Carnitine
Carnitine O-Palmitoyltransferase
Serine C-Palmitoyltransferase
Malonyl Coenzyme A
Palmitoyl Coenzyme A
Acyltransferases
Carnitine Acyltransferases
Carnitine O-Acetyltransferase
Acyl Coenzyme A
Mitochondria, Liver
Mitochondria in hepatocytes. As in all mitochondria, there are an outer membrane and an inner membrane, together creating two separate mitochondrial compartments: the internal matrix space and a much narrower intermembrane space. In the liver mitochondrion, an estimated 67% of the total mitochondrial proteins is located in the matrix. (From Alberts et al., Molecular Biology of the Cell, 2d ed, p343-4)
Palmitoylcarnitine
Sphingolipids
A class of membrane lipids that have a polar head and two nonpolar tails. They are composed of one molecule of the long-chain amino alcohol sphingosine (4-sphingenine) or one of its derivatives, one molecule of a long-chain acid, a polar head alcohol and sometimes phosphoric acid in diester linkage at the polar head group. (Lehninger et al, Principles of Biochemistry, 2nd ed)
Epoxy Compounds
Acetylcarnitine
Hereditary Sensory and Autonomic Neuropathies
A group of inherited disorders characterized by degeneration of dorsal root and autonomic ganglion cells, and clinically by loss of sensation and autonomic dysfunction. There are five subtypes. Type I features autosomal dominant inheritance and distal sensory involvement. Type II is characterized by autosomal inheritance and distal and proximal sensory loss. Type III is DYSAUTONOMIA, FAMILIAL. Type IV features insensitivity to pain, heat intolerance, and mental deficiency. Type V is characterized by a selective loss of pain with intact light touch and vibratory sensation. (From Joynt, Clinical Neurology, 1995, Ch51, pp142-4)
Fatty Acids
Ketone Bodies
Liver
Palmitic Acid
Oxidation-Reduction
A chemical reaction in which an electron is transferred from one molecule to another. The electron-donating molecule is the reducing agent or reductant; the electron-accepting molecule is the oxidizing agent or oxidant. Reducing and oxidizing agents function as conjugate reductant-oxidant pairs or redox pairs (Lehninger, Principles of Biochemistry, 1982, p471).
gamma-Butyrobetaine Dioxygenase
Rats, Inbred Strains
Organic Cation Transport Proteins
Submitochondrial Particles
Lipid Metabolism, Inborn Errors
Mitochondria, Muscle
Ceramides
Microbodies
Caprylates
Citrate (si)-Synthase
Sphingomonas
Digitonin
Sphingosine N-Acyltransferase
Intracellular Membranes
Isoenzymes
Lipid Metabolism
Escherichia coli O157
A verocytotoxin-producing serogroup belonging to the O subfamily of Escherichia coli which has been shown to cause severe food-borne disease. A strain from this serogroup, serotype H7, which produces SHIGA TOXINS, has been linked to human disease outbreaks resulting from contamination of foods by E. coli O157 from bovine origin.
Sphingosine
Molecular Sequence Data
Descriptions of specific amino acid, carbohydrate, or nucleotide sequences which have appeared in the published literature and/or are deposited in and maintained by databanks such as GENBANK, European Molecular Biology Laboratory (EMBL), National Biomedical Research Foundation (NBRF), or other sequence repositories.
Betaine
A naturally occurring compound that has been of interest for its role in osmoregulation. As a drug, betaine hydrochloride has been used as a source of hydrochloric acid in the treatment of hypochlorhydria. Betaine has also been used in the treatment of liver disorders, for hyperkalemia, for homocystinuria, and for gastrointestinal disturbances. (From Martindale, The Extra Pharmacopoeia, 30th ed, p1341)
Acetyl-CoA Carboxylase
Sphingomyelins
Oleic Acids
Mitochondria
Semiautonomous, self-reproducing organelles that occur in the cytoplasm of all cells of most, but not all, eukaryotes. Each mitochondrion is surrounded by a double limiting membrane. The inner membrane is highly invaginated, and its projections are called cristae. Mitochondria are the sites of the reactions of oxidative phosphorylation, which result in the formation of ATP. They contain distinctive RIBOSOMES, transfer RNAs (RNA, TRANSFER); AMINO ACYL T RNA SYNTHETASES; and elongation and termination factors. Mitochondria depend upon genes within the nucleus of the cells in which they reside for many essential messenger RNAs (RNA, MESSENGER). Mitochondria are believed to have arisen from aerobic bacteria that established a symbiotic relationship with primitive protoeukaryotes. (King & Stansfield, A Dictionary of Genetics, 4th ed)
Myocardium
Amino Acid Sequence
Muscle, Skeletal
Oleic Acid
Clofibrate
Gene Expression Regulation, Enzymologic
Acyl-CoA Oxidase
3-Hydroxyacyl CoA Dehydrogenases
Acyl-CoA Dehydrogenase, Long-Chain
Acyl-CoA Dehydrogenase
Rats, Wistar
RNA, Messenger
RNA sequences that serve as templates for protein synthesis. Bacterial mRNAs are generally primary transcripts in that they do not require post-transcriptional processing. Eukaryotic mRNA is synthesized in the nucleus and must be exported to the cytoplasm for translation. Most eukaryotic mRNAs have a sequence of polyadenylic acid at the 3' end, referred to as the poly(A) tail. The function of this tail is not known for certain, but it may play a role in the export of mature mRNA from the nucleus as well as in helping stabilize some mRNA molecules by retarding their degradation in the cytoplasm.
Pichia
Oxygen Consumption
PPAR alpha
Substrate Specificity
Bezafibrate
Esterification
The process of converting an acid into an alkyl or aryl derivative. Most frequently the process consists of the reaction of an acid with an alcohol in the presence of a trace of mineral acid as catalyst or the reaction of an acyl chloride with an alcohol. Esterification can also be accomplished by enzymatic processes.
Benzyl Alcohol
Detergents
O(6)-Methylguanine-DNA Methyltransferase
Ceramidases
Base Sequence
Fatty Acids, Nonesterified
Vitamin B Deficiency
Fumonisins
Diabetes Mellitus, Experimental
Energy Metabolism
Diabetic Ketoacidosis
Rats, Sprague-Dawley
Blotting, Western
Sequence Homology, Amino Acid
Cells, Cultured
Sphingomyelin Phosphodiesterase
Binding Sites
Catalysis
Mutation
Octoxynol
Oxidoreductases
The class of all enzymes catalyzing oxidoreduction reactions. The substrate that is oxidized is regarded as a hydrogen donor. The systematic name is based on donor:acceptor oxidoreductase. The recommended name will be dehydrogenase, wherever this is possible; as an alternative, reductase can be used. Oxidase is only used in cases where O2 is the acceptor. (Enzyme Nomenclature, 1992, p9)
DNA, Complementary
DNA Primers
3-Hydroxybutyric Acid
Microsomes
Artifactual vesicles formed from the endoplasmic reticulum when cells are disrupted. They are isolated by differential centrifugation and are composed of three structural features: rough vesicles, smooth vesicles, and ribosomes. Numerous enzyme activities are associated with the microsomal fraction. (Glick, Glossary of Biochemistry and Molecular Biology, 1990; from Rieger et al., Glossary of Genetics: Classical and Molecular, 5th ed)
Enzyme Inhibitors
Saccharomyces cerevisiae
Mitochondrial Membranes
The two lipoprotein layers in the MITOCHONDRION. The outer membrane encloses the entire mitochondrion and contains channels with TRANSPORT PROTEINS to move molecules and ions in and out of the organelle. The inner membrane folds into cristae and contains many ENZYMES important to cell METABOLISM and energy production (MITOCHONDRIAL ATP SYNTHASE).
AMP-Activated Protein Kinases
Intracellular signaling protein kinases that play a signaling role in the regulation of cellular energy metabolism. Their activity largely depends upon the concentration of cellular AMP which is increased under conditions of low energy or metabolic stress. AMP-activated protein kinases modify enzymes involved in LIPID METABOLISM, which in turn provide substrates needed to convert AMP into ATP.
Hydroxymethylglutaryl-CoA Synthase
Reye Syndrome
Glucose
Inhibitory Concentration 50
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).
Adipose Tissue, Brown
Glucagon
A 29-amino acid pancreatic peptide derived from proglucagon which is also the precursor of intestinal GLUCAGON-LIKE PEPTIDES. Glucagon is secreted by PANCREATIC ALPHA CELLS and plays an important role in regulation of BLOOD GLUCOSE concentration, ketone metabolism, and several other biochemical and physiological processes. (From Gilman et al., Goodman and Gilman's The Pharmacological Basis of Therapeutics, 9th ed, p1511)
Cloning, Molecular
Lipids
A generic term for fats and lipoids, the alcohol-ether-soluble constituents of protoplasm, which are insoluble in water. They comprise the fats, fatty oils, essential oils, waxes, phospholipids, glycolipids, sulfolipids, aminolipids, chromolipids (lipochromes), and fatty acids. (Grant & Hackh's Chemical Dictionary, 5th ed)
Receptors, Cytoplasmic and Nuclear
Intracellular receptors that can be found in the cytoplasm or in the nucleus. They bind to extracellular signaling molecules that migrate through or are transported across the CELL MEMBRANE. Many members of this class of receptors occur in the cytoplasm and are transported to the CELL NUCLEUS upon ligand-binding where they signal via DNA-binding and transcription regulation. Also included in this category are receptors found on INTRACELLULAR MEMBRANES that act via mechanisms similar to CELL SURFACE RECEPTORS.
Membrane Proteins
Fatty Acid Synthases
Gene Expression
Carrier Proteins
Transcription, Genetic
Hyperammonemia
Dietary Fats
Cell Fractionation
Protein Binding
Organ Specificity
Mutagenesis, Site-Directed
Cricetinae
Cell Membrane
Cattle
Mersalyl
Protein Structure, Tertiary
The level of protein structure in which combinations of secondary protein structures (alpha helices, beta sheets, loop regions, and motifs) pack together to form folded shapes called domains. Disulfide bridges between cysteines in two different parts of the polypeptide chain along with other interactions between the chains play a role in the formation and stabilization of tertiary structure. Small proteins usually consist of only one domain but larger proteins may contain a number of domains connected by segments of polypeptide chain which lack regular secondary structure.
Mitochondrial Proteins
Fatty Liver
Gene Expression Regulation
Modification of left ventricular hypertrophy by chronic etomixir treatment. (1/672)
1. Etomoxir (2[6(4-chlorophenoxy)hexyl]oxirane-2-carboxylate), an irreversible carnitine palmitoyl-transferase 1 inhibitor, reduces the expression of the myocardial foetal gene programme and the functional deterioration during heart adaption to a pressure-overload. Etomoxir may, however, also improve the depressed myocardial function of hypertrophied ventricles after a prolonged pressure overload. 2. To test this hypothesis, we administered racemic etomoxir (15 mg kg(-1) day(-1) for 6 weeks) to rats with ascending aortic constriction beginning 6 weeks after imposing the pressure overload. 3. The right ventricular/body weight ratio increased (P<0.05) by 20% in etomoxir treated rats (n = 10) versus untreated rats with ascending aortic constriction (n = 10). Left ventricular weight was increased (P<0.05) by 8%. Etomoxir blunted the increase in left ventricular chamber volume. Etomoxir raised the proportion of V1 isomyosin (35+/-4% versus 24+/-2%; P<0.05) and decreased the percentage of V3 isomyosin (36+/-4% versus 48+/-3%; P<0.05). 4. Maximum isovolumically developed pressure was higher in etomoxir treated rats than in untreated pressure overloaded rats (371+/-22 versus 315+/-23 mmHg; P<0.05). Maximum rates of ventricular pressure development (14,800+/-1310 versus 12,340+/-1030mmHg s(-1); P<0.05) and decline (6440+/-750 versus 5040+/-710 mmHg s(-1); P<0.05) were increased as well. Transformation of pressure values to ventricular wall stress data revealed an improved myocardial function which could partially account for the enhanced function of the whole left ventricle. 5. The co-ordinated action of etomoxir on ventricular mass, geometry and myocardial phenotype enhanced thus the pressure generating capacity of hypertrophied pressure-overloaded left ventricles and delayed the deleterious dilative remodelling. (+info)Pharmacokinetic analysis of the cardioprotective effect of 3-(2,2, 2-trimethylhydrazinium) propionate in mice: inhibition of carnitine transport in kidney. (2/672)
The site of action of 3-(2,2,2-trimethylhydrazinium) propionate (THP), a new cardioprotective agent, was investigated in mice and rats. I.p. administration of THP decreased the concentrations of free carnitine and long-chain acylcarnitine in heart tissue. In isolated myocytes, THP inhibited free carnitine transport with a Ki of 1340 microM, which is considerably higher than the observed serum concentration of THP. The major cause of the decreased free carnitine concentration in heart was found to be the decreased serum concentration of free carnitine that resulted from the increased renal clearance of carnitine by THP. The estimated Ki of THP for inhibiting the reabsorption of free carnitine in kidneys was 52.2 microM, which is consistent with the serum THP concentration range. No inhibition of THP on the carnitine palmitoyltransferase activity in isolated mitochondrial fractions was observed. These results indicate that the principal site of action of THP as a cardioprotective agent is the carnitine transport carrier in the kidney, but not the carrier in the heart. (+info)A single amino acid change (substitution of glutamate 3 with alanine) in the N-terminal region of rat liver carnitine palmitoyltransferase I abolishes malonyl-CoA inhibition and high affinity binding. (3/672)
We have recently shown by deletion mutation analysis that the conserved first 18 N-terminal amino acid residues of rat liver carnitine palmitoyltransferase I (L-CPTI) are essential for malonyl-CoA inhibition and binding (Shi, J., Zhu, H., Arvidson, D. N. , Cregg, J. M., and Woldegiorgis, G. (1998) Biochemistry 37, 11033-11038). To identify specific residue(s) involved in malonyl-CoA binding and inhibition of L-CPTI, we constructed two more deletion mutants, Delta12 and Delta6, and three substitution mutations within the conserved first six amino acid residues. Mutant L-CPTI, lacking either the first six N-terminal amino acid residues or with a change of glutamic acid 3 to alanine, was expressed at steady-state levels similar to wild type and had near wild type catalytic activity. However, malonyl-CoA inhibition of these mutant enzymes was reduced 100-fold, and high affinity malonyl-CoA binding was lost. A mutant L-CPTI with a change of histidine 5 to alanine caused only partial loss of malonyl-CoA inhibition, whereas a mutant L-CPTI with a change of glutamine 6 to alanine had wild type properties. These results demonstrate that glutamic acid 3 and histidine 5 are necessary for malonyl-CoA binding and inhibition of L-CPTI by malonyl-CoA but are not required for catalysis. (+info)Comparisons of flux control exerted by mitochondrial outer-membrane carnitine palmitoyltransferase over ketogenesis in hepatocytes and mitochondria isolated from suckling or adult rats. (4/672)
The primary aim of this paper was to calculate and report flux control coefficients for mitochondrial outer-membrane carnitine palmitoyltransferase (CPT I) over hepatic ketogenesis because its role in controlling this pathway during the neonatal period is of academic importance and immediate clinical relevance. Using hepatocytes isolated from suckling rats as our model system, we measured CPT I activity and carbon flux from palmitate to ketone bodies and to CO2 in the absence and presence of a range of concentrations of etomoxir. (This is converted in situ to etomoxir-CoA which is a specific inhibitor of the enzyme.) From these data we calculated the individual flux control coefficients for CPT I over ketogenesis, CO2 production and total carbon flux (0.51 +/- 0.03; -1.30 +/- 0.26; 0.55 +/- 0.07, respectively) and compared them with equivalent coefficients calculated by similar analyses [Drynan, L., Quant, P.A. & Zammit, V.A. (1996) Biochem. J. 317, 791-795] in hepatocytes isolated from adult rats (0.85 +/- 0.20; 0.23 +/- 0.06; 1.06 +/- 0.29). CPT I exerts significantly less control over ketogenesis in hepatocytes isolated from suckling rats than those from adult rats. In the suckling systems the flux control coefficients for CPT I over ketogenesis specifically and over total carbon flux (< 0.6) are not consistent with the enzyme being rate-limiting. Broadly similar results were obtained and conclusions drawn by reanalysis of previous data {from experiments in mitochondria isolated from suckling or adult rats [Krauss, S., Lascelles, C.V., Zammit, V.A. & Quant, P.A. (1996) Biochem. J. 319, 427-433]} using a different approach of control analysis, although it is not strictly valid to compare flux control coefficients from different systems. Our overall conclusion is that flux control coefficients for CPT I over oxidative fluxes from palmitate (or palmitoyl-CoA) differ markedly according to (a) the metabolic state, (b) the stage of development, (c) the specific pathway studied and (d) the model system. (+info)Evidence that carnitine palmitoyltransferase I (CPT I) is expressed in microsomes and peroxisomes of rat liver. Distinct immunoreactivity of the N-terminal domain of the microsomal protein. (5/672)
Mitochondria, microsomes and peroxisomes all express overt (cytosol-facing) carnitine palmitoyltransferase activity that is inhibitable by malonyl-CoA. The overt carnitine palmitoyltransferase activity (CPTo) associated with the different fractions was measured. Mitochondria accounted for 65% of total cellular CPTo activity, with the microsomal and peroxisomal contributions accounting for the remaining 25% and 10%, respectively. In parallel experiments, rat livers were perfused in situ with medium containing dinitrophenyl (DNP)-etomoxir in order to inhibit quantitatively and label covalently (with DNP-etomoxiryl-CoA) the molecular species responsible for CPTo activity in each of the membrane systems under near-physiological conditions. In all three membrane fractions, a single protein with an identical molecular mass of approximately 88,000 kDa (p88) was labelled after DNP-etomoxir perfusion of the liver. The abundance of labelled p88 was quantitatively related to the respective specific activities of CPTo in each fraction. On Western blots the same protein was immunoreactive with three anti-peptide antibodies raised against linear epitopes of the cytosolic N- and C-domains and of the inter-membrane space loop (L) domain of the mitochondrial enzyme (L-CPT I). However, the reaction of the microsomal protein with the anti-N peptide antibody (raised against epitope Val-14-Lys-29 of CPT I) was an order of magnitude stronger than expected from either microsomal CPTo activity or its DNP-etomoxiryl-CoA labelling. This suggests that the N-terminal domain of the microsomal protein differs from that in the mitochondrial or peroxisomal protein. This conclusion was confirmed using antibody back-titration experiments, in which the binding of anti-N and anti-C antibodies by mitochondria and microsomes was quantified. (+info)Expression of the rat liver carnitine palmitoyltransferase I (CPT-Ialpha) gene is regulated by Sp1 and nuclear factor Y: chromosomal localization and promoter characterization. (6/672)
Carnitine palmitoyltransferase (CPT)-I catalyses the transfer of long-chain fatty acids from CoA to carnitine for translocation across the mitochondrial inner membrane. Expression of the 'liver' isoform of the CPT-I gene (CPT-Ialpha) is subject to developmental, hormonal and tissue-specific regulation. To understand the basis for control of CPT-Ialpha gene expression, we have characterized the proximal promoter of the CPT-Ialpha gene. Here, we report the sequence of 6839 base pairs of the promoter and the localization of the rat CPT-Ialpha gene to region q43 on chromosome 1. Our studies show that the first 200 base pairs of the promoter are sufficient to drive transcription of the CPT-Ialpha gene. Within this region are two sites that bind both Sp1 and Sp3 transcription factors. In addition, nuclear factor Y (NF-Y) binds the proximal promoter. Mutation at the Sp1 or NF-Y sites severely decreases transcription from the CPT-Ialpha promoter. Other protein binding sites were identified within the first 200 base pairs of the promoter by DNase I footprinting, and these elements contribute to CPT-Ialpha gene expression. Our studies demonstrate that CPT-Ialpha is a TATA-less gene which utilizes NF-Y and Sp proteins to drive basal expression. (+info)Peroxisome proliferator-activated receptor alpha mediates the adaptive response to fasting. (7/672)
Prolonged deprivation of food induces dramatic changes in mammalian metabolism, including the release of large amounts of fatty acids from the adipose tissue, followed by their oxidation in the liver. The nuclear receptor known as peroxisome proliferator-activated receptor alpha (PPARalpha) was found to play a role in regulating mitochondrial and peroxisomal fatty acid oxidation, suggesting that PPARalpha may be involved in the transcriptional response to fasting. To investigate this possibility, PPARalpha-null mice were subjected to a high fat diet or to fasting, and their responses were compared with those of wild-type mice. PPARalpha-null mice chronically fed a high fat diet showed a massive accumulation of lipid in their livers. A similar phenotype was noted in PPARalpha-null mice fasted for 24 hours, who also displayed severe hypoglycemia, hypoketonemia, hypothermia, and elevated plasma free fatty acid levels, indicating a dramatic inhibition of fatty acid uptake and oxidation. It is shown that to accommodate the increased requirement for hepatic fatty acid oxidation, PPARalpha mRNA is induced during fasting in wild-type mice. The data indicate that PPARalpha plays a pivotal role in the management of energy stores during fasting. By modulating gene expression, PPARalpha stimulates hepatic fatty acid oxidation to supply substrates that can be metabolized by other tissues. (+info)Elevated body fat in rats by the dietary nitric oxide synthase inhibitor, L-N omega nitroarginine. (8/672)
The influence of the dietary nitric oxide (NO) synthase inhibitor, L-N omega nitroarginine (L-NNA) on body fat was examined in rats. In experiment 1, all rats were fed with the same amount of diet with or without 0.02% L-NNA for 8 wk. L-NNA intake caused elevations in serum triglyceride and body fat, and reduction in serum nitrate (a metabolite of nitric oxide). The activity of hepatic carnitine palmitoyltransferase was reduced by L-NNA. In experiment 2, rats were fed for 8 wk with the same amount of diets with or without 0.02% L-NNA supplemented or not with 4% L-arginine. The elevation in body fat, and the reductions in serum nitrate and in the activity of hepatic carnitine palmitoyltransferase by L-NNA were all suppressed by supplemental L-arginine. The results suggest that lower NO generation elevated not only serum triglyceride, but also body fat by reduced fatty acid oxidation. (+info)
Carnitine palmitoyltransferase II deficiency - Wikipedia
Carnitine palmitoyltransferase II deficiency - Genetics Home Reference
CPT2Z - Overview: Carnitine Palmitoyltransferase II Deficiency, Full Gene Analysis
Carnitine palmitoyltransferase II deficiency
Purification, characterization and partial amino acid sequences of carnitine palmitoyl-transferase from human liver<...
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Changes in carnitine palmitoyltransferase-i mrna abundance produced by hyperthyroidism and hypothyroidism parallel changes in...
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Conviction Chapter 9: Home again, a house, m.d. fanfic | FanFiction
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Interaction of malonyl-CoA and related compounds with mitochondria from different rat tissues. Relationship between ligand...
Carnitine and Carnitine Palmitoyltransferase in Fatty Acid Oxidation and Ketosis - PubMed
l-carnitine acyltransferase in intact peroxisomes is inhibited by malonyl-CoA | Biochemical Journal
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Downregulating carnitine palmitoyl transferase 1 affects disease progression in the SOD1 G93A mouse model of ALS -...
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CPT1B Gene - GeneCards | CPT1B Protein | CPT1B Antibody
CPT1B Gene - GeneCards | CPT1B Protein | CPT1B Antibody
Reflex Nutrition Clarinol CLA 90 Caps Weight Management
Search
anti-CPT2 (aa406-418) antibody, Internal | GeneTex
anti-CPT2 antibody [N1C1] | GeneTex
KAKEN - Research Projects | Brain type CPT II Deficiency ; CPT II analysis in the brain and its gene mutations. (KAKENHI...
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Smoke signals | MLB.com
Combined partial deficiency of muscle carnitine palmitoyltransferase and carnitine with autosomal dominant inheritance. |...
The effect of 5-aminoimidazole-4-carboxamide ribonucleoside (AICAR) on fatty acid oxidation in hepatocytes isolated from...
Human Metabolome Database: Showing metabocard for Dodecanedioic acid (HMDB0000623)
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Palmitoyltransferase ZDHHC2 | definition of palmitoyltransferase ZDHHC2 by Medical dictionary
Peroxisome proliferator-activated receptor alpha is an androgen-responsive gene in human prostate and is highly expressed in...
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Evidence for dissociation of gluconeogenesis stimulated by non-esterified fatty acids and changes in fructose 2,6-bisphosphate...
Carnitine
Carnitine in bacterial physiology and metabolism | Microbiology Society
Buy L Carnitine | L Carnitine Supplements | NetNutri
Carnitine Supplements Australia - eVitamins.com
Product information, instructions, health benefits, and usage for Carnitine
Carnitine Benefits & Side Effects - eVitamins.com
ZDHHC17 zinc finger DHHC-type palmitoyltransferase 17 [Homo sapiens (human)] - Gene - NCBI
Liquid Carnitine - CampusProtein.com
LCLT: Carnitine Tatrate - Prom-IN • Protein.sk
Physician resistance to EMR and why CPT should be replaced
پروفیسر صاحب کے 12 سال کی مہروں کی تکلیف بغیر دوائی بغیر آپریشن کیسے ٹھیک ہوئی آپ بھی جانئے CPT - CPT Pakistan
CPT® BHAT® Files | CCO Community
Transferase
Carnitine palmitoyltransferase II deficiency (also known as CPT-II deficiency) leads to an excess long chain fatty acids, as ... Weiser, Thomas (1993). "Carnitine Palmitoyltransferase II Deficiency". NIH. Retrieved 22 November 2013. "Galactosemia". ... "Carnitine plamitoyltransferase I deficiency". Genetics Home Reference. National Institute of Health. Retrieved 4 November 2013 ... Treatment generally includes dietary modifications and carnitine supplements. Galactosemia results from an inability to process ...
Palmitoylcarnitine
Carnitine O-palmitoyltransferase breaks it down into palmitoyl CoA. Carnitine O-palmitoyltransferase v t e Metabolism portal. ... Palmitoylcarnitine is an ester derivative of carnitine involved in the metabolism of fatty acids. ...
CZIB
"Homo sapiens carnitine palmitoyltransferase 2 (CPT2), mRNA - Nucleotide - NCBI". Ncbi.nlm.nih.gov. 2013-03-25. Retrieved 2013- ... This gene works with carnitine palmitoyltransferase I, and the encoded protein oxidizes long-chain fatty acids in the ... A portion of the 3' UTR of C1orf123 has 100% identity with the mRNA for Homo sapiens carnitine palmityoyltransferase 2 is a ...
CPT2
... may refer to: Carnitine palmitoyltransferase II, an important metabolic enzyme. Carnitine palmitoyltransferase II ...
Hormone-sensitive lipase
Crabtree B, Newsholme EA (December 1972). "The activities of lipases and carnitine palmitoyltransferase in muscles from ...
Chromosome 1
Carnitine palmitoyltransferase II deficiency. *Charcot-Marie-Tooth disease, types 1 and 2 ...
PPRC1
"Upstream stimulatory factor represses the induction of carnitine palmitoyltransferase-Ibeta expression by PGC-1". J. Biol. Chem ...
CHKB (gene)
1997). "Structural features of the gene encoding human muscle type carnitine palmitoyltransferase I". FEBS Lett. 409 (3): 401-6 ... "Structural features of the gene encoding human muscle type carnitine palmitoyltransferase I". FEBS Lett. 409 (3): 401-6. doi: ... of equivocal messages containing both regions of choline/ethanolamine kinase and muscle type carnitine palmitoyltransferase I ...
Myokymia
Blepharospasm Carnitine palmitoyltransferase II deficiency Fasciculation Myoclonic jerk (myoclonus) Superior oblique myokymia ...
Fasciculation
Blepharospasm Carnitine palmitoyltransferase II deficiency Myokymia Blackman G, Cherfi Y, Morrin H, et al. (2019). "The ...
USF2
"Upstream stimulatory factor represses the induction of carnitine palmitoyltransferase-Ibeta expression by PGC-1". The Journal ... "Upstream stimulatory factor represses the induction of carnitine palmitoyltransferase-Ibeta expression by PGC-1". The Journal ...
Randle cycle
Malonyl-CoA inhibits the carnitine palmitoyltransferase (CPT) that controls the entry and oxidation of LCFA. The glucose- ...
Protein kinase, AMP-activated, alpha 1
"Evidence that the AMP-activated protein kinase stimulates rat liver carnitine palmitoyltransferase I by phosphorylating ...
Ketogenesis
Carnitine palmitoyltransferase is also regulated by PPARα, which can affect fatty acid transportation into the mitochondria. ... Malonyl-CoA reduces the activity of carnitine palmitoyltransferase I, an enzyme that brings fatty acids into the mitochondria ...
AMP-activated protein kinase
Acetyl-CoA carboxylase (ACC) converts acetyl-CoA to malonyl-CoA, an inhibitor of carnitine palmitoyltransferase 1 (CPT-1). CPT- ...
Fatty acid oxidation inhibitors
... carnitine palmitoyl transferase) converts fatty acyl-CoA to fatty acyl-carnitine. Carnitine biosynthesis inhibitor: Mildronate ... December 2006). "Mildronate, an inhibitor of carnitine biosynthesis, induces an increase in gamma-butyrobetaine contents and ...
Triheptanoin
... such as pyruvate carboxylase deficiency and carnitine palmitoyltransferase II deficiency. It also appears to increase the ...
Etomoxir
2018). "Identifying off-target effects of etomoxir reveals that carnitine palmitoyltransferase I is essential for cancer cell ... Etomoxir, or 2[6(4-chlorophenoxy)hexyl]oxirane-2-carboxylate, is an irreversible inhibitor of carnitine palmitoyltransferase-1 ... This prevents the formation of acyl carnitines, a step that is necessary for the transport of fatty acyl chains from the ...
Kromosomang 1 (tao), ang malayang ensiklopedya
CPT2: carnitine palmitoyltransferase II. *DBT: dihydrolipoamide branched chain transacylase E2. *DIRAS3: DIRAS family, GTP- ...
Tumor metabolome
The carnitine palmitoyltransferase enzymes that regulate the β-oxidation of fatty acids may have a key role in determining some ...
Kromosomang 11 (tao), ang malayang ensiklopedya
carnitine palmitoyltransferase I deficiency. *Charcot-Marie-Tooth disease. *Charcot-Marie-Tooth disease, type 4 ...
Diagnosis (American TV series)
Angel receives a call from the Physician with a complete result and a solid diagnosis of Carnitine Palmitoyltransferase II ...
CROT (gene)
2002). "Structural model of a malonyl-CoA-binding site of carnitine octanoyltransferase and carnitine palmitoyltransferase I: ... Peroxisomal carnitine O-octanoyltransferase is an enzyme that in humans is encoded by the CROT gene. Carnitine ... is a carnitine acyltransferase that catalyzes the reversible transfer of fatty acyl groups between CoA and carnitine. This ... "Entrez Gene: CROT carnitine O-octanoyltransferase". Human CROT genome location and CROT gene details page in the UCSC Genome ...
Acyl-CoA
Transport of acyl-CoA into the mitochondria requires carnitine palmitoyltransferase 1 (CPT1), which converts acyl-CoA into ...
Fatty acid metabolism
... as a carnitine is shuttled outside. Acyl-carnitine is converted back to acyl-CoA by carnitine palmitoyltransferase II, located ... Acyl-CoA is transferred to the hydroxyl group of carnitine by carnitine palmitoyltransferase I, located on the cytosolic faces ... Acyl-carnitine is shuttled inside by a carnitine-acylcarnitine translocase, ... The liberated carnitine is shuttled back to the cytosol, as an acyl-CoA is shuttled into the matrix. Beta oxidation, in the ...
Palmitoyl-CoA hydrolase
... carnitine palmitoyltransferase and glycerophosphate acyltransferase compared to peroxisomal beta-oxidation and palmitoyl-CoA ...
Vitamin B12 deficiency
... carnitine palmitoyl transferase), leading to lipogenesis and insulin resistance. Further to this, reduced vitamin B12 ...
Meldonium
The co-A is then exchanged with carnitine (via the enzyme carnitine palmitoyltransferase I) to produce a fatty acid-carnitine ... Once inside, carnitine is liberated (catalysed by the enzyme carnitine palmitoyltransferase II) and transported back outside so ... Meldonium has also been shown by NMR to bind to carnitine acetyltransferase. Carnitine acetyltransferase belongs to a family of ... Carnitine is mainly absorbed from the diet, but can be formed through biosynthesis. To produce carnitine, lysine residues are ...
NRIP1
... including uncoupling protein 1 and carnitine palmitoyltransferase 1b. Estrogen-related receptor alpha (ERRa) can activate ...
HADHB
Carnitine-acylcarnitine translocase. *Carnitine palmitoyltransferase II. Beta oxidation. General. *Acyl CoA dehydrogenase ( ...
MT-TI
Carnitine palmitoyltransferase I. *Long-chain-fatty-acid-CoA ligase. tryptophan metabolism. *Kynureninase ...
Histone acetyltransferase
palmitoyltransferases: Carnitine O-palmitoyltransferase *CPT1. *CPT2. *Serine C-palmitoyltransferase *SPTLC1. *SPTLC2. *other: ...
Citrate synthase
Carnitine palmitoyltransferase I. *Long-chain-fatty-acid-CoA ligase. tryptophan metabolism. *Kynureninase ...
Acetyl-CoA carboxylase
Carnitine-acylcarnitine translocase. *Carnitine palmitoyltransferase II. Beta oxidation. General. *Acyl CoA dehydrogenase ( ... block for new fatty acids and can inhibit the transfer of the fatty acyl group from acyl CoA to carnitine with carnitine ...
3-oxoacyl-(acyl-carrier-protein) reductase
Carnitine-acylcarnitine translocase. *Carnitine palmitoyltransferase II. Beta oxidation. General. *Acyl CoA dehydrogenase ( ...
Succinate dehydrogenase
Carnitine palmitoyltransferase I. *Long-chain-fatty-acid-CoA ligase. tryptophan metabolism. *Kynureninase ...
Respiratory complex I
Carnitine palmitoyltransferase I. *Long-chain-fatty-acid-CoA ligase. tryptophan metabolism. *Kynureninase ...
ACADM
Carnitine-acylcarnitine translocase. *Carnitine palmitoyltransferase II. Beta oxidation. General. *Acyl CoA dehydrogenase ( ... carnitine biosynthetic process. • medium-chain fatty acid catabolic process. • carnitine metabolic process, CoA-linked. • fatty ...
Creatine kinase
Carnitine palmitoyltransferase I. *Long-chain-fatty-acid-CoA ligase. tryptophan metabolism. *Kynureninase ...
Diglyceride acyltransferase
palmitoyltransferases: Carnitine O-palmitoyltransferase *CPT1. *CPT2. *Serine C-palmitoyltransferase *SPTLC1. *SPTLC2. *other: ...
Fasciculation
Carnitine palmitoyltransferase II deficiency. *Myokymia. ReferencesEdit. *^ Blexrud MD, Windebank AJ, Daube JR (1993). "Long- ...
Fatty acid synthase
Carnitine-acylcarnitine translocase. *Carnitine palmitoyltransferase II. Beta oxidation. General. *Acyl CoA dehydrogenase ( ...
Humant kromosom 1
CPT2 carnitine palmitoyltransferase 2 [ Homo sapiens (human) ]». Gene. NCBI. 24. juni 2017. Henta 26. juni 2017.. ... carnitine palmitoyltransferase II deficiency». Genetics Home Reference. U.S. National Library of Medicine. Juni 2014. Henta 28 ... 1994). «Assignment of the human carnitine palmitoyltransferase II gene (CPT1) to chromosome 1p32». Genomics 24 (1): 195-7. PMID ...
Transfer RNA
Carnitine palmitoyltransferase I. *Long-chain-fatty-acid-CoA ligase. tryptophan metabolism. *Kynureninase ...
List of diseases (C)
... syndrome Carnitine palmitoyl transferase deficiency Carnitine palmitoyltransferase I deficiency Carnitine palmitoyltransferase ... II deficiency Carnitine transporter deficiency Carnitine-acylcarnitine translocase deficiency Carnosinase deficiency ...
Coenzyme A
... carnitine palmitoyl transferase, and cholesterol esterification) Propionyl-CoA Butyryl-CoA Myristoyl-CoA Crotonyl-CoA ...
Carnitine O-octanoyltransferase
... easily solubilized mitochondrial carnitine palmitoyltransferase, and overt mitochondrial carnitine palmitoyltransferase. As of ... Is overt carnitine palmitoyltransferase of liver peroxisomal carnitine octanoyltransferase?". Biochem. J. 249 (1): 231-7. doi: ... "Purification and properties of carnitine octanoyltransferase and carnitine palmitoyltransferase from rat liver". J. Biochem. ... Other names in common use include medium-chain/long-chain carnitine acyltransferase, carnitine medium-chain acyltransferase, ...
Palmitoyl-CoA
Carnitine <-> Palmitoyl-Carnitine + CoA-SH}}} This transesterification reaction is catalyzed by carnitine palmitoyl transferase ... Carnitine ⟷ Palmitoyl − Carnitine + CoA − SH {\displaystyle {\ce {Palmitoyl-CoA + ... Palmitoyl-Carnitine may translocate across the membrane, and once on matrix side, the reaction proceeds in reverse as CoA-SH is ... Unattached carnitine is then shuttled back to the cytosolic side of mitochondrial membrane. Once inside the mitochondrial ...
ACACB
... is thought to control fatty acid oxidation by means of the ability of malonyl-CoA to inhibit carnitine palmitoyltransferase I, ...
Carnitine palmitoyltransferase II deficiency: MedlinePlus Genetics
Carnitine palmitoyltransferase II (CPT II) deficiency is a condition that prevents the body from using certain fats for energy ... CARNITINE PALMITOYLTRANSFERASE II DEFICIENCY, LETHAL NEONATAL. *CARNITINE PALMITOYLTRANSFERASE II DEFICIENCY, MYOPATHIC, STRESS ... Carnitine palmitoyltransferase II deficiency *Genetic Testing Registry: Carnitine palmitoyltransferase II deficiency, infantile ... medlineplus.gov/genetics/condition/carnitine-palmitoyltransferase-ii-deficiency/ Carnitine palmitoyltransferase II deficiency. ...
Carnitine O-palmitoyltransferase - Wikipedia
Carnitine O-palmitoyltransferase (also called carnitine palmitoyltransferase) is a mitochondrial transferase enzyme (EC 2.3. ... associated with Carnitine palmitoyltransferase I deficiency CPT1B CPT1C CPT2 - associated with carnitine palmitoyltransferase ... in Pfam UMich Orientation of Proteins in Membranes protein/pdbid-2h4t Carnitine+O-Palmitoyltransferase at the US National ... A related transferase is carnitine acyltransferase. Palmitoylcarnitine Palmitoyl CoA There are four different forms of CPT in ...
Carnitine palmitoyltransferase I - Wikipedia
Carnitine palmitoyltransferase I (CPT1) also known as carnitine acyltransferase I, CPTI, CAT1, CoA:carnitine acyl transferase ( ... Carnitine palmitoyltransferase I is the first component and rate-limiting step of the carnitine palmitoyltransferase system, ... "Structural model of carnitine palmitoyltransferase I based on the carnitine acetyltransferase crystal". The Biochemical Journal ... The carnitine palmitoyltransferase system is an essential step in the beta-oxidation of long chain fatty acids. This transfer ...
Carnitine palmitoyltransferase 2 deficiency, infantile
Carnitine Palmitoyltransferase 1A Deficiency
... Review. In: GeneReviews® [Internet]. Seattle (WA): University of Washington, ... Clinical characteristics: Carnitine palmitoyltransferase 1A (CPT1A) deficiency is a disorder of long-chain fatty acid oxidation ... detection of biallelic pathogenic variants in CPT1A on molecular genetic testing or diminished carnitine palmitoyltransferase 1 ... C6-C10 fatty acids do not require the carnitine shuttle for entry into the mitochondrion). Prevention of secondary ...
Fenofibrate Therapy in Carnitine Palmitoyl Transferase Type 2 Deficiency
... improve beta-oxidation of fatty acids and to reduce episodes of rhabdomyolysis in patients with carnitine palmitoyltransferase ... Fenofibrate Therapy in Carnitine Palmitoyl Transferase Type 2 Deficiency. I. Hamilton-Craig. ,1 M. Yudi. ,2 L. Johnson. ,3 and ... Carnitine palmitoyl transferase type 2 (CPT2) deficiency is a rare autosomal recessive disorder of mitochondrial fatty acid ... E. Sigauke, D. Rakheja, K. Kitson, and M. J. Bennett, "Carnitine palmitoyltransferase II deficiency: a clinical, biochemical, ...
Carnitine palmitoyltransferase II deficiency
... Classification & external resources ... Carnitine palmitoyltransferase I deficiency This article incorporates public domain text from The U.S. National Library of ... Carnitine palmitoyltransferase II deficiency is a condition that prevents the body from converting certain fats called long- ... Mutations in the CPT2 gene cause carnitine palmitoyltransferase II deficiency, leading to the production of a defective version ...
Carnitine palmitoyltransferase 1B isoform a variant - Homo sapiens (Human)
Carnitine palmitoyltransferase 1B isoform a variantImported. ,p>Information which has been imported from another database using ... tr,Q53FV7,Q53FV7_HUMAN Carnitine palmitoyltransferase 1B isoform a variant (Fragment) OS=Homo sapiens OX=9606 PE=2 SV=1 ... Belongs to the carnitine/choline acetyltransferase family.UniRule annotation. ,p>Information which has been generated by the ...
Cpt1c MGI Mouse Gene Detail - MGI:2446526 - carnitine palmitoyltransferase 1c
The mitochondrial carnitine palmitoyltransferase system. From concept to molecular analysis
... the carnitine palmitoyltransferase (CPT) system has since come to be recognized as a pivotal component of fuel homeostasis. ... The mitochondrial carnitine palmitoyltransferase system. From concept to molecular analysis Eur J Biochem. 1997 Feb 15;244(1):1 ... the carnitine palmitoyltransferase (CPT) system has since come to be recognized as a pivotal component of fuel homeostasis. ...
Carnitine Palmitoyltransferase Ii Deficiency: Disease Bioinformatics: Novus Biologicals
Learn more about Carnitine Palmitoyltransferase Ii Deficiency from related diseases, pathways, genes and PTMs with the Novus ... Carnitine Palmitoyltransferase Ii Deficiency: Disease Bioinformatics. Research of Carnitine Palmitoyltransferase Ii Deficiency ... Carnitine Palmitoyltransferase Ii Deficiency is also known as Carnitine Palmitoyl Transferase 2 Deficiency. ... Explore more on Carnitine Palmitoyltransferase Ii Deficiency below! For more information on how to use Laverne, please read the ...
RCSB PDB - 4EP9: CRYSTAL STRUCTURE OF RAT CARNITINE PALMITOYLTRANSFERASE 2 IN COMPLEX WITH CoA-site inhibitor
Carnitine O-palmitoyltransferase 2, mitochondrial. A. 653. Rattus norvegicus. Mutation(s): 0 Gene Names: Cpt2, Cpt-2. EC: 2.3. ... Carnitine palmitoyl transferase 2 (CPT-2) is a key enzyme in the mitochondrial fatty acid metabolism. The active site is ... Carnitine palmitoyl transferase 2 (CPT-2) is a key enzyme in the mitochondrial fatty acid metabolism. The active site is ... CRYSTAL STRUCTURE OF RAT CARNITINE PALMITOYLTRANSFERASE 2 IN COMPLEX WITH CoA-site inhibitor. *DOI: 10.2210/pdb4EP9/pdb ...
CPT2Z - Overview: Carnitine Palmitoyltransferase II Deficiency, Full Gene Analysis
Carrier screening in cases where there is a family history of carnitine palmitoyltransferase II deficiency, but disease-causing ... 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 ...
Carnitine palmitoyltransferase-1b deficiency aggravates pressure overload-induced cardiac hypertrophy caused by lipotoxicity. ...
Carnitine palmitoyltransferase-1 (CPT1) is a rate-limiting step of mitochondrial β-oxidation by controlling the mitochondrial ... Carnitine palmitoyltransferase-1b deficiency aggravates pressure overload-induced cardiac hypertrophy caused by lipotoxicity.. ... Carnitine Palmitoyltransferase-1b (CPT1b) Deficiency Aggravates Pressure-Overload-Induced Cardiac Hypertrophy due to ... Carnitine Palmitoyltransferase-1b (CPT1b) Deficiency Aggravates Pressure-Overload-Induced Cardiac Hypertrophy due to ...
AID 49046 - Inhibitory activity against rat-liver mitochondrial Carnitine palmitoyl transferase II - PubChem
Carnitine O-Palmitoyltransferase | Semantic Scholar
CARNITINE PALMITOYLTRANSFERASE II DEFICIENCY, LATE-ONSET. Carnitine Palmitoyltransferase II. Carnitine palmitoyl transferase 1A ... Carnitine palmitoyltransferase 2/Citrate synthase:CRto:Pt:RBC:Qn. Carnitine palmitoyltransferase 2:CCnt:Pt:Amnio fld cells:Qn. ... Carnitine O-Palmitoyltransferase. Known as: Palmitoyltransferase, Carnitine, Acyltransferase, Palmitylcarnitine, O- ... Palmitoyltransferase, Carnitine Expand. An enzyme that catalyzes reversibly the conversion of palmitoyl-CoA to ...
Carnitine Palmitoyltransferase and Transport of Fatty Acids | Springer for Research & Development
... carnitine palmitoyltransferase (hexadecanoyl-CoA:carnitine O-hexadecanoyltransferase, EC 2.3.1.23), and its role in the... ... Carnitine and carnitine palmitoyltransferase in metabolic studies, in:Carnitine Biosynthesis, Metabolism, and Functions ( R. A ... Carnitine palmitoyltransferase II deficiency with normal carnitine palmitoyltransferase I in skeletal muscle and leukocytes, J ... Comparison of properties of carnitine palmitoyltransferase I with those of carnitine palmitoyltransferase II, and preparation ...
Prolonged Inhibition of Muscle Carnitine Palmitoyltransferase-1 Promotes Intramyocellular Lipid Accumulation and Insulin...
CPT, carnitine palmitoyltransferase; EMCL, extramyocellular lipid; FFA, free fatty acid; HMRS, 1H magnetic resonance ... Lopaschuk GD, Wall SR, Olley PM, Davies NJ: Etomoxir, a carnitine palmitoyltransferase I inhibitor, protects hearts from fatty ... Prolonged Inhibition of Muscle Carnitine Palmitoyltransferase-1 Promotes Intramyocellular Lipid Accumulation and Insulin ... Prolonged Inhibition of Muscle Carnitine Palmitoyltransferase-1 Promotes Intramyocellular Lipid Accumulation and Insulin ...
Molecules | Free Full-Text | Resveratrol-Induced Changes in MicroRNA Expression in Primary Human Fibroblasts Harboring...
Carnitine palmitoyltransferase-2 (CPT2) is a mitochondrial enzyme involved in long-chain fatty acid entry into mitochondria for ... Carnitine palmitoyltransferase-2 (CPT2) is a mitochondrial enzyme involved in long-chain fatty acid entry into mitochondria for ... Resveratrol-Induced Changes in MicroRNA Expression in Primary Human Fibroblasts Harboring Carnitine-Palmitoyl Transferase-2 ... "Resveratrol-Induced Changes in MicroRNA Expression in Primary Human Fibroblasts Harboring Carnitine-Palmitoyl Transferase-2 ...
RCSB PDB - 4EYW: Crystal structure of rat carnitine palmitoyltransferase 2 in complex with 1-[(R)-2-(3,4-Dihydro-1H...
Carnitine O-palmitoyltransferase 2, mitochondrial. A, B. 634. Rattus norvegicus. Mutation(s): 0 Gene Names: Cpt2, Cpt-2. EC: ... Carnitine palmitoyl transferase 2 (CPT-2) is a key enzyme in the mitochondrial fatty acid metabolism. The active site is ... Carnitine palmitoyl transferase 2 (CPT-2) is a key enzyme in the mitochondrial fatty acid metabolism. The active site is ... Crystal structure of rat carnitine palmitoyltransferase 2 in complex with 1-[(R)-2-(3,4-Dihydro-1H-isoquinoline-2-carbonyl)- ...
Intracellular in vitro probe acylcarnitine assay for identifying deficiencies of carnitine transporter and carnitine...
... disorders are caused by defects in one of the FAO enzymes that regulates cellular uptake of fatty acids and free carnitine. An ... However, the diagnosis of primary carnitine deficiency (PCD) or carnitine palmitoyltransferase-1 (CPT1) deficiency using the ... Extracellular acylcarnitine (AC) profiling detects carnitine palmitoyltransferase-2, carnitine acylcarnitine translocase, and ... in vitro probe acylcarnitine assay for identifying deficiencies of carnitine transporter and carnitine palmitoyltransferase-1. ...
Human Metabolome Database: Showing Protein Carnitine O-palmitoyltransferase 1, liver isoform (HMDBP00309)
Structural model of carnitine palmitoyltransferase I based on the carnitine acetyltransferase crystal. Biochem J. 2004 May 1; ... Showing Protein Carnitine O-palmitoyltransferase 1, liver isoform (HMDBP00309). IdentificationBiological propertiesGene ... IJlst L, Mandel H, Oostheim W, Ruiter JP, Gutman A, Wanders RJ: Molecular basis of hepatic carnitine palmitoyltransferase I ... Stoler JM, Sabry MA, Hanley C, Hoppel CL, Shih VE: Successful long-term treatment of hepatic carnitine palmitoyltransferase I ...
Overexpression of Carnitine Palmitoyltransferase-1 in Skeletal Muscle Is Sufficient to Enhance Fatty Acid Oxidation and Improve...
Carnitine palmitoyltransferase 1 (CPT1) is a mitochondrial transmembrane enzyme thought to be rate limiting for long-chain ... Overexpression of Carnitine Palmitoyltransferase-1 in Skeletal Muscle Is Sufficient to Enhance Fatty Acid Oxidation and Improve ... Overexpression of Carnitine Palmitoyltransferase-1 in Skeletal Muscle Is Sufficient to Enhance Fatty Acid Oxidation and Improve ... Overexpression of Carnitine Palmitoyltransferase-1 in Skeletal Muscle Is Sufficient to Enhance Fatty Acid Oxidation and Improve ...
Combined partial deficiency of muscle carnitine palmitoyltransferase and carnitine with autosomal dominant inheritance. |...
Combined partial deficiency of muscle carnitine palmitoyltransferase and carnitine with autosomal dominant inheritance. ... Combined partial deficiency of muscle carnitine palmitoyltransferase and carnitine with autosomal dominant inheritance. ... glycolysis and normal glycogen content but combined partial deficiency of muscle carnitine palmitoyltransferase and carnitine ... Plasma concentration of ketone bodies and acid soluble acyl-carnitine increased normally with prolonged fasting. The ...
Differential regulation in the heart of mitochondrial carnitine palmitoyltransferase-I muscle and liver isoforms
PROTECTIVE EFFECTS OF PEROXISOME PROLIFERATOR-ACTIVATED RECEPTOR (PPAR)-ß ACTIVATION ON LIPID-INDUCED ENDOTHELIAL DYSFUNCTION...
... carnitine palmitoyl transferase (CPT)-1.. Acetylcholine-induced endothelium-dependent relaxation in mouse thoracic aortic (MTA ... ß ACTIVATION ON LIPID-INDUCED ENDOTHELIAL DYSFUNCTION via CARNITINE PALMITOYL TRANSFERASE-1 UPREGULATION ... ß ACTIVATION ON LIPID-INDUCED ENDOTHELIAL DYSFUNCTION via CARNITINE PALMITOYL TRANSFERASE-1 UPREGULATION ...
Carnitine palmitoyl transferase 1 deficiency | Genetic and Rare Diseases Information Center (GARD) - an NCATS...
Her doctor said that she had SCOT deficiency OR carnitine palmitoyl transferase 1 deficiency. Now she is normal. Will this ... Will medical crises due to either carnitine palmitoyl transferase 1 deficiency or SCOT deficiency happen again? ... Will medical crises due to either carnitine palmitoyl transferase 1 deficiency or SCOT deficiency happen again? ...
Carnitine palmitoyltransferase | definition of carnitine palmitoyltransferase by Medical dictionary
What is carnitine palmitoyltransferase? Meaning of carnitine palmitoyltransferase medical term. What does carnitine ... Looking for online definition of carnitine palmitoyltransferase in the Medical Dictionary? carnitine palmitoyltransferase ... and carnitine palmitoyltransferase (CPT II) in the inner mitochondrial membrane (carnitine O-palmitoyltransferase EC 2.. ... Acetyl-L-Carnitine supplementation reverses the age-related decline in carnitine palmitoyltransferase 1 (cpt1) activity in ...
KAKEN - Research Projects | Molecular Pathology of Systemic Carnitine Palmitoyltransferase II Deficiency (KAKENHI-PROJECT...
Carnitine palmitoyltransferase (CPT) II deficiency results in two different clinical forms, one with systemic and the other ... CARNITINE PALMITOYLTRANSFERASE II / GENE ANALYSIS / MISSENSE MUTATION / TRANSFECTION / GENE POLYMORPHISM / 遺伝子導入. ... form of carnitine palmitoyltransferase II deficiency Human Genetics. 98. 116-118 (1996). *. Description. 「研究成果報告書概要(欧文)」より ... hepaticform of carnitine palmitoyltransferase II deficiency Human Genetics. 98. 116-118 (1996). *. Description. 「研究成果報
Selleck Chemicals Blog-Identifying off-target effects of etomoxir reveals that carnitine palmitoyltransferase I is essential...
Etomoxir sodium salt is an irreversible inhibitor of carnitine palmitoyltransferase-1 (CPT-1) on the outer face of the inner ... Here we show that when FAO was reduced approximately 90% by pharmacological inhibition of carnitine palmitoyltransferase I ( ... Identifying off-target effects of etomoxir reveals that carnitine palmitoyltransferase I is essential for cancer cell ... Identifying off-target effects of etomoxir reveals that carnitine palmitoyltransferase I is essential for cancer cell ...
CPT1ACPT1CPT2MitochondriaFacing Carnitine palmitoyltransferase I deficLong-chainTranslocaseSkeletal musclePatient with carnitine palmitoyltGeneCPT1CGenesAcyltransferaseAbstractCharacterizationAcylcarnitineProteinCPTIAssociation of carnitineInhibitorFatty acidMetabolismPalmitoyl transferase 1 deficiencyFingerprintForm of carnitineDiagnosis of carnitineInhibition of carnitineMutationsMitochondrionBeta-oxidation1.21AssayMuscleCPT1bAcetyltransferaseAcyl carnitineCoenzymeLethal neonatal formCatalyticRegulationGeneticHuman liver
CPT1A17
- Palmitoylcarnitine Palmitoyl CoA There are four different forms of CPT in humans: CPT1A - associated with Carnitine palmitoyltransferase I deficiency CPT1B CPT1C CPT2 - associated with carnitine palmitoyltransferase II deficiency Fatty acid degradation § Transport into the mitochondrial matrix Jogl G, Tong L (January 2003). (wikipedia.org)
- 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)
- Carnitine palmitoyltransferase 1A (CPT1A) deficiency is a disorder of long-chain fatty acid oxidation. (nih.gov)
- The diagnosis of CPT1A is established in a proband by the detection of biallelic pathogenic variants in CPT1A on molecular genetic testing or diminished carnitine palmitoyltransferase 1 (CPT 1) enzyme activity on cultured skin fibroblasts when molecular genetic testing is not definitive. (nih.gov)
- These pathways complement our catalog of research reagents for the study of Carnitine Palmitoyltransferase Ii Deficiency including antibodies and ELISA kits against CPT2, CPT1A, ETFDH, CHPT1, CHKA. (novusbio.com)
- Here, we show that carnitine palmitoyltransferase 1A (CPT1A) has lysine succinyltransferase (LSTase) activity in vivo and in vitro. (elsevier.com)
- Importantly, mutation of CPT1A Gly710 (G710E) selectively inactivated carnitine palmitoyltransferase (CPTase) activity but not the LSTase activity that decreased enolase activity in cells and promoted cell proliferation under glutamine depletion. (elsevier.com)
- Mutation of CPT1A Gly710 (G710E) selectively inactivates canonical carnitine palmitoyltransferase (CPTase) activity but not LSTase activity. (elsevier.com)
- The "CPT1A" form is associated with carnitine palmitoyltransferase I deficiency. (colegiomontpellier.net)
- Carnitine Palmitoyltransferase 1A Deficiency Bennett MJ - 2016 PMID: 20301700: Organization of the human liver carnitine palmitoyltransferase 1 gene ( CPT1A) and identification of novel mutations in hypoketotic hypoglycaemia. (colegiomontpellier.net)
- Diseases associated with CPT1A include Carnitine Palmitoyltransferase I Deficiency and Carnitine Palmitoyltransferase Ii Deficiency, Infantile . (genecards.org)
- T) variant of carnitine palmitoyltransferase 1 (CPT1A) is frequent in some aboriginal populations and may be associated with increased infant deaths. (cdc.gov)
- CPT1A sequencing is a molecular test used to identify variants in the gene associated with Carnitine Palmitoyltransferase IA Deficiency. (ggc.org)
- Prevalence of carnitine palmitoyltransferase 1A (CPT1A). (ubc.ca)
- carnitine palmitoyltransferase 1A , or CPT1A for short. (adn.com)
- Specifically, oxidation of fatty acids (FAO) was reduced in these cells, which linked to reduced carnitine palmitoyltransferase 1a (Cpt1a), an essential enzyme for carnitine shuttle. (frontiersin.org)
- L-Carnitine treatment augmented FAO but attenuated CSE-induced apoptosis by upregulating Cpt1a. (frontiersin.org)
CPT111
- 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 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)
- 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-1 (CPT1) is a rate-limiting step of mitochondrial β-oxidation by controlling the mitochondrial uptake of long-chain acyl-CoAs. (nih.gov)
- However, the diagnosis of primary carnitine deficiency (PCD) or carnitine palmitoyltransferase-1 (CPT1) deficiency using the conventional IVP assay has been hampered by the presence of a large amount of free carnitine (C0), a key molecule deregulated by these deficiencies. (biomedsearch.com)
- We hypothesized that skeletal muscle-specific overexpression of the muscle isoform of carnitine palmitoyltransferase 1 (CPT1), the enzyme that controls the entry of long-chain fatty acyl CoA into mitochondria, would enhance rates of fatty acid oxidation and improve insulin action in muscle in high-fat diet insulin-resistant rats. (diabetesjournals.org)
- We investigated whether the age-related impairment of cardiac fatty acid catabolism occurs, at least partially, through diminished levels of L-carnitine, which would adversely affect carnitine palmitoyltransferase 1 (CPT1), the rate-limiting enzyme for fatty acyl-CoA uptake into mitochondria for beta]-oxidation. (thefreedictionary.com)
- PubMed:14711372 ] Mitochondrial carnitine palmitoyltransferase‐1 (CPT1) is a target for oxidative inactivation in human cells. (colegiomontpellier.net)
- Fatty acid β-oxidation is the major metabolic pathway for generating adenosine triphosphate (ATP) in the kidneys, which is governed by carnitine palmitoyltransferase 1 (CPT1). (elsevier.com)
- Carnitine palmitoyltransferase I (CPT1) is the main rate-limiting step in β-oxidation and represents a key regulatory site controlling flux through this pathway by virtue of its inhibition by the fatty acid synthesis precursor malonyl-CoA ( McGarry and Mannaerts, 1977 ). (frontiersin.org)
- Dysregulated lipid metabolism and mitochondrial dysfunction are hypothesized to play a key role in MS. Carnitine Palmitoyl Transferase 1 (CPT1) is a rate-limiting enzyme for beta-oxidation of fatty acids in mitochondria. (au.dk)
CPT216
- Mutations in the CPT2 gene reduce the activity of carnitine palmitoyltransferase 2. (medlineplus.gov)
- Bezafibrate therapy has been shown to improve beta-oxidation of fatty acids and to reduce episodes of rhabdomyolysis in patients with carnitine palmitoyltransferase type-2 (CPT2) deficiency. (hindawi.com)
- Carnitine palmitoyl transferase type 2 (CPT2) deficiency is a rare autosomal recessive disorder of mitochondrial fatty acid oxidation [ 1 ]. (hindawi.com)
- Bezafibrate therapy resulted in 60-284% improvement in skeletal muscle palmitoyl L-carnitine oxidation levels, 20-93% increase in skeletal muscle CPT2 mRNA, and full correction of the initial defective fatty acid oxidation in myoblasts in vitro . (hindawi.com)
- Mutations in the CPT2 gene cause carnitine palmitoyltransferase II deficiency, leading to the production of a defective version of an enzyme called carnitine palmitoyltransferase II. (bionity.com)
- Carnitine palmitoyltransferase-2 ( CPT2 ) is a mitochondrial enzyme involved in long-chain fatty acid entry into mitochondria for their β-oxidation and energy production. (mdpi.com)
- Carnitine Palmitoyltransferase II (CPT2) deficiency (myopathic form) is a rare, progressive adult metabolic myopathy caused by a genetic insufficiency in CPT2 enzyme production. (thefreedictionary.com)
- If there is a mutation to the CPT2 gene, there is not enough CPII made to remove the carnitine. (diseaseinfosearch.org)
- Identification of 16 new disease-causing mutations in the CPT2 gene resulting in carnitine palmitoyltransferase II deficiency. (colegiomontpellier.net)
- Carnitine palmitoyltransferase 2 (CPT2) deficiency is a condition that prevents the body from using certain fats for energy, particularly during periods without food (fasting). (colegiomontpellier.net)
- Mutations in the CPT2 gene cause carnitine palmitoyltransferase II deficiency. (unt.edu)
- Mutations in the CPT2 gene lead to the production of a defective version of an enzyme called carnitine palmitoyltransferase II. (unt.edu)
- Should the Human Carnitine Palmitoyltransferase 2, Mitochondrial (CPT2) ELISA Kit is proven to show malperformance, you will receive a refund or a free replacement. (orbitalbiosciences.com)
- Description: A sandwich quantitative ELISA assay kit for detection of Human Carnitine Palmitoyltransferase 2, Mitochondrial (CPT2) in samples from tissue homogenates, cell lysates or other biological fluids. (orbitalbiosciences.com)
- Description: This is Double-antibody Sandwich Enzyme-linked immunosorbent assay for detection of Rat Carnitine Palmitoyltransferase 2, Mitochondrial (CPT2) in Tissue homogenates, cell lysates and other biological fluids. (orbitalbiosciences.com)
- Description: Enzyme-linked immunosorbent assay based on the Double-antibody Sandwich method for detection of Rat Carnitine Palmitoyltransferase 2, Mitochondrial (CPT2) in samples from Tissue homogenates, cell lysates and other biological fluids with no significant corss-reactivity with analogues from other species. (orbitalbiosciences.com)
Mitochondria11
- A group of fats called long-chain fatty acids must be attached to a substance known as carnitine to enter mitochondria. (medlineplus.gov)
- Once these fatty acids are inside mitochondria, carnitine palmitoyltransferase 2 removes the carnitine and prepares them for fatty acid oxidation. (medlineplus.gov)
- This "preparation" allows for subsequent movement of the acyl carnitine from the cytosol into the intermembrane space of mitochondria. (wikipedia.org)
- This enzyme attaches long chain fatty acids to carnitine so they can be moved into the mitochondria. (diseaseinfosearch.org)
- Fatty acids are then moved into the mitochondria (the part of cell where energy is made) using carnitine. (diseaseinfosearch.org)
- Once inside the mitochondria, CPII takes the carnitine off, so the fatty acids can then be used for energy. (diseaseinfosearch.org)
- Diagram of long-chain fatty acid (LCFA) import into the mitochondria by the carnitine shuttle. (colegiomontpellier.net)
- The protein encoded by this gene, a member of the carnitine/choline acetyltransferase family, is the rate-controlling enzyme of the long-chain fatty acid beta-oxidation pathway in muscle mitochondria. (nih.gov)
- Metabolomics revealed several acylcarnitines and their upstream regulatory gene, carnitine palmitoyltransferase 1C (CPT1C) involved in fatty acid β-oxidation in mitochondria, were strikingly decreased in senescent PANC-1 cells. (aging-us.com)
- Transport of fatty acids into the mitochondria for β-oxidation is mediated by a carnitine carrier system. (frontiersin.org)
- Carnitine palmitoyltransferase 1C (CPT1C), an enzyme located in the outer mitochondria membrane, has a crucial role in fatty acid transport and oxidation. (elsevier.com)
Facing Carnitine palmitoyltransferase I defic1
- considering the fact that basically the smallest fraction of data facing Carnitine palmitoyltransferase I deficiency is listed in se's, equivalent to www.google.com or others, a non-systematic method of net study should be not just time eating, but in addition incomplete. (manuel-monroy.com)
Long-chain12
- Without enough of this enzyme, carnitine is not removed from long-chain fatty acids. (medlineplus.gov)
- The carnitine palmitoyltransferase system is an essential step in the beta-oxidation of long chain fatty acids. (wikipedia.org)
- Carnitine palmitoyltransferase II deficiency is a condition that prevents the body from converting certain fats called long-chain fatty acids into energy, particularly during periods without food. (bionity.com)
- First conceptualized as a mechanism for the mitochondrial transport of long-chain fatty acids in the early 1960s, the carnitine palmitoyltransferase (CPT) system has since come to be recognized as a pivotal component of fuel homeostasis. (nih.gov)
- 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)
- 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)
- an enzyme that catalyzes the transfer between coenzyme A and carnitine of long-chain fatty acids. (thefreedictionary.com)
- Carnitine palmitoyltransferase II (CPT II) deficiency is an autosomal inherited metabolic disorder in which the β-oxidation of the long chain fatty acids is defective. (elitmed.hu)
- Long-chain fatty acids in all tissues and medium-chain fatty acids in most tissues (an important exception is the liver) are esterified to coenzyme A in the cytosol and cannot enter the mitochondrial matrix to undergo beta oxidation without the action of carnitine and 3 proteins (carnitine palmitoyltransferase 1, carnitine acylcarnitine translocase, and carnitine palmitoyltransferase 2). (colegiomontpellier.net)
- Together with carnitine, this enzyme helps release long-chain fatty acids from a particular transport enzyme. (unt.edu)
- If carnitine palmitoyltransferase II is defective, long-chain fatty acids from food and fats stored in the body cannot be broken down and processed. (unt.edu)
- The mitochondrial oxidation of long-chain fatty acids is initiated by the sequential action of carnitine palmitoyltransferase I (which is located in the outer membrane and is detergent-labile) and carnitine palmitoyltransferase II (which is located in the inner membrane and is detergent-stable), together with a carnitine-acylcarnitine translocase. (genecards.org)
Translocase3
- A translocase then shuttles the acyl carnitine across the inner mitochondrial membrane where it is converted back into palmitoyl-CoA. (wikipedia.org)
- Extracellular acylcarnitine (AC) profiling detects carnitine palmitoyltransferase-2, carnitine acylcarnitine translocase, and other FAO deficiencies. (biomedsearch.com)
- carnitine/acylcarnitine translocase (CACT) within the inner mitochondrial membrane, and carnitine palmitoyltransferase (CPT II) in the inner mitochondrial membrane (carnitine O-palmitoyltransferase EC 2. (thefreedictionary.com)
Skeletal muscle2
- Two additional groups (low-fat + etoxomir and lard + etoxomir) consumed diets containing 0.01% of the carnitine palmitoyltransferase-1 inhibitor, R-etomoxir, which produced chronic blockade of enzyme activity in liver and skeletal muscle. (diabetesjournals.org)
- Yeung, Oscar W.H. Carnitine palmitoyltransferase and carnitine octanoyltransferase activities in liver, kidney cortex, adipocyte, lactating mamary gland, skeletal muscle and heart. (colegiomontpellier.net)
Patient with carnitine palmitoylt1
- Severe rhabdomyolysis with hypoglycemia in an adult patient with carnitine palmitoyltransferase II deficiency. (colegiomontpellier.net)
Gene8
- This gene provides instructions for making an enzyme called carnitine palmitoyltransferase 2. (medlineplus.gov)
- Aires V, Delmas D, Djouadi F, Bastin J, Cherkaoui-Malki M, Latruffe N. Resveratrol-Induced Changes in MicroRNA Expression in Primary Human Fibroblasts Harboring Carnitine-Palmitoyl Transferase-2 Gene Mutation, Leading to Fatty Acid Oxidation Deficiency. (mdpi.com)
- Two novel gene mutations(Glu174→Lys,Phe383→Tyr)causing the 'hepatic'form of carnitine palmitoyltransferase II deficiency' Human Genetics. (nii.ac.jp)
- This gene provides the code to make the protein, carnitine palmitoyltransferase II (CPII). (diseaseinfosearch.org)
- CPT1B (Carnitine Palmitoyltransferase 1B) is a Protein Coding gene. (genecards.org)
- GO annotations related to this gene include transferase activity, transferring acyl groups and carnitine O-palmitoyltransferase activity . (genecards.org)
- Gene Ontology (GO) annotations related to this gene include identical protein binding and carnitine O-palmitoyltransferase activity . (genecards.org)
- Transgenic mice carrying the human heart muscle carnitine palmitoyltransferase I (M-CPTI) gene fused to a CAT reporter gene were generated to study the regulation of M-CPTI gene expression. (elsevier.com)
CPT1C1
- Here we report that carnitine palmitoyltransferase 1C (CPT1C), a brain-specific metabolic enzyme, may participate in metabolic transformation. (core.ac.uk)
Genes1
- What genes are related to carnitine palmitoyltransferase II deficiency? (unt.edu)
Acyltransferase3
- A related transferase is carnitine acyltransferase. (wikipedia.org)
- A primary biochemical action of malonyl-CoA is the inhibition of the acyltransferase activity of carnitine palmitoyltransferase-1 (CPT-1). (physiology.org)
- 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)
Abstract2
- abstract = "Carnitine palmitoyltransferase (CPT) is a mitochondrial-inner-membrane enzyme, with activities located on both the outer and inner sides of the membrane. (elsevier.com)
- abstract = "Exposure of rat liver mitochondrial membranes to octyl glucoside, Triton X-100, or Tween 20 solubilized an active and tetradecylglycidyl-CoA (TG-CoA)-insensitive carnitine palmitoyltransferase (presumed to be carnitine palmitoyltransferase II). (elsevier.com)
Characterization3
- Characterization of hepatic carnitine palmitoyltransferase. (elsevier.com)
- Fingerprint Dive into the research topics of 'Characterization of hepatic carnitine palmitoyltransferase. (elsevier.com)
- Characterization of the mitochondrial carnitine palmitoyltransferase enzyme system. (elsevier.com)
Acylcarnitine1
- Intracellular in vitro probe acylcarnitine assay for identifying deficiencies of carnitine transporter and carnitine palmitoyltransferase-1. (biomedsearch.com)
Protein3
- Tandem mass spectrometry identified protein abundance differences per mitochondrial mass in insulin resistance, including lower abundance of complex I subunits and enzymes involved in the oxidation of branched-chain amino acids (BCAA) and fatty acids (e.g., carnitine palmitoyltransferase 1B). (forskningsdatabasen.dk)
- These and other studies on the effects of detergents on the mitochondrial [3H]TG-CoA binding protein provide further support for the model of carnitine palmitoyltransferase proposed in the preceding paper. (elsevier.com)
- While carnitine palmitoyltransferase I is a different protein in liver and muscle, it seems likely that both tissues share the same transferase II. (elsevier.com)
CPTI2
- Using a real time confocal microscopy assay, it was found that the carnitine palmitoyltransferase I (CPTI) inhibitor, etomoxir, reduced mitochondrial fusion dynamics in a time-dependent manner. (frontiersin.org)
- The predicted amino acid sequence showed the highest homology (62.6%) with that of carnitine palmitoyltransferase I (CPTI) from rat liver. (tokushima-u.ac.jp)
Association of carnitine1
- 3) association of carnitine palmitoyltransferase I with a membrane component(s) is necessary for catalytic activity. (elsevier.com)
Inhibitor1
- Etomoxir sodium salt is an irreversible inhibitor of carnitine palmitoyltransferase-1 (CPT-1) on the outer face of the inner mitochondrial membrane. (selleckchem.com)
Fatty acid10
- Researched pathways related to Carnitine Palmitoyltransferase Ii Deficiency include Fatty Acid Oxidation, Lipid Storage, Transport, Lipid Oxidation, Reverse Transcription. (novusbio.com)
- Carnitine palmitoyl transferase 2 (CPT-2) is a key enzyme in the mitochondrial fatty acid metabolism. (rcsb.org)
- Mitochondrial fatty acid oxidation (FAO) disorders are caused by defects in one of the FAO enzymes that regulates cellular uptake of fatty acids and free carnitine. (biomedsearch.com)
- Carnitine palmitoyltransferase-I (CPT-I) plays a crucial role in regulating cardiac fatty acid oxidation which provides the primary source of energy for cardiac muscle contraction. (ovid.com)
- For example, preliminary evidence suggests that carnitine palmitoyltransferase type 1A deficiency, a fatty acid disorder highly prevalent in AN and other indigenous circumpolar populations, might contribute to infant mortality (8). (thefreedictionary.com)
- A key regulatory point in the control of fatty acid (FA) oxidation is thought to be transport of FAs across the mitochondrial membrane by carnitine palmitoyltransferase I (CPT I). To investigate the role of CPT I in FA metabolism, we used in vivo electrotransfer (IVE) to locally overexpress CPT I in muscle of rodents. (garvan.org.au)
- Carnitine palmitoyltransferase (CPT) deficiencies are common disorders of mitochondrial fatty acid oxidation. (colegiomontpellier.net)
- CPT I is the key enzyme in the carnitine-dependent transport across the mitochondrial inner membrane and its deficiency results in a decreased rate of fatty acid beta-oxidation. (genecards.org)
- β-blockers have been proposed to inhibit fatty acid oxidation by decreasing the activity of the enzyme carnitine palmitoyltransferase-1 (CPT-1). (ubc.ca)
- 19 Inactivation of ACC reduces the synthesis of malonyl-CoA, which in turn activates carnitine palmitoyltransferase-1A (CPT-1A) and increases fatty acid oxidation. (rsc.org)
Metabolism4
- Carnitine O-palmitoyltransferase (also called carnitine palmitoyltransferase) is a mitochondrial transferase enzyme (EC 2.3.1.21) involved in the metabolism of palmitoylcarnitine into palmitoyl-CoA. (wikipedia.org)
- Research of Carnitine Palmitoyltransferase Ii Deficiency has been linked to Inborn Errors Of Metabolism, Myalgia, Rhabdomyolysis, Hypoglycemia, Weakness. (novusbio.com)
- Carnitine--metabolism and functions. (semanticscholar.org)
- Carnitine Palmitoyl Transferase 1 - a Potential Target to Restore Dysregulated Metabolism in Neurodegenerative Diseases? (aau.dk)
Palmitoyl transferase 1 deficiency3
- Her doctor said that she had SCOT deficiency OR carnitine palmitoyl transferase 1 deficiency. (nih.gov)
- Will medical crises due to either carnitine palmitoyl transferase 1 deficiency or SCOT deficiency happen again? (nih.gov)
- Following organizations serve the condition "Carnitine Palmitoyl Transferase 1 Deficiency" for support, advocacy or research. (diseaseinfosearch.org)
Fingerprint1
- Fingerprint Dive into the research topics of 'Are there two forms of carnitine palmitoyltransferase in muscle? (elsevier.com)
Form of carnitine1
- The myopathic form is the most frequently seen and least severe form of carnitine palmitoyltransferase II deficiency. (bionity.com)
Diagnosis of carnitine1
- A carnitine palmitoyl transferase test is a blood test that measures the amount of carnitine in the blood and is used in the diagnosis of carnitine palmitoyl transferase deficiency. (nicklauschildrens.org)
Inhibition of carnitine1
- Pharmacological inhibition of carnitine palmitoyl transfe. (au.dk)
Mutations2
- Novel mutations associated with carnitine palmitoyltransferase II deficiency. (colegiomontpellier.net)
- Genetic mutations causative for McArdle disease, carnitine palmitoyl transferase deficiency 2, myoadenylate deaminase deficiency, and malignant hyperthermia have all been associated with Exertional rhabdomyolysis. (nih.gov)
Mitochondrion1
- adults need a high-carbohydrate, low-fat diet to provide a constant supply of carbohydrate energy and medium-chain triglycerides to provide approximately one third of total calories (C6-C10 fatty acids do not require the carnitine shuttle for entry into the mitochondrion). (nih.gov)
Beta-oxidation4
- We investigated whether PPARβ activation can prevent palmitate-induced endothelial dysfunction using ex vivo and in vitro models, focusing on the rate-limiting enzyme for beta-oxidation, carnitine palmitoyl transferase (CPT)-1. (bmj.com)
- Carnitine palmitoyl transferase I and the control of myocardial beta-oxidation flux. (semanticscholar.org)
- article{Eaton2001CarnitinePT, title={Carnitine palmitoyl transferase I and the control of myocardial beta-oxidation flux. (semanticscholar.org)
- Carnitine palmitoyltransferase I is assumed to be rate limiting for beta-oxidation in all tissues. (semanticscholar.org)
1.211
- Information on EC 2.3.1.21 - carnitine O-palmitoyltransferase. (colegiomontpellier.net)
Assay1
- Serum samples collected on 7th, 15th and 22nd days and carnitine palmitoyl transferase assay done. (jyoungpharm.org)
Muscle5
- Combined partial deficiency of muscle carnitine palmitoyltransferase and carnitine with autosomal dominant inheritance. (bmj.com)
- The biochemical evaluation of the muscle tissue revealed intact anaerobic glycolysis and normal glycogen content but combined partial deficiency of muscle carnitine palmitoyltransferase and carnitine in both cases. (bmj.com)
- Carnitine palmitoyl transferase (CPT) deficiency is a rare condition that causes muscle weakness and other symptoms. (umassmemorial.org)
- Are there two forms of carnitine palmitoyltransferase in muscle? (elsevier.com)
- Because we had found whole testis from adult rats to be much richer in the messenger RNA for the muscle (M) than for the liver (L) form of mitochondrial carnitine palmitoyltransferase I (CPT I), we sought to determine which cell type(s) accounts for this expression pattern and how it might relate to reproductive function. (elsevier.com)
CPT1b1
- CPT1B heterozygous variants of G320D and S427C among control subjects showed significantly higher levels of total and free carnitine in the blood compared to acute myocardial infarction patients. (nih.gov)
Acetyltransferase1
- 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)
Acyl carnitine1
- Plasma concentration of ketone bodies and acid soluble acyl-carnitine increased normally with prolonged fasting. (bmj.com)
Coenzyme1
- 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)
Lethal neonatal form3
- The term 'prevalence' of Carnitine palmitoyl transferase II deficiency, lethal neonatal form usually refers to the estimated population of people who are managing Carnitine palmitoyl transferase II deficiency, lethal neonatal form at any given time. (rightdiagnosis.com)
- The term 'incidence' of Carnitine palmitoyl transferase II deficiency, lethal neonatal form refers to the annual diagnosis rate, or the number of new cases of Carnitine palmitoyl transferase II deficiency, lethal neonatal form diagnosed each year. (rightdiagnosis.com)
- Carnitine palmitoyltransferase II deficiency (CPTII), lethal neonatal form is a rare genetic condition. (diseaseinfosearch.org)
Catalytic1
- Comparison of the catalytic activities of three isozymes of carnitine palmitoyltransferase 1 expressed in COS7 cells. (semanticscholar.org)
Regulation1
- To study the regulation of carnitine palmitoyltransferase-I by thyroid hormone, a cDNA was obtained by PCR amplification of DNA obtained by reverse transcription of rat liver RNA. (unthsc.edu)
Genetic2
- Carnitine palmitoyltransferase I deficiency (CPT-IA) is a rare genetic condition. (diseaseinfosearch.org)
- 42 Carnitine Palmitoyltransferase I Deficiency How Is Genetic Testing Done? (manuel-monroy.com)
Human liver1
- Carnitine palmitoyl-transferase has been extracted with 0.5% Tween-20 from human liver homogenatc and purified to homogeneity. (elsevier.com)