Carnitine acyltransferases. Medical search

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.

An enzyme that catalyzes the formation of O-acetylcarnitine from acetyl-CoA plus carnitine. EC 2.3.1.7.

A constituent of STRIATED MUSCLE and LIVER. It is an amino acid derivative and an essential cofactor for fatty acid metabolism.

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.

An enzyme that catalyzes reversibly the conversion of palmitoyl-CoA to palmitoylcarnitine in the inner mitochondrial membrane. EC 2.3.1.21.

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 2.3.1.23.

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 2.3.1.15.

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.

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 2.3.1.124.

The addition of an organic acid radical into a molecule.

A coenzyme A derivative which plays a key role in the fatty acid synthesis in the cytoplasmic and microsomal systems.

Microbodies which occur in animal and plant cells and in certain fungi and protozoa. They contain peroxidase, catalase, and allied enzymes. (From Singleton and Sainsbury, Dictionary of Microbiology and Molecular Biology, 2nd ed)

Electron-dense cytoplasmic particles bounded by a single membrane, such as PEROXISOMES; GLYOXYSOMES; and glycosomes.

The glyceryl esters of a fatty acid, or of a mixture of fatty acids. They are generally odorless, colorless, and tasteless if pure, but they may be flavored according to origin. Fats are insoluble in water, soluble in most organic solvents. They occur in animal and vegetable tissue and are generally obtained by boiling or by extraction under pressure. They are important in the diet (DIETARY FATS) as a source of energy. (Grant & Hackh's Chemical Dictionary, 5th ed)

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.

Specialized connective tissue composed of fat cells (ADIPOCYTES). It is the site of stored FATS, usually in the form of TRIGLYCERIDES. In mammals, there are two types of adipose tissue, the WHITE FAT and the BROWN FAT. Their relative distributions vary in different species with most adipose tissue being white.

A chromosome disorder associated either with an extra chromosome 21 or an effective trisomy for chromosome 21. Clinical manifestations include hypotonia, short stature, brachycephaly, upslanting palpebral fissures, epicanthus, Brushfield spots on the iris, protruding tongue, small ears, short, broad hands, fifth finger clinodactyly, Simian crease, and moderate to severe INTELLECTUAL DISABILITY. Cardiac and gastrointestinal malformations, a marked increase in the incidence of LEUKEMIA, and the early onset of ALZHEIMER DISEASE are also associated with this condition. Pathologic features include the development of NEUROFIBRILLARY TANGLES in neurons and the deposition of AMYLOID BETA-PROTEIN, similar to the pathology of ALZHEIMER DISEASE. (Menkes, Textbook of Child Neurology, 5th ed, p213)

Interruptions in the sugar-phosphate backbone of DNA, across both strands adjacently.

LIPOLYSIS of stored LIPIDS in the ADIPOSE TISSUE to release FREE FATTY ACIDS. Mobilization of stored lipids is under the regulation of lipolytic signals (CATECHOLAMINES) or anti-lipolytic signals (INSULIN) via their actions on the hormone-sensitive LIPASE. This concept does not include lipid transport.

The metabolic process of breaking down LIPIDS to release FREE FATTY ACIDS, the major oxidative fuel for the body. Lipolysis may involve dietary lipids in the DIGESTIVE TRACT, circulating lipids in the BLOOD, and stored lipids in the ADIPOSE TISSUE or the LIVER. A number of enzymes are involved in such lipid hydrolysis, such as LIPASE and LIPOPROTEIN LIPASE from various tissues.

One of the BIOLOGICAL SCIENCE DISCIPLINES concerned with the origin, structure, development, growth, function, genetics, and reproduction of animals, plants, and microorganisms.

An order of fish comprising salmons, trouts, whitefish, graylings, and other families. They are both marine and freshwater fish, found in all oceans and are quite numerous in the Northern Hemisphere. (From Nelson: Fishes of the World)

A superorder of CEPHALOPODS comprised of squid, cuttlefish, and their relatives. Their distinguishing feature is the modification of their fourth pair of arms into tentacles, resulting in 10 limbs.

Periodic movements of animals in response to seasonal changes or reproductive instinct. Hormonal changes are the trigger in at least some animals. Most migrations are made for reasons of climatic change, feeding, or breeding.

A huge subclass of mostly marine CRUSTACEA, containing over 14,000 species. The 10 orders comprise both planktonic and benthic organisms, and include both free-living and parasitic forms. Planktonic copepods form the principle link between PHYTOPLANKTON and the higher trophic levels of the marine food chains.

The physical measurements of a body.

Developmental or acquired stricture or narrowing of the LARYNX. Symptoms of respiratory difficulty depend on the degree of laryngeal narrowing.

A chronic multi-system disorder of CONNECTIVE TISSUE. It is characterized by SCLEROSIS in the SKIN, the LUNGS, the HEART, the GASTROINTESTINAL TRACT, the KIDNEYS, and the MUSCULOSKELETAL SYSTEM. Other important features include diseased small BLOOD VESSELS and AUTOANTIBODIES. The disorder is named for its most prominent feature (hard skin), and classified into subsets by the extent of skin thickening: LIMITED SCLERODERMA and DIFFUSE SCLERODERMA.

A type of extracellular vesicle, containing RNA and proteins, that is secreted into the extracellular space by EXOCYTOSIS when MULTIVESICULAR BODIES fuse with the PLASMA MEMBRANE.

Any pathological condition where fibrous connective tissue invades any organ, usually as a consequence of inflammation or other injury.

A meshwork-like substance found within the extracellular space and in association with the basement membrane of the cell surface. It promotes cellular proliferation and provides a supporting structure to which cells or cell lysates in culture dishes adhere.

The unfavorable effect of environmental factors (stressors) on the physiological functions of an organism. Prolonged unresolved physiological stress can affect HOMEOSTASIS of the organism, and may lead to damaging or pathological conditions.

In the YIN-YANG system of philosophy and medicine, a lack of vital energy (called yangxu in Chinese). It manifests itself in various systemic and organic diseases. (The Pinyin Chinese-English Dictionary, 1979)

Individual's rights to obtain and use information collected or generated by others.

The dynamic collection of metabolites which represent a cell's or organism's net metabolic response to current conditions.

The systematic identification and quantitation of all the metabolic products of a cell, tissue, organ, or organism under varying conditions. The METABOLOME of a cell or organism is a dynamic collection of metabolites which represent its net response to current conditions.

A quantitative measure of the frequency on average with which articles in a journal have been cited in a given period of time.

The period of history before 500 of the common era.

Therapeutic practices which are not currently considered an integral part of conventional allopathic medical practice. They may lack biomedical explanations but as they become better researched some (PHYSICAL THERAPY MODALITIES; DIET; ACUPUNCTURE) become widely accepted whereas others (humors, radium therapy) quietly fade away, yet are important historical footnotes. Therapies are termed as Complementary when used in addition to conventional treatments and as Alternative when used instead of conventional treatment.

Diminished effectiveness of INSULIN in lowering blood sugar levels: requiring the use of 200 units or more of insulin per day to prevent HYPERGLYCEMIA or KETOSIS.

An extremely variable eczematous skin disease that is presumed to be a response to prolonged vigorous scratching, rubbing, or pinching to relieve intense pruritus. It varies in intensity, severity, course, and morphologic expression in different individuals. Neurodermatitis is believed by some to be psychogenic. The circumscribed or localized form is often referred to as lichen simplex chronicus.

A primary source of energy for living organisms. It is naturally occurring and is found in fruits and other parts of plants in its free state. It is used therapeutically in fluid and nutrient replacement.

A 51-amino acid pancreatic hormone that plays a major role in the regulation of glucose metabolism, directly by suppressing endogenous glucose production (GLYCOGENOLYSIS; GLUCONEOGENESIS) and indirectly by suppressing GLUCAGON secretion and LIPOLYSIS. Native insulin is a globular protein comprised of a zinc-coordinated hexamer. Each insulin monomer containing two chains, A (21 residues) and B (30 residues), linked by two disulfide bonds. Insulin is used as a drug to control insulin-dependent diabetes mellitus (DIABETES MELLITUS, TYPE 1).

FATTY ACIDS found in the plasma that are complexed with SERUM ALBUMIN for transport. These fatty acids are not in glycerol ester form.

Physiological processes in biosynthesis (anabolism) and degradation (catabolism) of LIPIDS.

The hollow, muscular organ that maintains the circulation of the blood.

Substances that lower the levels of certain LIPIDS in the BLOOD. They are used to treat HYPERLIPIDEMIAS.

Chemicals that bind to and remove ions from solutions. Many chelating agents function through the formation of COORDINATION COMPLEXES with METALS.

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).

Conditions with excess LIPIDS in the blood.

A metallic element of atomic number 30 and atomic weight 65.38. It is a necessary trace element in the diet, forming an essential part of many enzymes, and playing an important role in protein synthesis and in cell division. Zinc deficiency is associated with ANEMIA, short stature, HYPOGONADISM, impaired WOUND HEALING, and geophagia. It is known by the symbol Zn.

Submitochondrial and subcellular distributions of the carnitine-acylcarnitine carrier. (1/96)

The submitochondrial and subcellular distributions of the carnitine-acylcarnitine translocase (CAC) have been studied. CAC is enriched to a much lesser extent than the carnitine palmitoyltransferases within the contact sites of mitochondria. A high-abundance protein of identical molecular size as the mitochondrial CAC that is immunoreactive with an anti-peptide antibody raised against a linear epitope of mitochondrial CAC is present in peroxisomes but not in microsomes. This suggests that CAC is targeted to at least two different locations within the liver cell and that acylcarnitine transport into peroxisomes is CAC mediated. (+info)

Molecular characterization of carnitine-dependent transport of acetyl-CoA from peroxisomes to mitochondria in Saccharomyces cerevisiae and identification of a plasma membrane carnitine transporter, Agp2p. (2/96)

In Saccharomyces cerevisiae, beta-oxidation of fatty acids is confined to peroxisomes. The acetyl-CoA produced has to be transported from the peroxisomes via the cytoplasm to the mitochondrial matrix in order to be degraded to CO(2) and H(2)O. Two pathways for the transport of acetyl-CoA to the mitochondria have been proposed. The first involves peroxisomal conversion of acetyl-CoA into glyoxylate cycle intermediates followed by transport of these intermediates to the mitochondria. The second pathway involves peroxisomal conversion of acetyl-CoA into acetylcarnitine, which is subsequently transported to the mitochondria. Using a selective screen, we have isolated several mutants that are specifically affected in the second pathway, the carnitine-dependent acetyl-CoA transport from the peroxisomes to the mitochondria, and assigned these CDAT mutants to three different complementation groups. The corresponding genes were identified using functional complementation of the mutants with a genomic DNA library. In addition to the previously reported carnitine acetyl-CoA transferase (CAT2), we identified the genes for the yeast orthologue of the human mitochondrial carnitine acylcarnitine translocase (YOR100C or CAC) and for a transport protein (AGP2) required for carnitine transport across the plasma membrane. (+info)

Arrhythmias and conduction defects as presenting symptoms of fatty acid oxidation disorders in children. (3/96)

BACKGROUND: The clinical manifestations of inherited disorders of fatty acid oxidation vary according to the enzymatic defect. They may present as isolated cardiomyopathy, sudden death, progressive skeletal myopathy, or hepatic failure. Arrhythmia is an unusual presenting symptom of fatty acid oxidation deficiencies. METHODS AND RESULTS: Over a period of 25 years, 107 patients were diagnosed with an inherited fatty acid oxidation disorder. Arrhythmia was the predominant presenting symptom in 24 cases. These 24 cases included 15 ventricular tachycardias, 4 atrial tachycardias, 4 sinus node dysfunctions with episodes of atrial tachycardia, 6 atrioventricular blocks, and 4 left bundle-branch blocks in newborn infants. Conduction disorders and atrial tachycardias were observed in patients with defects of long-chain fatty acid transport across the inner mitochondrial membrane (carnitine palmitoyl transferase type II deficiency and carnitine acylcarnitine translocase deficiency) and in patients with trifunctional protein deficiency. Ventricular tachycardias were observed in patients with any type of fatty acid oxidation deficiency. Arrhythmias were absent in patients with primary carnitine carrier, carnitine palmitoyl transferase I, and medium chain acyl coenzyme A dehydrogenase deficiencies. CONCLUSIONS: The accumulation of arrhythmogenic intermediary metabolites of fatty acids, such as long-chain acylcarnitines, may be responsible for arrhythmias. Inborn errors of fatty acid oxidation should be considered in unexplained sudden death or near-miss in infants and in infants with conduction defects or ventricular tachycardia. Diagnosis can be easily ascertained by an acylcarnitine profile from blood spots on filter paper. (+info)

Evidence for triacylglycerol synthesis in the lumen of microsomes via a lipolysis-esterification pathway involving carnitine acyltransferases. (4/96)

In this study a pathway for the synthesis of triacylglycerol (TAG) within the lumen of the endoplasmic reticulum has been identified, using microsomes that had been preconditioned by depleting their endogenous substrates and then fusing them with biotinylated phosphatidylserine liposomes containing CoASH and Mg(2+). Incubating these fused microsomes with tri[(3)H] oleoylglycerol and [(14)C]oleoyl-CoA yielded microsome-associated triacylglycerol, which resisted extensive washing and had a [(3)H]:[(14)C] ratio close to 2:1. The data suggest that the precursor tri[(3)H]oleoylglycerol was hydrolyzed by microsomal lipase to membrane-bound di[(3)H]oleoylglycerol and subsequently re-esterified with luminal [(14)C]oleoyl-CoA. The accumulation of TAG within the microsomes, even when overt diacylglycerol acyltransferase (DGAT I) was inactive, is consistent with the existence of a latent diacylglycerol acyltransferase (DGAT II) within the microsomal lumen. Moreover, because luminal synthesis of TAG was carnitine-dependent and markedly reduced by glybenclamide, a potent carnitine acyltransferase inhibitor, microsomal carnitine acyltransferase appears to be essential for trafficking the [(14)C]oleoyl-CoA into the microsomal lumen for subsequent incorporation into newly synthesized TAG. This study thus provides the first direct demonstration of an enzymatic process leading to the synthesis of luminal triacylglycerol, which is a major component of very low density lipoproteins. (+info)

Identification of the two histidine residues responsible for the inhibition by malonyl-CoA in peroxisomal carnitine octanoyltransferase from rat liver. (5/96)

Carnitine octanoyltransferase (COT), an enzyme that facilitates the transport of medium chain fatty acids through peroxisomal membranes, is inhibited by malonyl-CoA. cDNAs encoding full-length wild-type COT and one double mutant variant from rat peroxisomal COT were expressed in Saccharomyces cerevisiae. Both expressed forms were expressed similarly in quantitative terms and exhibited full enzyme activity. The wild-type-expressed COT was inhibited by malonyl-CoA like the liver enzyme. The activity of the enzyme encoded by the double mutant H131A/H340A was completely insensitive to malonyl-CoA in the range assayed (2-200 microM). These results indicate that the two histidine residues, H131 and H340, are the sites responsible for inhibition by malonyl-CoA. Another mutant variant, H327A, abolishes the enzyme activity, from which it is concluded that it plays an important role in catalysis. (+info)

Identification and functions of new transporters in yeast mitochondria. (6/96)

The genome of Saccharomyces cerevisiae encodes 35 putative members of the mitochondrial carrier family. Known members of this family transport substrates and products across the inner membranes of mitochondria. We are attempting to identify the functions of the yeast mitochondrial transporters via high-yield expression in Escherichia coli and/or S. cerevisiae, purification and reconstitution of their protein products into liposomes, where their transport properties are investigated. With this strategy, we have already identified the functions of seven S. cerevisiae gene products, whose structural and functional properties assigned them to the mitochondrial carrier family. The functional information obtained in the reconstituted system and the use of knock-out yeast strains can be usefully exploited for the investigation of the physiological role of individual transporters. Furthermore, the yeast carrier sequences can be used to identify the orthologous proteins in other organisms, including man. (+info)

Inhibition by etomoxir of rat liver carnitine octanoyltransferase is produced through the co-ordinate interaction with two histidine residues. (7/96)

Rat peroxisomal carnitine octanoyltransferase (COT), which facilitates the transport of medium-chain fatty acids through the peroxisomal membrane, is irreversibly inhibited by the hypoglycaemia-inducing drug etomoxir. To identify the molecular basis of this inhibition, cDNAs encoding full-length wild-type COT, two different variant point mutants and one variant double mutant from rat peroxisomal COT were expressed in Saccharomyces cerevisiae, an organism devoid of endogenous COT activity. The recombinant mutated enzymes showed activity towards both carnitine and decanoyl-CoA in the same range as the wild type. Whereas the wild-type version expressed in yeast was inhibited by etomoxir in an identical manner to COT from rat liver peroxisomes, the activity of the enzyme containing the double mutation H131A/H340A was completely insensitive to etomoxir. Individual point mutations H131A and H340A also drastically reduced sensitivity to etomoxir. Taken together, these results indicate that the two histidine residues, H131 and H340, are the sites responsible for inhibition by etomoxir and that the full inhibitory properties of the drug will be shown only if both histidines are intact at the same time. Our data demonstrate that both etomoxir and malonyl-CoA inhibit COT by interacting with the same sites. (+info)

Molecular enzymology of carnitine transfer and transport. (8/96)

Carnitine (L-3-hydroxy-4-N-trimethylaminobutyric acid) forms esters with a wide range of acyl groups and functions to transport and excrete these groups. It is found in most cells at millimolar levels after uptake via the sodium-dependent carrier, OCTN2. The acylation state of the mobile carnitine pool is linked to that of the limited and compartmentalised coenzyme A pools by the action of the family of carnitine acyltransferases and the mitochondrial membrane transporter, CACT. The genes and sequences of the carriers and the acyltransferases are reviewed along with mutations that affect activity. After summarising the accepted enzymatic background, recent molecular studies on the carnitine acyltransferases are described to provide a picture of the role and function of these freely reversible enzymes. The kinetic and chemical mechanisms are also discussed in relation to the different inhibitors under study for their potential to control diseases of lipid metabolism. (+info)

Carnitine octanoyltransferase (EC 2.3.1.137) 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: ...

https://en.wikipedia.org/wiki/CROT_(gene)

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 ...

https://en.wikipedia.org/wiki/Fatty_acyl-CoA_esters

... carnitine palmitoyltransferase and glycerophosphate acyltransferase compared to peroxisomal beta-oxidation and palmitoyl-CoA ...

https://en.wikipedia.org/wiki/Palmitoyl-CoA_hydrolase

... carnitine acyltransferase, AMP-activated protein kinase, and others. These enzymes are important targets for drug discovery ...

https://en.wikipedia.org/wiki/Liang_Tong

... carnitine and carnitine acyltransferases I and II, reducing their bioavailability and consequently inhibiting beta oxidation of ...

https://en.wikipedia.org/wiki/Blighia_sapida

... new fatty acids and can inhibit the transfer of the fatty acyl group from acyl CoA to carnitine with carnitine acyltransferase ...

https://en.wikipedia.org/wiki/Acetyl-CoA_carboxylase

... carnitine acyltransferases MeSH D08.811.913.050.350.170 - carnitine O-acetyltransferase MeSH D08.811.913.050.350.200 - ... diacylglycerol o-acyltransferase MeSH D08.811.913.050.425 - glycerol-3-phosphate O-acyltransferase MeSH D08.811.913.050.600 - ... phosphatidylcholine-sterol O-acyltransferase MeSH D08.811.913.050.646 - retinol O-fatty-acyltransferase MeSH D08.811.913.050. ... 1-acylglycerol-3-phosphate O-acyltransferase MeSH D08.811.913.050.175 - 1-acylglycerophosphocholine O-acyltransferase MeSH ...

https://en.wikipedia.org/wiki/List_of_MeSH_codes_(D08)

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 ...

https://en.wikipedia.org/wiki/Chromosome_11

... but not as a protease or a carnitine acyltransferase". Archives of Biochemistry and Biophysics. 323 (2): 397-403. doi:10.1006/ ...

https://en.wikipedia.org/wiki/PDIA3

Family 4.C.2 The Carnitine O-Acyl Transferase (CrAT) Family 4.C.3 The Acyl-CoA Thioesterase (AcoT) Family 4.D.1 The Putative ... Family 2.A.14 Lactate Permease Family 2.A.15 The Betaine/Carnitine/Choline Transporter (BCCT) Family 2.A.16 Tellurite- ...

https://en.wikipedia.org/wiki/Transporter_Classification_Database

... diacylglycerol O-acyltransferase EC 2.3.1.21: carnitine O-palmitoyltransferase EC 2.3.1.22: 2-acylglycerol O-acyltransferase EC ... phosphatidylcholine-retinol O-acyltransferase EC 2.3.1.136: polysialic-acid O-acetyltransferase EC 2.3.1.137: carnitine O- ... sphingosine N-acyltransferase EC 2.3.1.25: plasmalogen synthase EC 2.3.1.26: sterol O-acyltransferase EC 2.3.1.27: cortisol O- ... retinol O-fatty-acyltransferase EC 2.3.1.77: triacylglycerol-sterol O-acyltransferase EC 2.3.1.78: heparan-a-glucosaminide N- ...

https://en.wikipedia.org/wiki/List_of_EC_numbers_(EC_2)

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 ...

https://en.wikipedia.org/wiki/Fatty_acid_degradation

This reaction takes place in the mitochondrial matrix and is catalyzed by carnitine acyltransferase 2 (also called carnitine ... L-Carnitine, acetyl-l-carnitine, and propionyl-l-carnitine are available in dietary supplement pills or powders, with a daily ... Carnitine exists as one of two stereoisomers (the two enantiomers d-carnitine (S-(+)-) and l-carnitine (R-(-)-)). Both are ... Two types of carnitine deficiency states exist. Primary carnitine deficiency is a genetic disorder of the cellular carnitine- ...

https://en.wikipedia.org/wiki/Carnitine

"Downregulation of carnitine acyltransferases and organic cation transporter OCTN2 in mononuclear cells in healthy elderly and ... an organic cation/carnitine transporter, lead to deficient cellular carnitine uptake in primary carnitine deficiency". Human ... characterized by impaired carnitine transport, urinary carnitine wasting, low serum carnitine levels, reduced intracellular ... "Primary systemic carnitine deficiency is caused by mutations in a gene encoding sodium ion-dependent carnitine transporter". ...

https://en.wikipedia.org/wiki/SLC22A5

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. doi: ... easily solubilized mitochondrial carnitine palmitoyltransferase, and overt mitochondrial carnitine palmitoyltransferase. As of ...

https://en.wikipedia.org/wiki/Carnitine_O-octanoyltransferase

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 ...

https://en.wikipedia.org/wiki/Carnitine_O-palmitoyltransferase

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 ...

https://en.wikipedia.org/wiki/Carnitine_O-acetyltransferase

... (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 ... van der Leij FR, Huijkman NC, Boomsma C, Kuipers JR, Bartelds B (2000). "Genomics of the human carnitine acyltransferase genes ... Carnitine palmitoyltransferase I is the first component and rate-limiting step of the carnitine palmitoyltransferase system, ...

https://en.wikipedia.org/wiki/Carnitine_palmitoyltransferase_I

CPT II shares structural elements with other members of the carnitine acyltransferase protein family. The crystal structure of ... 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 ...

https://en.wikipedia.org/wiki/Carnitine_palmitoyltransferase_II_deficiency

... (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 ...

https://en.wikipedia.org/wiki/Diglyceride_acyltransferase

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 ...

https://en.wikipedia.org/wiki/Beta_oxidation

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 ( ...

https://en.wikipedia.org/wiki/HADHB

Glycerol-3-phosphate O-acyltransferase. *1-acylglycerol-3-phosphate O-acyltransferase. *2-acylglycerol-3-phosphate O- ... Carnitine palmitoyltransferase I. *Long-chain-fatty-acid-CoA ligase. tryptophan metabolism. *Kynureninase ...

https://en.wikipedia.org/wiki/Citrate_synthase

Glycerol-3-phosphate O-acyltransferase. *1-acylglycerol-3-phosphate O-acyltransferase. *2-acylglycerol-3-phosphate O- ... Carnitine-acylcarnitine translocase. *Carnitine palmitoyltransferase II. Beta oxidation. General. *Acyl CoA dehydrogenase ( ...

https://en.wikipedia.org/wiki/Fatty_acid_synthase

Glycerol-3-phosphate O-acyltransferase. *1-acylglycerol-3-phosphate O-acyltransferase. *2-acylglycerol-3-phosphate O- ...

https://en.wikipedia.org/wiki/Histone_acetyltransferase

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. "Compound Summary". p- ...

https://en.wikipedia.org/wiki/Methamphetamine

However, no formal assessment of the utility of carnitine and arginine supplementation has been published, and its uses have ... Neuwald AF (August 1997). "Barth syndrome may be due to an acyltransferase deficiency". Current Biology. 7 (8): R465-6. doi: ... Metabolic deficiencies have been treated by oral arginine and carnitine supplementation, which has been shown to ameliorate ... CL remodeling in mammals requires additional enzymes, such as monolysocardiolipin acyltransferase (MLCLAT), acyl-CoA: ...

https://en.wikipedia.org/wiki/Tafazzin

... : diacylglycerol acyltransferase (DGAT) plays an important role in energy metabolism on account of key enzyme in ... Transport of acyl-CoA into the mitochondria requires carnitine palmitoyltransferase 1 (CPT1), which converts acyl-CoA into ... Yu, Yi‐Hao; Ginsberg, Henry (8 July 2009). "The role of acyl‐CoA:diacylglycerol acyltransferase (DGAT) in energy metabolism". ...

https://en.wikipedia.org/wiki/Acyl-CoA

Lysophosphatidylcholine acyltransferase 1 LYSMD3: LysM and putative peptidoglycan-binding domain-containing protein 3 MAN2A1: ... carboxylase deficiency Myelodysplastic syndrome Netherton syndrome Nicotine dependency Parkinson's disease Primary carnitine ...

https://en.wikipedia.org/wiki/Chromosome_5

Acyl-carnitine is shuttled inside by a carnitine-acylcarnitine translocase, as a carnitine is shuttled outside. Acyl-carnitine ... Enzymes, acyltransferases and transacylases, incorporate fatty acids in phospholipids, triacylglycerols, etc. by transferring ... Acyl-CoA is transferred to the hydroxyl group of carnitine by carnitine palmitoyltransferase I, located on the cytosolic faces ... The liberated carnitine is shuttled back to the cytosol, as an acyl-CoA is shuttled into the mitochondrial matrix. Beta ...

https://en.wikipedia.org/wiki/Fatty_acid_metabolism

Carnitine dehydrogenase. *D-malate dehydrogenase (decarboxylating). *DXP reductoisomerase. *Glucose-6-phosphate dehydrogenase ... Acetyl-CoA C-acyltransferase. Unsaturated. *Enoyl CoA isomerase. *2,4 Dienoyl-CoA reductase ...

https://en.wikipedia.org/wiki/Beta-Ketoacyl_ACP_reductase

carnitine biosynthetic process. • medium-chain fatty acid catabolic process. • carnitine metabolic process, CoA-linked. • fatty ... Acetyl-CoA C-acyltransferase. Unsaturated. *Enoyl CoA isomerase. *2,4 Dienoyl-CoA reductase ...

https://en.wikipedia.org/wiki/ACADM

Category:EC 2.3 (acyltransferases)Edit. *Category:EC 2.3.1 *Aminolevulinic acid synthase EC 2.3.1.37 ... EC 6.2.1.48: Carnitine--CoA ligase. *EC 6.2.1.49: Long-chain fatty acid adenylyltransferase FadD28 ...

https://en.m.wikipedia.org/wiki/List_of_enzymes

"glycine N-acyltransferase. BRENDA. Technische Universität Braunschweig.. *^ "Compound Summary". p-Hydroxyamphetamine. PubChem ... and SLC22A5 is a high-affinity carnitine transporter.[97][108] ... and glycine N-acyltransferase are the enzymes known to ...

https://en.m.wikipedia.org/wiki/Methamphetamine

Carnitine-acylcarnitine translocase. *Carnitine palmitoyltransferase II. Beta oxidation. General. *Acyl CoA dehydrogenase ( ... Acetyl-CoA C-acyltransferase. Unsaturated. *Enoyl CoA isomerase. *2,4 Dienoyl-CoA reductase ...

https://en.wikipedia.org/wiki/Enoyl-acyl_carrier_protein_reductase

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 Opinion on Drug Metabolism ...

https://en.wikipedia.org/wiki/Amphetamine

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 ...

http://www.biochemj.org/content/262/3/801

This enzyme, called carnitine acyltransferase, moves the activated fatty acid across the borders of the mitochondrion and into ... Carnitine Acyltransferase. Drs. Mary Campbell and Shawn Farrell, in their textbook "Biochemistry," explain that a further ...

https://www.livestrong.com/article/206612-enzymes-that-break-down-fat-cells/

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 ...

http://www.pnas.org/content/113/41/E6064

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 ...

https://link.springer.com/chapter/10.1007/978-1-4684-2832-2_11

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 ...

https://www.jci.org/articles/view/34260

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 ...

https://diabetes.diabetesjournals.org/content/51/suppl_3/S405

Carnitine Acyltransferases); EC 2.3.1.21 (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 2.3.1.16 (Hadhb protein, mouse); EC 2.3.1.16 ( ... A group of genes, namely CPT1A, CPT1B, CPT1C, CPT2, CRAT and CROT, encode for three carnitine acyltransferases that are ...

http://bases.bireme.br/cgi-bin/wxislind.exe/iah/online/?IsisScript=iah/iah.xis&nextAction=lnk&base=MEDLINE&lang=p&format=detailed.pft&indexSearch=EX&exprSearch=D08.811.913.050.350

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.. ...

http://bases.bireme.br/cgi-bin/wxislind.exe/iah/online/?IsisScript=iah/iah.xis&nextAction=lnk&base=MEDLINE&lang=p&format=detailed.pft&indexSearch=EX&exprSearch=D02.033.800.175

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. ...

https://warwick.ac.uk/fac/sci/med/staff/zammit/

https://en.wikipedia.org/wiki/Carnitine_O-acetyltransferase

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 ... van der Leij FR, Huijkman NC, Boomsma C, Kuipers JR, Bartelds B (2000). "Genomics of the human carnitine acyltransferase genes ... Carnitine palmitoyltransferase I is the first component and rate-limiting step of the carnitine palmitoyltransferase system, ...

https://en.wikipedia.org/wiki/Carnitine_palmitoyltransferase_I

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) ...

http://www.ebi.ac.uk/interpro/member-database/Pfam

... 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 ...

http://jeb.biologists.org/content/205/3/297.full

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 ...

http://www.uniprot.org/uniprot/Q53FV7

... 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 ...

https://www.jci.org/articles/view/34275

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, ( ...

https://www.frontiersin.org/articles/10.3389/fnins.2017.00037/full

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 ...

http://www.informatics.jax.org/marker/MGI:2446526

... acetyl-Coenzyme A acyltransferase 2; BCKDHA: branched-chain alpha-keto acid dehydrogenase; NDUFS: NADH-ubiquinone ... Carnitine palmitoyl-transferase; MPC: mitochondrial pyruvate carrier; PDC: Pyruvate dehydrogenase complex; ACAD: Acyl-CoA ...

https://www.intechopen.com/online-first/the-cellular-stress-response-interactome-and-extracellular-matrix-cross-talk-during-fibrosis-a-stres

Study Biochemistry flashcards from Tali M

https://www.brainscape.com/flashcards/biochemistry-35677/packs/150329?page=7

Study Set 16 Enzymes and Hormones flashcards from Languages 247365

https://www.brainscape.com/flashcards/set-16-enzymes-and-hormones-5314056/packs/7935138

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 ...

https://www.hindawi.com/journals/ecam/2013/540957/

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, ...

http://atvb.ahajournals.org/content/32/9/2068.long

Intraparticulate localization of peroxisomal carnitine acyltransferases in chick embryo liver. （1987） * 20 ...

https://ci.nii.ac.jp/author?q=Watanabe+Takafumi

Effect of etomoxiryl-CoA on different carnitine acyltransferases. Biochem Pharmacol 1992; 43: 353-61. ...

https://cancerres.aacrjournals.org/content/67/14/6745?ijkey=5d45447dc08257a83de1c1e3c7f8fef6da849037&keytype2=tf_ipsecsha

Effect of etomoxiryl-CoA on different carnitine acyltransferases. Biochem Pharmacol 1992; 43: 353-61. ...

http://cancerres.aacrjournals.org/content/67/14/6745

chol: cholesterol; FFAs: free fatty acids; CPT-1: carnitine-palmitoyl-transferase-1; HMG-CoA red: 3-hydroxymethyl-3-glutaryl- ... glycerol 3 phosphate acyl transferase; PPAR. : peroxisomes proliferator activated receptor ; ... carnitine-palmytoil-transferase-1; ACN: anthocyanins; OA: oleic acid; tBH: tert-butyl hydroperoxide; MTT: 3-(4,5-dimethyl-2- ... glycerol 3 phophate acyl transferase; PKC: protein kinase C; OMM: outer mitochondrial membrane; SOD: superoxide dismutase; MAPK ...

https://www.hindawi.com/journals/omcl/2013/145421/

Key words: Carnitine acyltransferases; Deficiency; Sudden infant death. View this abstract indexed in MEDLINE:. ... Carnitine-acylcarnitine translocase deficiency in three neonates presenting with rapid deterioration and cardiac arrest ... The clustering of three cases in 5 years suggests that carnitine-acylcarnitine translocase deficiency is not rare in our ... We report on three Chinese neonates with carnitine-acylcarnitine translocase deficiency. They presented within the first 48 ...

http://www.hkmj.org/abstracts/v13n1/66.htm

1. Carnitine O-acetyltransferase. General function:. Involved in acyltransferase activity. Specific function:. Carnitine ... 255120 (Carnitine palmitoyltransferase I deficiency). *212140 (Carnitine transporter defect; primary systemic carnitine ... carnitine is essential to life and carnitine supplements are valuable. carnitine therapy may also be useful in a wide variety ... 4. Peroxisomal carnitine O-octanoyltransferase. General function:. Involved in acyltransferase activity. Specific function:. ...

http://www.hmdb.ca/metabolites/HMDB00062

The CPT1A gene provides instructions for making an enzyme called carnitine palmitoyltransferase 1A, which is found in the liver ... Carnitine acyltransferases and their influence on CoA pools in health and disease. Mol Aspects Med. 2004 Oct-Dec;25(5-6):475-93 ... Carnitine palmitoyltransferase I deficiency. More than 20 mutations in the CPT1A gene have been found to cause carnitine ... Carnitine palmitoyltransferase 1A connects carnitine to long-chain fatty acids so they can cross the inner membrane of ...

https://medlineplus.gov/genetics/gene/cpt1a/

Carnitine acyltransferases catalyze the exchange of acyl groups between coenzyme A (CoA) and carnitine. They have important ... Carnitine acyltransferases catalyze the exchange of acyl groups between coenzyme A (CoA) and carnitine. They have important ... Carnitine acetyltransferase. A. 618. Mus musculus. Mutation(s): 1 Gene Names: Crat. EC: 2.3.1.137 (UniProt), 2.3.1.7 (UniProt) ... We have determined the crystal structure of the F565A mutant in a ternary complex with both the carnitine and CoA substrates at ...

https://www.rcsb.org/structure/1T7N

Carnitine in intermediary metabolism. (springer.com)
The metabolism of fatty acid esters of carnitine by mitochondria. (springer.com)
Carnitine is an essential factor in fatty acid metabolism in mammals. (hmdb.ca)
Inborn errors of carnitine metabolism can lead to brain deterioration like that of Reye's syndrome, gradually worsening muscle weakness, Duchenne-like muscular dystrophy and extreme muscle weakness with fat accumulation in muscles. (hmdb.ca)
In all these conditions and the inborn errors of carnitine metabolism, carnitine is essential to life and carnitine supplements are valuable. (hmdb.ca)
Tissues such as cardiac muscle and skeletal muscle require carnitine for normal fuel metabolism but cannot synthesize carnitine and are totally dependent on the transport of carnitine from other tissues. (dsm.com)
The role of carnitine in the transport of long-chain fatty acids across the inner mitochondrial membrane is known, but liver medium-chain fatty acid (MCFA) metabolism has been considered carnitine independent owing to MCFAs' passive diffusion through the inner mitochondrial membrane and intramitochondrial activation (Bremer, 1990). (dsm.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)
This chapter will review the data describing the mitochondrial enzyme, carnitine palmitoyltransferase (hexadecanoyl-CoA:carnitine O -hexadecanoyltransferase, EC 2.3.1.23), and its role in the transport and metabolism of long-chain fatty acids. (springer.com)
This gene encodes carnitine O-acetyltransferase, a member of the carnitine acyltransferase family and a key metabolic pathway enzyme which plays an important role in energy homeostasis and fat metabolism. (genecards.org)
Exercise Metabolism: Role Of Glycine Propionyl-L-Carnitine (GPLC)! (bodybuilding.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)
Apparently the extra carnitine allows more rapid transport of fatty acids and a more efficient energy metabolism. (cliffsnotes.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)

This enzyme, called carnitine acyltransferase, moves the activated fatty acid across the borders of the mitochondrion and into its interior. (livestrong.com)
Carnitine O-acetyltransferase also called carnitine acetyltransferase (CRAT, or CAT) (EC 2.3.1.7) is an enzyme that encoded by the CRAT gene that catalyzes the chemical reaction acetyl-CoA + carnitine ⇌ {\displaystyle \rightleftharpoons } CoA + acetylcarnitine where the acetyl group displaces the hydrogen atom in the central hydroxyl group of carnitine. (wikipedia.org)
Thus, the two substrates of this enzyme are acetyl-CoA and carnitine, whereas its two products are CoA and O-acetylcarnitine. (wikipedia.org)
This enzyme belongs to the family of transferases, to be specific those acyltransferases transferring groups other than aminoacyl groups. (wikipedia.org)
The systematic name of this enzyme class is acetyl-CoA:carnitine O-acetyltransferase. (wikipedia.org)
Upon binding, a face of carnitine is left exposed to the space outside the enzyme. (wikipedia.org)
Carnitine palmitoyltransferase I (CPT1) also known as carnitine acyltransferase I, CPTI, CAT1, CoA:carnitine acyl transferase (CCAT), or palmitoylCoA transferase I, is a mitochondrial enzyme responsible for the formation of acyl carnitines by catalyzing the transfer of the acyl group of a long-chain fatty acyl-CoA from coenzyme A to l-carnitine. (wikipedia.org)
The CPT1A gene provides instructions for making an enzyme called carnitine palmitoyltransferase 1A, which is found in the liver. (medlineplus.gov)
Without enough of this enzyme, carnitine is not attached to long-chain fatty acids. (medlineplus.gov)
Carnitine acyltransferase is the enzyme responsible for this shuttle mechanism. (dsm.com)
After the long-chain fatty acid is activated to acyl-CoA, it is converted to acylcarnitine by the enzyme carnitine acyltransferase I and crosses to the matrix side of the inner mitochondrial membrane. (dsm.com)
The ER/peroxisome reaction pathway uses the peroxisomal enzyme DHAP acyltransferase to acylate DHAP to acyl-DHAP which is then reduced by acyl-DHAP reductase. (hmdb.ca)
The CPT2 gene provides instructions for making an enzyme called carnitine palmitoyltransferase 2. (medlineplus.gov)
This enzyme catalyzes the reversible transfer of acyl groups from an acyl-CoA thioester to carnitine and regulates the ratio of acyl-CoA/CoA. (genecards.org)
In mammalian cells, cholesterol esterification is catalyzed by the enzyme acyl CoA:cholesterol acyltransferase (E.C. 2.3.1.26, ACAT). (google.com)
The acyl‐ carnitine then moves across the intermembrane space to a translocase enzyme, which, in turn, moves the acyl‐carnitine to carnitine acyltransferase II, which exchanges the carnitine for Coenzyme A. (cliffsnotes.com)
A key enzyme involved in fatty acid oxidation is carnitine palmitoyltransferase-1, which is located on the outer mitochondrial membrane, and acts as the rate-limiting step for mitochondrial fatty acid uptake (1) . (onlinejacc.org)
2008). Cloning and characterization of an acyl-CoA-dependent diacylglycerol acyltransferase 1 (DGAT1) gene from Tropaeolum majus, and a study of the functional motifs of the DGAT protein using site-directed mutagenesis to modify enzyme activity and oil content. (creative-biostructure.com)

Two major isoforms of diacylglycerol acyltransferase (DGAT) catalyze the third esterification to form triacylglycerol (TAG). (nih.gov)
Adipocyte cell sizes, TAG content and expression of lipogenic-related genes including, adipose differentiated related protein (ADRP) and diacylglycerol acyltransferase 1 (DGAT1) were elevated by DHA in vivo and in vitro, indicating DHA promoted adipogenesis to trap TAG in adipose tissue. (biomedcentral.com)

Thus the overt carnitine acyltransferase activities in peroxisomes and mitochondria have the same properties. (biochemj.org)
Reversible acetylation 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)
This "preparation" allows for subsequent movement of the acyl carnitine from the cytosol into the intermembrane space of mitochondria. (wikipedia.org)
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)
Carnitine is required for transport of long-chain fatty acids into the matrix compartment of mitochondria from cytoplasm for subsequent oxidation by the fatty acid oxidase complex for energy production. (dsm.com)
Carnitine facilitates the beta-oxidation of long-chain fatty acids in the mitochondria by transporting the substrate into the mitochondria. (dsm.com)
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)
Briefly, L-carnitine is needed to transport long-chain FA from the cytosol to sites of beta-oxidation in the mitochondria. (plos.org)
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)
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)
Gerbling, H., Gerhardt, B. (1988) Carnitine acyltransferase activity of mitochondria from mung bean hypocotyls. (springer.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)

Other names in common use include acetyl-CoA-carnitine O-acetyltransferase, acetylcarnitine transferase, carnitine acetyl coenzyme A transferase, carnitine acetylase, carnitine acetyltransferase, carnitine-acetyl-CoA transferase, and CATC. (wikipedia.org)
The exact structure of any of the CPT1 isoforms has not yet been determined, although a variety of in silico models for CPT1 have been created based on closely related carnitine acyltransferases, such as carnitine acetyltransferase (CRAT). (wikipedia.org)
One such mechanism based upon a carnitine acetyltransferase model is shown below in which the His 473 deprotonates carnitine while a nearby serine residue stabilizes the tetrahedral oxyanion intermediate. (wikipedia.org)
Structural information on carnitine acetyltransferase suggests that residues Met-564 and Phe-565 may be important determinants of substrate selectivity with the side chain of Met-564 located in the putative binding pocket for acyl groups. (rcsb.org)
Intestinal peroxisomes of goldfish (Carrassius auratus) - examination for hydrolase, dehydrogenase and carnitine acetyltransferase activities. (athabascau.ca)
CRAT (Carnitine O-Acetyltransferase) is a Protein Coding gene. (genecards.org)
Gene Ontology (GO) annotations related to this gene include signaling receptor binding and carnitine O-acetyltransferase activity . (genecards.org)
EC 2.3.1.7 carnitine O-acetyltransferase and EC 2.3.1.137 carnitine O-octanoyltransferase. (genome.jp)

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)
A primary biochemical action of malonyl-CoA is the inhibition of the acyltransferase activity of carnitine palmitoyltransferase-1 (CPT-1). (physiology.org)
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)

carnitine supplementation has improved some patients who have angina secondary to coronary artery disease. (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)

The carnitine palmitoyltransferase system is an essential step in the beta-oxidation of long chain fatty acids. (wikipedia.org)
The mainstay of initial treatment is glucose infusion at a rate greater than 7 mg/kg/minute, which inhibits beta-oxidation of fatty acids (the defective enzymatic steps in carnitine-acylcarnitine translocase deficiency) and thus prevents the accumulation of toxic long-chain acylcarnitines. (hkmj.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)
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)
These supplements shift tissue partitioning of carnitine that favors fat mobilization, incomplete oxidation of fatty acids and disposal of their carbons in urine. (dsm.com)
The oxidation of long-chain fatty acids in animal tissues is dependent on carnitine because it allows long-chain acyl-CoA esters to cross the mitochondrial membrane, which is otherwise impermeable to CoA compounds. (dsm.com)
Carnitine is an essential factor in long-chain fatty acid oxidation. (nih.gov)
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)
Carnitine performs an obligate role in mitochondrial oxidation of long-chain fatty acids (FA) through the action of specialized acyltransferases [4] . (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 [5] . (plos.org)
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)
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)

and diacylglycerol O-acyltransferase 1 (DGAT1), PPARgamma, and hormone-sensitive lipase (HSL) for triglyceride synthesis and catalysis. (unboundmedicine.com)
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)

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)
The Effect of Carnitine on the Rate of Palmitate Incoporation into Mitochondrial Phospholipids. (semanticscholar.org)

These channels are utilized by the substrates of CRAT, one channel for carnitine, and one for CoA. (wikipedia.org)
Carnitine binds to CRAT in a partially folded state, with its hydroxyl group and carboxyl group facing opposite directions. (wikipedia.org)
This CRAT catalysis is stereospecific for carnitine, as the stereoisomer of the 3-hydroxyl group cannot sufficiently interact with the CRAT carnitine binding site. (wikipedia.org)
CRAT undergoes minor conformational changes upon binding with carnitine. (wikipedia.org)
The His343 residue at the active site of CRAT acts as a base that is able to deprotonate the CoA thiol group or the Carnitine 3'-hydroxyl group depending on the direction of the reaction. (wikipedia.org)
The literature suggests that the trimethylammonium group on carnitine may be a crucial factor in CRAT catalysis. (wikipedia.org)
An important structural difference between CPT1 and CPT2, CRAT and carnitine octanoyltransferase (COT) is that CPT1 contains an additional domain at its N-terminal consisting of about 160 amino acids. (wikipedia.org)

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)

We advocate that investigation for metabolic diseases including carnitine-acylcarnitine translocase deficiency should be performed in cases of sudden infant death and unexplained abrupt clinical deterioration in the early neonatal period. (hkmj.org)
carnitine supplements may be useful in many forms of toxic or metabolic liver disease and in cases of heart muscle disease. (hmdb.ca)
Recent evidence suggests that a low carnitine availability may limit acetylcarnitine formation, thereby reducing metabolic flexibility. (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)
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)
Carnitine is used as a dietary supplement by endurance athletes and in the treatment of certain metabolic diseases. (cliffsnotes.com)
Treatment with L-carnitine, or HMF extract improved obesity and its associated metabolic problems in different degrees. (biomedcentral.com)

We report on three Chinese neonates with carnitine-acylcarnitine translocase deficiency. (hkmj.org)
The clustering of three cases in 5 years suggests that carnitine-acylcarnitine translocase deficiency is not rare in our Chinese population. (hkmj.org)
carnitine deficiency is noted in abnormal liver function, renal dialysis patients, and severe to moderate muscular weakness with associated anorexia. (hmdb.ca)
More than 20 mutations in the CPT1A gene have been found to cause carnitine palmitoyltransferase I (CPT I) deficiency. (medlineplus.gov)
Bennett MJ, Boriack RL, Narayan S, Rutledge SL, Raff ML. Novel mutations in CPT 1A define molecular heterogeneity of hepatic carnitine palmitoyltransferase I deficiency. (medlineplus.gov)
Deficiency in any cofactor will cause L-carnitine deficiency. (dsm.com)
1994). It has been suggested that early features of scurvy (fatigue and weakness) may be attributed to carnitine deficiency. (dsm.com)
Clinical features and new molecular findings in Carnitine Palmitoyltransferase II (CPT II) deficiency. (medlineplus.gov)
Deschauer M, Wieser T, Zierz S. Muscle carnitine palmitoyltransferase II deficiency: clinical and molecular genetic features and diagnostic aspects. (medlineplus.gov)
CPT2 gene mutations resulting in lethal neonatal or severe infantile carnitine palmitoyltransferase II deficiency. (medlineplus.gov)
Mutation and biochemical analysis in carnitine palmitoyltransferase type II (CPT II) deficiency. (medlineplus.gov)
Carnitine palmitoyltransferase II deficiency: a clinical, biochemical, and molecular review. (medlineplus.gov)

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)

Glycerol-3-phosphate acyltransferases: rate limiting enzymes of triacylglycerol biosynthesis. (nih.gov)
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)

It is part of a family of enzymes called carnitine acyltransferases. (wikipedia.org)

Vitamin C is a cofactor for two alpha-ketoglutarate-requiring dioxygenase reactions (epsilon-N-trimethyllysine hydroxylase and gamma-butyrobetaine hydroxylase) in the pathway of carnitine biosynthesis. (dsm.com)
The biosynthesis of L-carnitine in eukaryotic organisms was first elucidated in the ascomycete Neurospora crassa. (sun.ac.za)

Intraparticulate localization of peroxisomal carnitine acyltransferases in chick embryo liver. (nii.ac.jp)

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)

liver carnitine is correlated with the rate of hepatic ketoacid production. (nih.gov)
whereas administration of L-carnitine or herbal extract significantly ameliorated these hepatic alterations. (biomedcentral.com)

Where GPLC may play an important role is step number 5, in which carnitine is essential for the actual transport of fatty acids inside the mitochondrial matrix in order to be oxidized. (bodybuilding.com)

Carnitine (earlier known as vitamin Bt) is a quaternary amine, beta-hydroxy-gamma-trimethylaminobutyrate. (dsm.com)
Naturally occurring, l -carnitine is a quaternary amine (3-hydroxy-4- N -trimethylaminobutyrate) found in all mammalian species. (mdpi.com)
Carnitine is a quaternary amine compound prevalent in animal tissues, and a potential carbon, nitrogen and energy source for pathogens during infection. (microbiologyresearch.org)

In the hypothalamus, the predominant isoform of CPT-1 that possesses the acyltransferase activity is CPT-1 liver type (CPT-1a). (physiology.org)

Zonal rotor study of the subcellular distribution of acyl-CoA synthetases, carnitine acyl transferases and phosphatidate phosphatase in the guinea-pig small intestine. (athabascau.ca)

Citrate synthase, carnitine palmitoyl transferase and β-hydroxyacyl Co-A dehydrogenase activities in the pectoralis were generally higher for LD and cold groups. (biologists.org)
The PA5388 - PA5384 region contains the predicted P. aeruginosa carnitine dehydrogenase homologue along with other genes required for growth on carnitine. (microbiologyresearch.org)

In heart muscle, malonyl CoA is a very potent inhibitor of carnitine palmitoyltransferase-1 (3) . (onlinejacc.org)

Thus, utilization of long-chain fatty acids as a fuel source depends on adequate concentrations of carnitine. (dsm.com)

The uptake of carnitine from the intestinal lumen into the mucosa is rapid, and about one-half of the carnitine taken up is acetylated in that tissue. (dsm.com)

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)

In this article, we describe recent preclinical studies suggesting that a gene usually expressed only in the brain, carnitine palmitoyltransferase (CPT)1C, promotes cancer cell survival and tumor growth. (aacrjournals.org)

Binienda Z, Virmani A. The Mitochondriotropic effects of L-carnitine and its esters in the central nervous system. (springermedizin.de)

The binding of malonyl-CoA to either the A and O sites inhibits the action of CPT1A by excluding the binding of carnitine to CPT1A. (wikipedia.org)

It is a very hygroscopic compound and is found in biological samples both as free carnitine and as the ester of a wide variety of acyl compounds. (dsm.com)
Free carnitine is excreted in urine, with the principal excretory product being trimethylamine oxide (Mitchell, 1978). (dsm.com)
Enzymological Determination of Free Carnitine Concentrations in Rat Tissues. (semanticscholar.org)

Two-dimensional Thin-layer Chromatographic Isolation of Fatty Acyl Carnitines. (semanticscholar.org)

But like choline, which is sometimes considered to be a B vitamin, carnitine is also an alcohol (specifically, a trimethylated carboxy-alcohol). (hmdb.ca)
Choline has also been shown to affect carnitine homeostasis in humans and guinea pigs (Daily and Sachan, 1995). (dsm.com)
choline resulted in the conservation of carnitine in guinea pigs. (dsm.com)
In adult women Hongu and Sachan (2003) concluded that the choline-induced decrease in serum and urinary carnitine is buffered by carnitine preloading. (dsm.com)
Most notably, CoA-dependent acyltransferase is a superfamily which consists of three families: CAT-like family, Choline/Carnitine O-acyltransferase family and NRPS condensation domain family. (creative-biostructure.com)

In this work, we present the structure of the bacterial LPLAT protein LpxM, and we describe a high-throughput, label-free mass spectrometric assay to characterize acyltransferase enzymatic activity. (pnas.org)
Post-translational modification by acetylation regulates the mitochondrial carnitine/acylcarnitine transport protein. (bireme.br)
Thus, carnitine is an unusual amino acid and has different functions than most other amino acids, which are most usually employed by the body in the construction of protein. (hmdb.ca)
Most of these mutations change single protein building blocks (amino acids) within carnitine palmitoyltransferase 1A. (medlineplus.gov)
Based on current animal studies, a role of l -carnitine in the prevention of age-associated muscle protein degradation and regulation of mitochondrial homeostasis is suggested. (mdpi.com)
Creative Biostructure can provide high-yield and high-quality CoA-dependent acyltransferases by our best Mempro™ cell-free protein production services. (creative-biostructure.com)
Mempro™ membrane protein expression by bacterial extract provides you the enhancement of expression of the CoA-dependent acyltransferases, and easy to perform scale-up membrane protein production. (creative-biostructure.com)

carnitine may improve muscle building by improving fat utilization and may even be useful in treating obesity. (hmdb.ca)

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)

Carnitine is highly conserved by the human kidney, which reabsorbs more than 90% of filtered carnitine, thus, playing an important role in the regulation of carnitine concentration in blood. (dsm.com)

We have determined the crystal structure of the F565A mutant in a ternary complex with both the carnitine and CoA substrates at a 1.8-A resolution. (rcsb.org)
CoA-dependent acyltransferase, is also termed glycerophospholipid acyltransferase, the substrates of which are 1-organyl-2-acyl-sn-glycero-3-phosphocholine and 1-organyl-2-lyso-sn-glycero-3-phosphoethanolamine, whereas its two final products are 1-organyl-2-acyl-sn-glycero-3-phosphoethanolamine and 1-organyl-2-lyso-sn-glycero-3-phosphocholine. (creative-biostructure.com)

Carnitine concentrations are variably low in some tissues of vitamin C-deficient guinea pigs (Rebouche, 1991). (dsm.com)

Characterization of activities in Pseudomonas aeruginosa cell lysates has previously shown that carnitine is converted to 3-dehydrocarnitine (3-dhc) which is in turn metabolized to glycine betaine (GB), an intermediate metabolite in the catabolism of carnitine to glycine. (microbiologyresearch.org)

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Submitochondrial and subcellular distributions of the carnitine-acylcarnitine carrier. (1/96)

Molecular characterization of carnitine-dependent transport of acetyl-CoA from peroxisomes to mitochondria in Saccharomyces cerevisiae and identification of a plasma membrane carnitine transporter, Agp2p. (2/96)

Arrhythmias and conduction defects as presenting symptoms of fatty acid oxidation disorders in children. (3/96)

Evidence for triacylglycerol synthesis in the lumen of microsomes via a lipolysis-esterification pathway involving carnitine acyltransferases. (4/96)

Identification of the two histidine residues responsible for the inhibition by malonyl-CoA in peroxisomal carnitine octanoyltransferase from rat liver. (5/96)

Identification and functions of new transporters in yeast mitochondria. (6/96)

Inhibition by etomoxir of rat liver carnitine octanoyltransferase is produced through the co-ordinate interaction with two histidine residues. (7/96)

Molecular enzymology of carnitine transfer and transport. (8/96)

Carnitine Acyltransferases

Carnitine O-Acetyltransferase

Carnitine

Acyltransferases

Carnitine O-Palmitoyltransferase

1-Acylglycerophosphocholine O-Acyltransferase

Glycerol-3-Phosphate O-Acyltransferase

Acyl Coenzyme A

Diacylglycerol O-Acyltransferase

Acylation

Malonyl Coenzyme A

Peroxisomes

Microbodies

Fats

Dietary Fats

Adipose Tissue

Down Syndrome

DNA Breaks, Double-Stranded

Lipid Mobilization

Lipolysis

Methylamines

Biology

Salmoniformes

Decapodiformes

Animal Migration

Copepoda

Body Size

Laryngostenosis

Scleroderma, Systemic

Tracheal Stenosis

Exosomes

Fibrosis

Extracellular Matrix

Stress, Physiological

Yang Deficiency

Access to Information

Metabolome

Metabolomics

Journal Impact Factor

History, Ancient

Complementary Therapies

Insulin Resistance

Neurodermatitis

Glucose

Insulin

Fatty Acids, Nonesterified

Lipid Metabolism

Heart

Ethylenediamines

Hypolipidemic Agents

Chelating Agents

Oxidation-Reduction

Hyperlipidemias

Zinc

Submitochondrial and subcellular distributions of the carnitine-acylcarnitine carrier. (1/96)

Molecular characterization of carnitine-dependent transport of acetyl-CoA from peroxisomes to mitochondria in Saccharomyces cerevisiae and identification of a plasma membrane carnitine transporter, Agp2p. (2/96)

Arrhythmias and conduction defects as presenting symptoms of fatty acid oxidation disorders in children. (3/96)

Evidence for triacylglycerol synthesis in the lumen of microsomes via a lipolysis-esterification pathway involving carnitine acyltransferases. (4/96)

Identification of the two histidine residues responsible for the inhibition by malonyl-CoA in peroxisomal carnitine octanoyltransferase from rat liver. (5/96)

Identification and functions of new transporters in yeast mitochondria. (6/96)

Inhibition by etomoxir of rat liver carnitine octanoyltransferase is produced through the co-ordinate interaction with two histidine residues. (7/96)

Molecular enzymology of carnitine transfer and transport. (8/96)

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