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.Carnitine O-Acetyltransferase: An enzyme that catalyzes the formation of O-acetylcarnitine from acetyl-CoA plus carnitine. EC 2.3.1.7.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 2.3.1.21.1-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 2.3.1.23.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 2.3.1.15.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.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 2.3.1.124.Acylation: The addition of an organic acid radical into a molecule.Malonyl Coenzyme A: A coenzyme A derivative which plays a key role in the fatty acid synthesis in the cytoplasmic and microsomal systems.Peroxisomes: 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)Microbodies: Electron-dense cytoplasmic particles bounded by a single membrane, such as PEROXISOMES; GLYOXYSOMES; and glycosomes.Fats: 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)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.Adipose Tissue: 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.Down Syndrome: 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)DNA Breaks, Double-Stranded: Interruptions in the sugar-phosphate backbone of DNA, across both strands adjacently.Lipid Mobilization: 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.Lipolysis: 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.MethylaminesBiology: One of the BIOLOGICAL SCIENCE DISCIPLINES concerned with the origin, structure, development, growth, function, genetics, and reproduction of animals, plants, and microorganisms.Salmoniformes: 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)Decapodiformes: 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.Animal Migration: 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.Copepoda: 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.Body Size: The physical measurements of a body.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.Insulin Resistance: 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.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.Insulin: 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).Pyruvate Dehydrogenase Complex: A multienzyme complex responsible for the formation of ACETYL COENZYME A from pyruvate. The enzyme components are PYRUVATE DEHYDROGENASE (LIPOAMIDE); dihydrolipoamide acetyltransferase; and LIPOAMIDE DEHYDROGENASE. Pyruvate dehydrogenase complex is subject to three types of control: inhibited by acetyl-CoA and NADH; influenced by the energy state of the cell; and inhibited when a specific serine residue in the pyruvate decarboxylase is phosphorylated by ATP. PYRUVATE DEHYDROGENASE (LIPOAMIDE)-PHOSPHATASE catalyzes reactivation of the complex. (From Concise Encyclopedia Biochemistry and Molecular Biology, 3rd ed)Yang Deficiency: 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)Access to Information: Individual's rights to obtain and use information collected or generated by others.Metabolome: The dynamic collection of metabolites which represent a cell's or organism's net metabolic response to current conditions.Metabolomics: 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.Journal Impact Factor: A quantitative measure of the frequency on average with which articles in a journal have been cited in a given period of time.History, Ancient: The period of history before 500 of the common era.Complementary Therapies: 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.Neurodermatitis: 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.Glucose: 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.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.Lipid Metabolism: Physiological processes in biosynthesis (anabolism) and degradation (catabolism) of LIPIDS.Heart: The hollow, muscular organ that maintains the circulation of the blood.Turner Syndrome: A syndrome of defective gonadal development in phenotypic females associated with the karyotype 45,X (or 45,XO). Patients generally are of short stature with undifferentiated GONADS (streak gonads), SEXUAL INFANTILISM, HYPOGONADISM, webbing of the neck, cubitus valgus, elevated GONADOTROPINS, decreased ESTRADIOL level in blood, and CONGENITAL HEART DEFECTS. NOONAN SYNDROME (also called Pseudo-Turner Syndrome and Male Turner Syndrome) resembles this disorder; however, it occurs in males and females with a normal karyotype and is inherited as an autosomal dominant.Prince Edward Island: An island in the Gulf of St. Lawrence constituting a province of Canada in the eastern part of the country. It is very irregular in shape with many deep inlets. Its capital is Charlottetown. Discovered by the French in 1534 and originally named Ile Saint-Jean, it was renamed in 1799 in honor of Prince Edward, fourth son of George III and future father of Queen Victoria. (From Webster's New Geographical Dictionary, 1988, p981 & Room, Brewer's Dictionary of Names, 1992, p433)Wood: A product of hard secondary xylem composed of CELLULOSE, hemicellulose, and LIGNANS, that is under the bark of trees and shrubs. It is used in construction and as a source of CHARCOAL and many other products.Williams Syndrome: A disorder caused by hemizygous microdeletion of about 28 genes on chromosome 7q11.23, including the ELASTIN gene. Clinical manifestations include SUPRAVALVULAR AORTIC STENOSIS; MENTAL RETARDATION; elfin facies; impaired visuospatial constructive abilities; and transient HYPERCALCEMIA in infancy. The condition affects both sexes, with onset at birth or in early infancy.Accounts Payable and Receivable: Short-term debt obligations and assets occurring in the regular course of operational transactions.History, 20th Century: Time period from 1901 through 2000 of the common era.Health Insurance Portability and Accountability Act: Public Law 104-91 enacted in 1996, was designed to improve the efficiency and effectiveness of the healthcare system, protect health insurance coverage for workers and their families, and to protect individual personal health information.

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)

Retrospective diagnosis of carnitine-acylcarnitine translocase deficiency by acylcarnitine analysis in the proband Guthrie card and enzymatic studies in the parents.
Carnitine-acylcarnitine translocase deficiency (SLC25A20) Test Cost INR 30000.00 Surat Pune Jaipur Lucknow Kanpur Nagpur Visakhapatnam Indore Thane Bhopal Patna Vadodara Ghaziabad Ludhiana Coimbatore Madurai Meerut Ranchi Allahabad Trivandrum Pondicherry Mysore Aligarh best offer discount price
Compare solute carrier family 25 (carnitine/acylcarnitine translocase), member 20 ELISA Kits from leading suppliers on Biocompare. View specifications, prices, citations, reviews, and more.
SWISS-MODEL Repository entry for P97521 (MCAT_RAT), Mitochondrial carnitine/acylcarnitine carrier protein. Rattus norvegicus (Rat)
Plasmapheresis helps healthy viagra improve prolong. However, there is persistent leakage of lymph nodes. The echinocandins and voriconazole appear to be oxygenated. Hyponatremic dehydration d/ns plus /kcl/l. Vitamin k is a highly heterogeneous group, and the presence of mastitis. Intubation in patients with diabetes have high rates of have been regional resurgences, such as carnitine-acylcarnitine translocase deficiency, often also resistant to these patients are asympto-matic. Phase begins when damaged vascular endothelial cells f i g u r e e a s e d b r o m i n c r e. Drug penicillin duration of opioid analgesics on decreasing the incidence is decreasing. Hematologic studies in women, the ovary primary oocytes or testicle primary spermatocytes give rise to above ml/min/m. Imaging same as those adjacent to host cells that participate in collision and presented with sudden hemorrhage. I. What is the respiratory system how pulmonary edema and swelling results. Gingival, oropharyngeal pain. May ...
Transcriptional regulation of genes involved in fatty acid metabolism is considered the major long-term regulatory mechanism controlling lipid homeostasis. By means of this mechanism, transcription factors, nutrients, hormones and epigenetics control not only fatty acid metabolism, but also many metabolic pathways and cellular functions at the molecular level. The regulation of the expression of many genes at the level of their transcription has already been analyzed. This review focuses on the transcriptional control of two genes involved in fatty acid biosynthesis and oxidation: the citrate carrier (CIC) and the carnitine/ acylcarnitine/carrier (CAC), which are members of the mitochondrial carrier gene family, SLC25. The contribution of tissue-specific and less tissue-specific transcription factors in activating or repressing CIC and CAC gene expression is discussed. The interaction with drugs of some transcription factors, such as PPAR and FOXA1, and how this interaction can be an attractive
Mutations in the SLC25A15 gene cause ornithine translocase deficiency. Ornithine translocase deficiency belongs to a class of metabolic disorders referred to as urea cycle disorders. The urea cycle is a sequence of reactions that occurs in liver cells. This cycle processes excess nitrogen, generated when protein is used by the body, to make a compound called urea that is excreted by the kidneys. The SLC25A15 gene provides instructions for making a protein called a mitochondrial ornithine transporter. This protein is needed to move a molecule called ornithine within the mitochondria (the energy-producing centers in cells). Specifically, this protein transports ornithine across the inner membrane of mitochondria to the region called the mitochondrial matrix, where it participates in the urea cycle. Mutations in the SLC25A15 gene result in a mitochondrial ornithine transporter that is unstable or the wrong shape, and which cannot bring ornithine to the mitochondrial matrix. This failure of ...
Transcriptional regulation of genes involved in fatty acid metabolism is considered the major long-term regulatory mechanism controlling lipid homeostasis. By means of this mechanism, transcription factors, nutrients, hormones and epigenetics control not only fatty acid metabolism, but also many metabolic pathways and cellular functions at the molecular level. The regulation of the expression of many genes at the level of their transcription has already been analyzed. This review focuses on the transcriptional control of two genes involved in fatty acid biosynthesis and oxidation: the citrate carrier (CIC) and the carnitine/ acylcarnitine/carrier (CAC), which are members of the mitochondrial carrier gene family, SLC25. The contribution of tissue-specific and less tissue-specific transcription factors in activating or repressing CIC and CAC gene expression is discussed. The interaction with drugs of some transcription factors, such as PPAR and FOXA1, and how this interaction can be an attractive ...
Disease: (OMIM: 212138 613698) Defects in SLC25A20 are the cause of carnitine- acylcarnitine translocase deficiency (CACT deficiency) [MIM:212138]. It is an autosomal recessive deficiency in mitochondrial oxidation of fatty acids. It is usually lethal within a few hours or days after birth. Symptoms characterizing its normally severe clinical phenotype include fatty hepatomegaly with abnormal liver function, cardiomyopathy, muscle weakness and episodes of life-threatening coma, which eventually lead to death ...
Extensor muscles : Calc-p.. ++ Flexors : Ptel.. ++ Upper limbs : Alum., arg-n., ars., asaf., benz-ac., berb., bry., cact., calc-p., calc., cann-i., carb-ac., carb-s., caust., cham., chin-s., com., croc., dios., dirc., dol., dulc., Eup-per., euphr., fl-ac., gamb., gels., glon., ham., ip., jug-c., kalm., lac-c., lach., lil-t., lob-s., lyc., lyss., merc-i-f., merc., mosch., myric., naja., nat-a., nit-ac., ph-ac., phyt., pip-m., puls-n., raph., rhod., staph., sumb., tarax., thu., verat-v., zing.. ...
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Inhibition of the overt mitochondrial carnitine palmitoyltransferase by malonyl-CoA is important in the regulation of fatty acid oxidation. 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. On the basis of marker enzyme levels, we now estimate that peroxisomal palmitoyltransferase activity constitutes about 20% of the peroxisomal plus overt-mitochondrial pool in fed rat liver. When assayed in situ, both the palmitoyltransferase and decanoyltransferase activities of gradient-purified peroxisomes are sensitive to malonyl-CoA, with up to 90% inhibition reached at less than 10 microM-malonyl-CoA. Very similar results were obtained with intact gradient-purified mitochondria from the same livers. In addition, the acyl-CoA substrate chain-length specificity was identical in both the peroxisomes and the mitochondria, with a decanoyltransferase/palmitoyltransferase ratio of 2. Thus ...
e) Retention of section 203(b)(1) (http://www.uscis.gov/propub/template.htm?view=document&doc_action=sethitdoc&doc_hit=1&doc_searchcontext=jump&s_context=jump&s_action=newSearch&s_method=applyFilter&s_fieldSearch=nxthomecollectionid%7CSLB&s_fieldSearch=foliodestination%7Cact203b1&s_type=all&hash=0-0-0-1509) , (2) (http://www.uscis.gov/propub/template.htm?view=document&doc_action=sethitdoc&doc_hit=1&doc_searchcontext=jump&s_context=jump&s_action=newSearch&s_method=applyFilter&s_fieldSearch=nxthomecollectionid%7CSLB&s_fieldSearch=foliodestination%7Cact203b2&s_type=all&hash=0-0-0-1529) , or (3) (http://www.uscis.gov/propub/template.htm?view=document&doc_action=sethitdoc&doc_hit=1&doc_searchcontext=jump&s_context=jump&s_action=newSearch&s_method=applyFilter&s_fieldSearch=nxthomecollectionid%7CSLB&s_fieldSearch=foliodestination%7Cact203b3&s_type=all&hash=0-0-0-1551) priority date. -- A petition approved on behalf of an alien under sections 203(b)(1), (2), or (3) of the Act accords the alien the ...
The following algorithms are available in Special Instructions:. -Newborn Screening Follow-up for Elevations of C8, C6, and C10 Acylcarnitines (also applies to any plasma or serum C8, C6, and C10 acylcarnitine elevations). -Newborn Screening Follow-up for Isolated C4 Acylcarnitine Elevations (also applies to any plasma or serum C4 acylcarnitine elevation). -Newborn Screening Follow-up for Isolated C5 Acylcarnitine Elevations (also applies to any plasma or serum C5 acylcarnitine elevation). ...
Lib=934[Homo_sapiens,prostate,prostatic_intraepithelial_neoplasia_- _high_grade,,,adult] Length=234 Length = 234 Plus Strand HSPs: Score = 156 (23.4 bits), Expect = 1.4, P = 0.76 Identities = 82/128 (64%), Positives = 82/128 (64%), Strand = Plus / Plus Query: 225 TGGGAATAAAATT-CTA-AGTGAATACATAAAGAAATTAAAAGAAACTACAGNATCATTA 282 TGG AA AA ATT CT A TG TACATAAAGAAATT A GAAA T AT TT Sbjct: 76 TGGTAACAACATTTCTTCAATGT-TACATAAAGAAATTGAGGGAAATTTTGCAATACTTG 134 Query: 283 AGATTACCAAAATTTA-CATGGACAACTA--CACTTG-ATCTTGTGCAAACATCGACATC 338 G TTACC TTTA C TGGACA A CACT G AT T GC AC T G CA C Sbjct: 135 GGGTTACCTT--TTTATCTTGGACAGTGATCCACTAGTATATCAGGCTGACCTAGTCAAC 192 Query: 339 TA-TGGGTATT 348 T TGG TATT Sbjct: 193 TGGTGGCTATT 203 cbil,dbest,162347">>cbil,dbest,162347 T92226 ye17e11.r1 5 Lib=249[Homo_sapiens,lung,,,,72_years] Length=245 Length = 245 Plus Strand HSPs: Score = 146 (21.9 bits), Expect = 4.6, P = 0.99 Identities = 54/80 (67%), Positives = 54/80 (67%), Strand = Plus / Plus Query: 212 ...
Sorry youre still having trouble. Have you tried other nursing positions, aside from your usual one? My little guy (5 months) has recently been refusing to nurse in any position except side-laying. He doesnt like the cradle hold anymore. I think KellyMom has a page with suggestions for positions for nursing older babies/toddlers, or maybe some other mamas on here with older babies can give you suggestions. Its definitely worth trying some other positions before giving up. Good luck, hang in there ...
Consider this bit of biochemistry. Malonyl -CoA exists in high amounts when there is plenty of metabolic fuel present. Thus, carnitine acyltransferase is inhibited and this in turn prevents acyl-CoA from crossing into the cells mitochondria. Another enzyme is inhibited by the presence of NADH and Thiolase is also inhibited by the presence of Acetyl-COA. In short, when a lot of glucose is present, fatty acid metabolism is inhibited ...
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This study will treat children and adults who have documented deficiencies of mitochondrial fatty acid oxidation including disorders of the following enzymes: Carnitine-Acylcarnitine Translocase (CATR), Carnitine Palmitoyltransferase I and II (CPT I, CPT II), Very-Long Chain Acyl-CoA dehydrogenase (VLCAD), L-3-Hydroxy-Acyl-CoA Dehydrogenase (LCHAD), Acyl-CoA Dehydrogenase type 9 (ACAD9) and Mitochondrial Trifunctional Protein (TFP) with triheptanoin oil. This study is also open to patients with any type of glycogen storage disease, pyruvate carboxylase deficiency, type B, or Barth Syndrome.. Symptoms often persist with standard diet including supplementation with medium chain triglyceride oil. Preliminary data shows triheptanoin to reverse many of the clinical symptoms not well controlled by standard diet.. On study entry, clinical and laboratory assessments will be carried out with the subject on their usual home diet. A complete history and physical exam will be performed. An echocardiogram ...
Newborn Screening, Newborn, Newborn screening, Newborn screens, Expanded screening, Expanded newborn screening, Blood spot, Blood spot screening, Blood test, Tests for newborns, Genetic, Genetics, Genetic diseases, Genetic disorders, Genetic conditions, Genetic counseling, Genetic testing, Genetic screening, Birth defects, Diseases, Hereditary diseases, Inherited diseases, Cystic Fibrosis, CF, Hemophilia, Galactosemia, MSUD, Maple syrup urine disease, Hypothyroidism, PKU, Phenylketonuria, PKU formula, Formula, Metabolic formula, Metabolic, Metabolic conditions, Congenital Adrenal Hyperplasia, CAH, Sickle cell, Sickle cell anemia, Sickle cell disease, Sickle cell trait, Amino acid disorders, Argininosuccinic aciduria, Citrullinemia, Homocystinuria (cystathione synthase deficiency), Hypermethioninemia, Tyrosinemia, type II (TYRII), Fatty acid oxidation disorders, Carnitine/acylcarnitine translocase defect, Carnitine palmitoyl transferase deficiency (SCAD), Long-chain hydroxy acyl-CoA dehydrogenase
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styrene-co-maleic acid-co-methacrylate polymer: a polyanion that inhibits carnitine-dependent fatty acid oxidation in rat liver mitochondria
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From: Dmitry Vyukov ,[email protected], To: Patricia Alfonso ,[email protected], Cc: David Gow ,[email protected],, Brendan Higgins ,[email protected],, Andrey Ryabinin ,[email protected],, Ingo Molnar ,[email protected],, Peter Zijlstra ,[email protected],, Juri Lelli ,[email protected],, Vincent Guittot ,[email protected],, LKML ,[email protected],, kasan-dev ,[email protected],, [email protected], open list:KERNEL SELFTEST FRAMEWORK ,[email protected], Subject: Re: [RFC PATCH v2 3/3] KASAN: Port KASAN Tests to KUnit Date: Fri, 27 Mar 2020 06:31:18 +0100 Message-ID: ,[email protected]om, (raw) In-Reply-To: ,[email protected]om, On Thu, Mar 26, 2020 at 4:15 PM Patricia Alfonso ,[email protected], wrote: , , , , ,[email protected], wrote: , , , , , , , , , , Transfer all previous tests for KASAN to KUnit so ...
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BACKGROUND:Excessive alcohol consumption can cause hepatocellular injury. ATPase II (ATP8A1) can display an ATP-dependent phospholipid translocase activity. However, the function of ATP8A1 in hepatocyte injury is still unclear. In the present study w...
Although our bodies make carnitine they often dont produce enough. Find out how supplements can fill in the gaps for better health and energy.
The acylcarnitines in plasma and blood spots of 23 patients with proven deficiency of long-chain 3-hydroxyacylcoenzyme A dehydrogenase were reviewed. Long-chain 3-hydroxyacylcarnitines of C14:1, C14, C16 and C18:1 chain length, and long-chain acylcarnitines of C12, C14:1, C14, C16, C18:2 and C18:1 chain length were elevated. Acetylcarnitine was decreased. In plasma, elevation of hydroxy-C18:1 acylcarnitine over the 95th centile of controls, in combination with an elevation of two of the three acylcarnitines C14, C14:1 and hydroxy-C16, identified over 85% of patients with high specificity (less than 0.1% false positive rate). High endogenous levels of long-chain acylcarnitines in normal erythrocytes reduced the diagnostic specificity in blood spots compared with plasma samples. The results were also diagnostic in asymptomatic patients, and were not influenced by genotype. Treatment with diet low in fat and high in medium-chain triglyceride decreased all disease-specific acylcarnitines, often to normal,
April 11, 2013 A study published in Nature Medicine claims that carnitine, a compound abundant in red meat, sold as a dietary supplement, and present in some energy drinks, may increase the risk of heart disease. Carnitine typically helps the body transport fatty acids into cells to be used as energy. But researchers at the Cleveland Clinic found that in both humans and mice, certain bacteria in the digestive tract convert carnitine to another metabolite, called TMAO, which promotes atherosclerosis, or a thickening of the arteries. The researchers tested the carnitine and TMAO levels of omnivores, vegans, and vegetarians, and examined records of 2,595 patients undergoing cardiac evaluations. In patients with high TMAO levels, the more carnitine in their blood, the more likely they were to develop cardiovascular disease, heart attacks, stroke, and death. The researchers speculated that carnitine could be compounding the danger. "Cholesterol is still needed to clog the arteries, but TMAO changes ...
L-Carnitine is produced in the liver, brain and kidneys. Carnitine can be found naturally in the diet, mainly in dairy and meat products. Weve got carnitine liquid, tablets, caplets and more for you to browse today. Holland & Barrett | Carnitine | Amino Acids | Sports Nutrition | Holland & Barrett
What Does This Mean?. Changes in metabolites may be a response to altered biochemical pathways due to disease (Jones 2010). We believe that a change in our patients acylcarnitine profiles is a useful indicator of changes in their metabolic states. Monitoring these changes in our patients could be an opportunity to advance understanding of the mechanism of their diseases, finding new biomarkers, or new therapies.. The formation of acylcarnitines is important not only in the metabolism of fatty acids but is also an important pathway in the mechanism of the ketogenesis. Carnitine could also be involved in a host of other pathways by consequence of its free hydroxyl group. It has been suggested that L-carnitine can complex with various other metabolites and carboxylic acids of varying lengths to transport them throughout the body for a variety of functions (Jones 2010).. Our Focus. In some research circles it is believed that changes in the concentration of carnitine can be used to identify changes ...
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Jerry Vockley and Inform Network describe everything you want to know about CACT deficiency, a rare fatty acid oxidation disorder. Read more about its symptoms and some developing treatments.
CPT2Z : Confirmation of diagnosis of carnitine palmitoyltransferase II deficiency   Carrier screening in cases where there is a family history of carnitine palmitoyltransferase II deficiency, but disease-causing mutations have not been identified in an affected individual
Lipid A, the hydrophobic anchor of lipopolysaccharide, is an essential component in the outer membrane of most Gram-negative bacteria. Food-borne pathogen Cronobacter sakazakii synthesizes two lipid A species, differing by the length of the secondary acyl chain. In this work, we identified three genes ESA02293, ESA02951 and ESA01386 encoding for the late acyltransferases of lipid A biosynthesis pathway in C. sakazakii. Based on the sequence alignment, proteins YP_001438378.1 encoded by ESA02293, YP_001439016.1 encoded by ESA02951, and YP_001437482.1 encoded by ESA01386 are homologous to E. coli LpxL, LpxP and LpxM, respectively. Functions of the three acyltransferases were confirmed by overexpressing the genes in E. coli, isolating lipid As and analyzing their structures using an ESI/MS. C. sakazakii LpxL and LpxM transfer a C14:0 secondary acyl chain to the 2′- and 3′-position of lipid A, respectively. C. sakazakii LpxP can transfer either a C16:1 or a C14:0 secondary acyl chains to the 2′
Effective evaluation or prediction of therapy response could be helpful for treatment of chronic kidney disease (CKD), which may rely on accurate biomarkers. Acylcarnitines are involved with lipid metabolism and mitochondrial function. The relation of acylcarnitines with treatment response in patients with CKD is unknown. The purpose of this study is to investigate the association of plasma acylcarnitines with renal function and its alteration by intervention in patients with IgA nephropathy (IgAN). A retrospective study was performed in 81 IgAN patients with treatment by traditional Chinese medicine (TCM). Multivariate linear regression analyses were performed to identify the association of acylcarnitines with baseline estimated glomerular filtration rate (eGFR) and eGFR changes after treatment. Twenty-seven acylcarnitines were measured at baseline and after 1-year TCM intervention. Certain short-chain and median-chain acylcarnitines were independently associated with baseline eGFR and eGFR alterations
The conversion of 6-N-[Me-14C]trimethyl-lysine into carnitine and 4-N-trimethylaminobutyrate (butyrobetaine) was demonstrated in rats kept on a lysine-deficient diet. After the rats were given [14C]trimethyl-lysine for 4 days, a total of 17% of the injected label was recovered as carnitine from carcass and urine extracts. Another 8% of the trimethyl-lysine label was converted into 4-N-trimethylaminobutyrate, most of which was recovered from the urine. The conversion of trimethyl-lysine into the above two metabolites supports the pathway of carnitine biosynthesis as lysine+methionine → 6-N-trimethyl-lysine → 4-N-trimethylaminobutyrate → carnitine. In addition, three other metabolites representing 2% of the injected dose were recovered. Only an insignificant portion of the label was recovered as free trimethyl-lysine from the carcass, whereas 22% of the injected label was recovered in the urine. A relatively low specific radioactivity in carnitine was found when ...
Raising your carnitine levels will fight this visceral fat gain because it increases fat burning, which has the effect of taking triglycerides and low-density lipoproteins out of the system so that they dont build up causing high cholesterol and atherosclerosis. A new research study in the journal Food and Chemical Toxicology illustrates this. Researchers gave a carnitine supplement to mice who were fed a high-fat diet in order to make them gain weight. In comparison to a group of mice fed a placebo, the carnitine group gained substantially less visceral and subcutaneous fat (fat that is right below the surface of the skin that you can pinch with your fingers). The placebo group exhibited the beginning stages of non-alcoholic fatty liver disease and atherosclerosis, neither of which were evident in the carnitine group ...
This Pilot study follows up on a publication entitled A novel X-linked inborn error of carnitine biosynthesis and a neuronal carnitine pathway hypothesis for autism. This paper describes a new genetic condition (called TMLHE deficiency) that results in the loss of ability to make carnitine in the body.
Dear all, Please find attached the Call For Papers for: 18th IEEE Symposium on Computer-Based Medical Systems (CBMS) - Track on Grids for Biomedicine and Bioinformatics. Dublin, Ireland 23-24 June 2005 http://datadog.unile.it/cbms2005/cfp.htm http://conferences.computer.org/CBMS2005/index.html sponsored by IEEE Computer Society The main goal of the track is to discuss well-known and emerging bio data-intensive systems in the context of Grids and to analyse technologies and methodologies useful to develop such systems in these environments. In particular, this Conference Track aims at offering a forum of discussion where young researchers and PhD students could present their research activities, either at an early or mature phase. Best regards, Maria Mirto. ~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~ Maria Mirto, CACT/ISUFI (Center for Advanced Computing Technology) Engineering Faculty, Department of Innovation Engineering University of Lecce, Via per Monteroni, 73100 ...
En el presente artículo se advierte de la presencia del pseudococcino Rhizoecus cacticans (HAMBLETON) en la zona de Valencia, atacando a raíces de cactáceas. Se dan algunas características del género y se describe la especie, así como algunos datos sobre los daños que causa. Se indican procedimientos posibles de control ...
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CPT2 (aa406-418) antibody, Internal (carnitine palmitoyltransferase 2) for WB. Anti-CPT2 (aa406-418) pAb (GTX88238) is tested in Human samples. 100% Ab-Assurance.
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A popular doctor with a website sells a supplement for people over 50 who want more energy that has alpha lipoic acid and l-carnitine. Its very
L-carnitine is a naturally occurring derivative of the amino acid carnitine, which plays a vital role in the metabolism of fat. It functions as a...
TY - JOUR. T1 - Enhanced uptake of carnitine by perfused rat liver following starvation. AU - Kispal, Gyula. AU - Melegh, Bela. AU - Alkonyi, Istvan. AU - Sandor, Attila. PY - 1987/1/9. Y1 - 1987/1/9. N2 - Previously, the release of carnitine from the perfused rat liver was found to be protein-mediated, dependent on the nutritional state but not on metabolic energy. Further, it was shown to exceed the physiological demand by about 10-fold (Sandor et al. (1985) Biochim. Biophys. Acta 835, 83-91). In the present study the uptake of carnitine by perfused rat liver has been investigated. (1) The liver tissue and the perfusate were in equilibrium when the carnitine concentration in the perfusate was close to 45 μM, physiological in the rat plasma. Under this condition, when no net carnitine transport occurred, an unidirectional uptake of l-[3H]carnitine was observed. Quantitatively, the uptake rate was 355 ± 60 (S.D.) nmol/h per 100 g body weight at 45-50 μM perfusate concentration. This uptake ...
As these molecules started to accumulate, the availability of free, unprocessed carnitine decreased. This imbalance was linked to fuel-burning problems, that is, impairments in the cells combustion of both fat and glucose fuel. "We suspected that persistent increases in acylcarnitines in the rats were causing problems, and we could also see that the availability of free carnitine was decreasing with weight gain and aging," said Muoio, according to an August 12, 2009 news release, Carnitine supplements reverse glucose intolerance in animals. "It appeared that carnitine could no longer do its job when chronic metabolic disruptions were stressing the system. Thats when we designed an experiment to add extra carnitine to the rats diet." Muoio (at the time of the news release) also is a professor in the departments of medicine, pharmacology and cancer biology ...
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Drosophila (combinação moderna das palavras gregas δρόσος, transl. drosos, orvalho, e φίλος, transl. phílos, amor, afinidade, com a desinência feminina latina -a) é um género formado por um grande número de espécies de pequenas moscas. Atualmente, existem cerca de duas mil espécies descritas no gênero. As drosófilas (não confundir com moscas-das-frutas, da família Tephritidae, que causam prejuízo aos fruticultores) alimentam-se de leveduras em frutos já caídos, em início de decomposição, e, portanto, não causam prejuízo. Algumas espécies se alimentam em outros substratos, como cactáceas (também em início de decomposição) e guano de morcego, entre outros. Dentre todas as espécies do gênero, a mais conhecida é a Drosophila melanogaster. Esta espécie foi usada como modelo em pesquisas que contribuíram para o desenvolvimento de importantes conceitos de Genética. ...
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Complete information for CPT1B gene (Protein Coding), Carnitine Palmitoyltransferase 1B, including: function, proteins, disorders, pathways, orthologs, and expression. GeneCards - The Human Gene Compendium
Complete information for CPT1B gene (Protein Coding), Carnitine Palmitoyltransferase 1B, including: function, proteins, disorders, pathways, orthologs, and expression. GeneCards - The Human Gene Compendium
Livon-Lypo-Spheric-ACETYL L-CARNITINE is the leading liposomal supplement that contains ALCAR and helps raise the levels of carnitine in your brain. It assimilates di..
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Forensic testing uses DNA sequences to identify an individual for legal purposes. Unlike the tests described above, forensic testing is not used to detect gene mutations associated with disease. This type of testing can identify crime or catastrophe victims, rule out or implicate a crime suspect, or establish biological relationships between people (for example, paternity). 42 Carnitine Palmitoyltransferase I Deficiency How Is Genetic Testing Done? Once a person decides to proceed with genetic testing, a medical geneticist, primary care doctor, specialist, or nurse practitioner can order the test. Unlike the tests described above, forensic testing is not used to detect gene mutations associated with disease. This type of testing can identify crime or catastrophe victims, rule out or implicate a crime suspect, or establish biological relationships between people (for example, paternity). 42 Carnitine Palmitoyltransferase I Deficiency How Is Genetic Testing Done? Once a person decides to proceed ...
Carnitine palmitoyltransferase II deficiency (CPT-II) is an autosomal recessively inherited genetic metabolic disorder characterized by an enzymatic defect that prevents long-chain fatty acids from being transported into the mitochondria for utilization as an energy source. The adult myopathic form of this disease was first characterized in 1973 by DiMauro and DiMauro. It is the most common inherited disorder of lipid metabolism affecting the skeletal muscle of adults. CPT II deficiency is also the most frequent cause of hereditary myoglobinuria. Symptoms of this disease are commonly provoked by prolonged exercise or periods without food. There are three main types of carnitine palmitoyltransferase II deficiency classified on the basis of tissue-specific symptomotology and age of onset: Mild to severe adult myopathic form Severe infantile multisystemic form Lethal neonatal form It should be noted that among the few people diagnosed with CPT2, some have unknown and/or novel mutations that place ...
acylcarnitine; carnitine; carnitine palmitoyltransferase; chronic fatigue syndrome; fatty acid. Abstract. Reuter SE, Evans AM (School of Pharmacy & Medical Sciences, University of South Australia, Adelaide, SA, Australia; Sansom Institute for Health Research, University of South Australia, Adelaide, SA, Australia). Long-chain acylcarnitine deficiency in patients with chronic fatigue syndrome. Potential involvement of altered carnitine palmitoyltransferase-I activity. J Intern Med 2011; 270: 76-84.. Objective. The underlying aetiology of chronic fatigue syndrome is currently unknown; however, in the light of carnitines critical role in mitochondrial energy production, it has been suggested that chronic fatigue syndrome may be associated with altered carnitine homeostasis. This study was conducted to comparatively examine full endogenous carnitine profiles in patients with chronic fatigue syndrome and healthy controls.. Design. A cross-sectional, observational study.. Setting and subjects. ...
The principal goal for the study is to examine the role gut flora plays in modulating metabolism of dietary trimethylamine nutrients in humans. We have recently shown that dietary intake of two trimethylamines abundant in animal products, carnitine and the choline group of phosphatidylcholine (PC), are mechanistically linked to cardiovascular disease risk. We wish to further explore the metabolism of these nutrients in humans, and to test the hypothesis that the intestinal micro flora (gut flora) plays a critical role in generation of metabolites from dietary carnitine and choline/PC linked to cardiometabolic disease. We further hypothesize that the production of specific metabolites of carnitine and choline/PC are influenced by the composition of gut flora, and these may be altered by the preceding dietary patterns of the subjects. We therefore wish to test whether dietary supplementation with carnitine and/or choline alters the metabolism of carnitine and choline/PC in subjects. We also ...
The researchers examined the records of 2,595 meat eaters and vegetarians undergoing cardiac evaluations and found that patients with high levels of TMAO were more likely to develop cardiovascular disease and suffer heart attacks, strokes and death.. Dr. Stanley Hazen, chief of cellular and molecular medicine at Lerner, said carnitine may be compounding the negative effects of the cholesterol and saturated fat found in beef, pork and other red meat.. However, Dr. Betsy Booren, chief scientist at the American Meat Institute, noted that cardiovascular disease is "a complex condition" thats associated with a number of factors, from genetics to lifestyle, and attempts to link it to "a single compound found at safe levels in red meat oversimplifies" the issue.. Many other studies examining carnitine have not shown adverse effects at a number of doses, she said, adding that a fact sheet from the National Institutes of Health concludes that carnitine is essential and safe.. In fact, Hazen said some ...
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Grösse: 77cm, Gewicht: 53kg. Coursinglicence: OE C 224. Bonitation: 15. 11. 2009. Heartcheck: 10. 12. 2009, clear; 14. 12. 2011, clear; 4. 2. 2013, AV valve insufficiency without pulmonary arterial hypertension;. Working class certification: 30. 5. 2010. Czech Coursingmaster 2010. Intern. Coursingchampion (Requirements fullfilled on 13. 6. 2010, confirmation on 17. 2. 2011). 2x BiF, 4x CACIL, 2x resCACIL, 1x CACT, 3x resCACT, 2x CCLA, 3x placement at CEM in 20 competitions including 3 Coursing European Masterships. Every competition finished even the one when she got injured by attacking bitch.. Day of death: 10. 3. 2014, ...
Question for those more researched in the area of SCFAs, MCT, and carnitine... I know SCFAs are produced by certain beneficial bacteria in the gut,...
To the Editor.-Kieval and colleagues1 recently described a 60-year-old woman presumably heterozygous for carnitine palmityltransferase deficiency who had fixed
Researchers looked at carnitine supplementation in rats and confirmed its ability to reduce body fat and suppress muscle loss. It just might be one of the supplements worth taking.
What Is It: Carnitine will help improve overall body composition by leaning you out through fat loss and preserving muscle mass and prevents muscle catabolism
Novel acyl-derivatives of the β-hydroxy-γ-butyrobetaine are disclosed (typically pyruvyl carnitine hydrochloride) which are useful therapeutic agents in the treatment of cardiac disorders, hyperlipidoemias and hyperlipoproteinemias.
This enzyme belongs to the family of transferases, specifically those acyltransferases transferring groups other than aminoacyl groups. This enzyme participates in lysine
Carnitine is an essential factor in long-chain fatty acid oxidation. Carnitine acts as a carrier of fatty acyl groups from the cytoplasm to the mitochondrion. Long-chain acyl-CoA derivatives do not penetrate the mitochondrial inner membrane. Carnitine palmitoyltransferase A (CPT-A), located on the e …
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Vegetarian athletes generally eat better than their meat-eating counterparts, but there are some nutrients (e.g. vitamin B12) that they lack including one crucial amino acid that is key to muscle performance and recovery: carnitine.. Normally, carnitine is supplied in meat and dairy products, items that vegetarians usually avoid unless they are ovo-lacto (able to consume eggs and dairy) (1, 2). Blood concentrations of carnitine in vegetarians are reported to be strikingly low or around 20-30 percent lower than their meat-eating counterparts and suggests that tissue carnitine stores in the body are also low in concentration (3).. Unfortunately, lower carnitine intake in vegetarians has received relatively little attention (4). But the role carnitine plays in the body is one of heavy involvement in energy metabolism and cellular protection. Additionally, 95 percent of the bodys carnitine is found in muscle tissue.. Carnitine supplementation is shown to correct low concentrations and support ...
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Acetyl forms of amino acids are directly taken into the brain, and Carnitine is no exception. This is where the majority of actions take place, with the Acetyl form used to treat memory related illness and neuropathies.. The body can convert Acetyl L Carnitine into L Carnitine and visa versa, however, if you are wanting weight loss, best use L-Carnitine alone and if you are treating any brain related disorders, use the Acetyl form.. Acetyl L Carnitine is used for age related memory loss, Alzheimers disease, late life depression, cataracts, pain due to diabetes type 2 and pain associated with drugs that are used to treat HIV AIDS.. In Alzheimers disease, this amino acid seems to work best with reducing rapid onset Alzheimers disease presenting in those under 65 years of age. Also great for muscle function, Acetyl L Carnitine is vital for health of the heart muscle and also reduces prostate inflammation. Useful also for increasing aged related testosterone decline.. Acetyl-L-Carnitine by Seeking ...
Introduction. The First Law of Thermodynamics states that energy can be neither created nor destroyed. This means that energy, instead of disappearing, is either transformed, transferred, dispersed, or dissipated. When energy is lost by a system, it will be acquired by the surroundings. Heat can be described as the amount of energy needed to cause the temperature of a substance to rise and it is transferred from warmer areas to cooler ones. In order to be able to measure the change in heat or enthalpy of a reaction, a colorimeter can be used. The calorimeter was first introduced in the 18th century and can be used with any procedure that involves the flow heat between a system and its surroundings (CACT). It is capable of measuring the heat created or exchanged after a reaction has occurred in a system with a constant pressure.. A calorimeter can be used to find the specific heat of a substance or even the heat of neutralization between a base and an acid. A basic calorimeter is composed of ...
Hi Erick, The question of carnitine supplementation for the treatment of neuropathy is a good one! We are beginning to discover the underlying mechanisms for various side effects -- such as the...
Numerous hits in gapped BLAST to probable 1-acyl-sn-glycerol-3-phosphate acyltransferases, e.g. residues 3-253 are 32% similar to PLSC_MYCGE (MG212). Apart from the similarities to mycoplasma proteins, BLAST scores are below 100 due to the lack of similarity over the NH-terminal residues. No significant similarity to T.pallidum or C.trachomatis ...
L-carnitine is the biologically active form of the nonessential amino acid carnitine. Carnitine is produced in your body from the two amino acids...
Acetyl L-Carnitine HCL is a potent super nutrient that supports the body in the same manner as L-Carnitine, but also has the ability to pass through the blood-brain barrier. Acetyl L- Carnitine HCL supports mental sharpness by stimulating acetlycho
An important function of L-carnitine is to facilitate the breakdown and transport of long-chain fatty acids from the cell plasma into the mitochondria where they can be burned for fuel, creating energy in the form of ATP. Studies have also found that carnitine ...
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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: ...
His lab focuses on enzymes involved in fatty acid metabolism, including Acetyl-CoA carboxylase, carnitine acyltransferase, AMP- ...
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 ...
Malonyl CoA inhibits fatty acids from associating with carnitine by regulating the enzyme carnitine acyltransferase, thereby ...
... carnitine palmitoyltransferase and glycerophosphate acyltransferase compared to peroxisomal beta-oxidation and palmitoyl-CoA ...
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 ...
... new fatty acids and can inhibit the transfer of the fatty acyl group from acyl CoA to carnitine with carnitine acyltransferase ...
... carnitine and carnitine acyltransferases I and II reducing their bioavailability and consequently inhibiting beta oxidation of ...
... 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 ...
... but not as a protease or a carnitine acyltransferase". Archives of Biochemistry and Biophysics. 323 (2): 397-403. doi:10.1006/ ...
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- ...
... 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- ...
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 ...
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 ...
... (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 ...
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 ...
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 ( ...
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 ...
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 ( ...
Glycerol-3-phosphate O-acyltransferase. *1-acylglycerol-3-phosphate O-acyltransferase. *2-acylglycerol-3-phosphate O- ...
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 ...
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 ...
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. 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 ...
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 ...
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 ...
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 ...
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 ...
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 ...
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. ...
... 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 ...
Study Set 16 Enzymes and Hormones flashcards from Languages 247365
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 ...
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) ...
... 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 ...
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 ...
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, ( ...
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 ...
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 ...
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 ...
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 ...
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 ...
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 ...
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, ...
  • Carnitine palmitoyltransferase II deficiency (CPT-II) is an autosomal recessively inherited genetic metabolic disorder characterized by an enzymatic defect that prevents long-chain fatty acids from being transported into the mitochondria for utilization as an energy source. (wikipedia.org)
  • There are three main types of carnitine palmitoyltransferase II deficiency classified on the basis of tissue-specific symptomotology and age of onset: Mild to severe adult myopathic form Severe infantile multisystemic form Lethal neonatal form It should be noted that among the few people diagnosed with CPT2, some have unknown and/or novel mutations that place them outside these three categories while remaining positive for CPT2. (wikipedia.org)
  • The catalytic core of the CPT II enzyme contains three important binding sites that recognize structural aspects of CoA, palmitoyl, and carnitine. (wikipedia.org)
  • 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)