-CoA. (nih.gov)
- Expression of the PPAR-α and γ, lipoprotein lipase (LPL), hormone sensitive lipase (HSL), carnitine palmitoyl acyltransferase-1 (CPT-1), insulin receptor, GLUT-4 and adipokines were assessed in epidydimal fat. (greenmedinfo.com)
- Fatty acid synthase activity was suppressed in mice fed the 0.4% and 0.8% puerarin diets, while the activities of AMP-activated protein kinase (AMPK), carnitine acyltransferase (CAT) and hormone-sensitive lipase (HSL) were increased. (jove.com)
Groups other than aminoacyl groups1
- Carnitine octanoyltransferase (CROT or COT), also known as octanoyl-CoA: L-carnitine O-octanoyltransferase, medium-chain/long-chain carnitine acyltransferase, and carnitine medium-chain acyltransferase, is a carnitine acyltransferase belonging to the family of transferases, specifically those acyltransferases transferring groups other than aminoacyl groups that catalyzes the reversible transfer of fatty acyl groups between CoA and carnitine. (sinobiological.com)
- A translocase then shuttles the acyl carnitine across the inner mitochondrial membrane where it is converted back into palmitoyl-CoA. (wikipedia.org)
- An inner membrane carnitine-acylcarnitine translocase exchanges carnitine and acylcarnitine across the inner membrane but its role is long-chain acyl transfer has not been established. (nih.gov)
Administration of L-Carnitine1
Inhibitor of carnitine1
- Furthermore, a secondary objective is to examine the molecular pathways of carnitine and acetylcarnitine, responsible for muscle insulin sensitivity. (clinicaltrials.gov)
- Carnitine, acetylcarnitine and the activity of carnitine acyltransferases in seminal plasma and sper. (biomedsearch.com)
- Carnitine, acetylcarnitine and the activity of carnitine acyltransferases in seminal plasma and spermatozoa of men, rams and rats. (springermedizin.de)
- the results from animal studies and preliminary human studies show that carnitine availability and acetylcarnitine concentrations are low in insulin resistant states such as with type 2 di. (bioportfolio.com)
- Carnitine palmitoyltransferase A (CPT-A), located on the external surface of the inner membrane, catalyzes the conversion of cytoplasmic long-chain acyl-CoA and carnitine into acylcarnitine. (nih.gov)
- The acylcarnitine is reconverted to intramitochondrial acyl-CoA by the action of carnitine palmitoyltransferase B located in the inner membrane. (nih.gov)
- A small molecule, carnitine , serves as a carrier across the mitochondrial membrane. (cliffsnotes.com)
- Carnitine acyltransferase I, which is located on the outer mitochondrial membrane, transfers the fatty acyl group from fatty acyl‐CoA to the hydroxyl (OH) group of carnitine. (cliffsnotes.com)
- To address whether the dietary addition of acetyl- l -carnitine [ALCAR, 1.5% (wt/vol) in the drinking water] and/or ( R )-α-lipoic acid [LA, 0.5% (wt/wt) in the chow] improved these endpoints, young (2-4 mo) and old (24-28 mo) F344 rats were supplemented for up to 1 mo before death and hepatocyte isolation. (pnas.org)
- In summary, dietary MCT, fish oil, and L-carnitine counterbalanced the effects of aging on circulating concentrations of these compounds. (plos.org)
- These mutations lead to reduced activity of carnitine palmitoyltransferase 2. (medlineplus.gov)
- It is known that malonyl-CoA inhibits the acyltransferase activity of carnitine palmitoyltransferase-1 (CPT-1) that converts long-chain fatty acyl-CoA (LCFA-CoA) to long-chain acylcarnitine ( 9 , 23 ). (physiology.org)
- Comparison of the effects of L-carnitine and acetyl-L-carnitine on carnitine levels, ambulatory activity, and oxidative stress biomarkers in the brain of old rats. (springermedizin.de)