Carnitine Acyltransferases
Carnitine O-Acetyltransferase
Carnitine
Acyltransferases
Carnitine O-Palmitoyltransferase
1-Acylglycerophosphocholine O-Acyltransferase
Glycerol-3-Phosphate O-Acyltransferase
Acyl Coenzyme A
Diacylglycerol O-Acyltransferase
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)
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Carnitine O-acetyltransferase
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 ... van der Leij FR, Huijkman NC, Boomsma C, Kuipers JR, Bartelds B (2000). "Genomics of the human carnitine acyltransferase genes ... Carnitine palmitoyltransferase I is the first component and rate-limiting step of the carnitine palmitoyltransferase system, ...
Carnitine palmitoyltransferase II
van der Leij FR, Huijkman NC, Boomsma C, Kuipers JR, Bartelds B (2000). "Genomics of the human carnitine acyltransferase genes ... Carnitine O-palmitoyltransferase 2, mitochondrial is an enzyme that in humans is encoded by the CPT2 gene. Carnitine ... CPT2 together with carnitine palmitoyltransferase I oxidizes long-chain fatty acids in the mitochondria. Defects in this gene ... Verderio E, Cavadini P, Montermini L, Wang H, Lamantea E, Finocchiaro G, DiDonato S, Gellera C, Taroni F (1995). "Carnitine ...
Carnitine O-octanoyltransferase
Other names in common use include medium-chain/long-chain carnitine acyltransferase, carnitine medium-chain acyltransferase, ... Farrell SO, Fiol CJ, Reddy JK, Bieber LL (1984). "Properties of purified carnitine acyltransferases of mouse liver peroxisomes ... Is overt carnitine palmitoyltransferase of liver peroxisomal carnitine octanoyltransferase?". Biochem. J. 249 (1): 231-7. doi: ... easily solubilized mitochondrial carnitine palmitoyltransferase, and overt mitochondrial carnitine palmitoyltransferase. As of ...
Carnitine O-palmitoyltransferase
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 ...
Carnitine palmitoyltransferase II deficiency
CPT II shares structural elements with other members of the carnitine acyltransferase protein family. The crystal structure of ... Carnitine O-palmitoyltransferase Carnitine palmitoyltransferase I deficiency Fasciculation Myokymia Primary carnitine ... 1993). "Comparison of the active sites of the purified carnitine acyltransferases from peroxisomes and mitochondria by using a ... The "carnitine shuttle" is composed of three enzymes that utilize carnitine to facilitate the import of hydrophobic long-chain ...
CROT (gene)
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: ...
Fatty acyl-CoA esters
There carnitine acyltransferase II reverses the process, producing fatty acyl-CoA and carnitine. This shuttle mechanism is ... They are converted to fatty acyl carnitine by carnitine acyltransferase I, an enzyme of the inner leaflet of the outer ... Fatty acyl carnitine is then transported by an antiport in exchange for free carnitine to the inner surface of the inner ...
Carnitine
This reaction takes place in the mitochondrial matrix and is catalyzed by carnitine acyltransferase 2 (also called carnitine ... L-Carnitine, acetyl-l-carnitine, and propionyl-l-carnitine are available in dietary supplement pills or powders, with a daily ... Carnitine exists as one of two stereoisomers (the two enantiomers d-carnitine (S-(+)-) and l-carnitine (R-(−)-)). Both are ... Two types of carnitine deficiency states exist. Primary carnitine deficiency is a genetic disorder of the cellular carnitine- ...
Palmitoyl-CoA hydrolase
... carnitine palmitoyltransferase and glycerophosphate acyltransferase compared to peroxisomal β-oxidation and palmitoyl-CoA ...
Malonyl-CoA
... inhibits fatty acids from associating with carnitine by regulating the enzyme carnitine acyltransferase, thereby ...
Liang Tong
... carnitine acyltransferase, AMP-activated protein kinase, and others. These enzymes are important targets for drug discovery ...
Blighia sapida
... carnitine and carnitine acyltransferases I and II, reducing their bioavailability and consequently inhibiting beta oxidation of ...
Acetyl-CoA carboxylase
... new fatty acids and can inhibit the transfer of the fatty acyl group from acyl CoA to carnitine with carnitine acyltransferase ...
List of MeSH codes (D08)
... carnitine acyltransferases MeSH D08.811.913.050.350.170 - carnitine O-acetyltransferase MeSH D08.811.913.050.350.200 - ... diacylglycerol o-acyltransferase MeSH D08.811.913.050.425 - glycerol-3-phosphate O-acyltransferase MeSH D08.811.913.050.600 - ... phosphatidylcholine-sterol O-acyltransferase MeSH D08.811.913.050.646 - retinol O-fatty-acyltransferase MeSH D08.811.913.050. ... 1-acylglycerol-3-phosphate O-acyltransferase MeSH D08.811.913.050.175 - 1-acylglycerophosphocholine O-acyltransferase MeSH ...
Chromosome 11
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 ...
PDIA3
... but not as a protease or a carnitine acyltransferase". Archives of Biochemistry and Biophysics. 323 (2): 397-403. doi:10.1006/ ...
Transporter Classification Database
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- ...
List of EC numbers (EC 2)
... 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- ... dolichol O-acyltransferase EC 2.3.1.124: Already listed as EC 2.3.1.20 EC 2.3.1.125: 1-alkyl-2-acetylglycerol O-acyltransferase ... 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- ...
Fatty acid degradation
The liberated carnitine returns to the cytosol. It is important to note that carnitine acyltransferase I undergoes allosteric ... This occurs via a series of similar steps: Acyl CoA is conjugated to carnitine by carnitine acyltransferase I ( ... is converted to acyl CoA by carnitine acyltransferase (palmitoyltransferase) II located on the inner mitochondrial membrane. ... I located on the outer mitochondrial membrane Acyl carnitine is shuttled inside by a translocase Acyl carnitine (such as ...
SLC22A5
"Downregulation of carnitine acyltransferases and organic cation transporter OCTN2 in mononuclear cells in healthy elderly and ... an organic cation/carnitine transporter, lead to deficient cellular carnitine uptake in primary carnitine deficiency". Human ... characterized by impaired carnitine transport, urinary carnitine wasting, low serum carnitine levels, reduced intracellular ... "Primary systemic carnitine deficiency is caused by mutations in a gene encoding sodium ion-dependent carnitine transporter". ...
Beta oxidation
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 ...
Tafazzin
However, no formal assessment of the utility of carnitine and arginine supplementation has been published, and its uses have ... Neuwald AF (August 1997). "Barth syndrome may be due to an acyltransferase deficiency". Current Biology. 7 (8): R465-6. doi: ... Metabolic deficiencies have been treated by oral arginine and carnitine supplementation, which has been shown to ameliorate ... CL remodeling in mammals requires additional enzymes, such as monolysocardiolipin acyltransferase (MLCLAT), acyl-CoA: ...
Methamphetamine
SLC22A3 is an extraneuronal monoamine transporter that is present in astrocytes, and SLC22A5 is a high-affinity carnitine ... CYP2D6, dopamine β-hydroxylase, flavin-containing monooxygenase 3, butyrate-CoA ligase, and glycine N-acyltransferase are the ... "Substrate/Product". glycine N-acyltransferase. BRENDA. Technische Universität Braunschweig. "Compound Summary". p- ...
Acyl-CoA
... : diacylglycerol acyltransferase (DGAT) plays an important role in energy metabolism on account of key enzyme in ... Transport of acyl-CoA into the mitochondria requires carnitine palmitoyltransferase 1 (CPT1), which converts acyl-CoA into ... Yu, Yi‐Hao; Ginsberg, Henry (8 July 2009). "The role of acyl‐CoA:diacylglycerol acyltransferase (DGAT) in energy metabolism". ...
Chromosome 5
Lysophosphatidylcholine acyltransferase 1 LYRM7: encoding protein LYR motif containing 7 LYSMD3: LysM and putative ... carboxylase deficiency Myelodysplastic syndrome Netherton syndrome Nicotine dependency Parkinson's disease Primary carnitine ...
Glibenclamide
It works by inhibiting the enzyme Carnityl Acyl Transferase I (CAT-I) indirectly which is present in the mitochondria. This ... "More direct evidence for a malonyl-CoA-carnitine palmitoyltransferase I interaction as a key event in pancreatic beta-cell ... "Glibenclamide inhibits islet carnitine palmitoyltransferase 1 activity, leading to PKC-dependent insulin exocytosis". American ...
Fatty acid metabolism
Acyl-carnitine is shuttled inside by a carnitine-acylcarnitine translocase, as a carnitine is shuttled outside. Acyl-carnitine ... Enzymes, acyltransferases and transacylases, incorporate fatty acids in phospholipids, triacylglycerols, etc. by transferring ... Acyl-CoA is transferred to the hydroxyl group of carnitine by carnitine palmitoyltransferase I, located on the cytosolic faces ... The liberated carnitine is shuttled back to the cytosol, as an acyl-CoA is shuttled into the mitochondrial matrix. Beta ...
Amphetamine
Rytting E, Audus KL (January 2005). "Novel organic cation transporter 2-mediated carnitine uptake in placental choriocarcinoma ... and glycine N-acyltransferase (GLYAT) are the enzymes known to metabolize amphetamine or its metabolites in humans. Amphetamine ... and SLC22A5 is a high-affinity carnitine transporter. Amphetamine is known to strongly induce cocaine- and amphetamine- ... the role of glycine N-acyltransferase, and factors that influence interindividual variation". Expert Opinion on Drug Metabolism ...
2014 Ju-Jitsu World Championships
The 2014 Ju-Jitsu World Championship were the 12th edition of the Ju-Jitsu World Championships, and were held in Paris, France from November 28 to November 30, 2014. 28.11.2014 - Men's and Women's Fighting System, Men's and Women's Jiu-Jitsu (ne-waza), Men's Duo System - Classic 29.11.2014 - Men's and Women's Fighting System, Men's and Women's Jiu-Jitsu (ne-waza), Women's Duo System - Classic 30.11.2014 - Men's Jiu-Jitsu (ne-waza), Mixed Duo System - Classic, Team event Vincent MATCZAK (2014-09-30). "4TH INVITAION TO WORLD CHAMPIONSHIP 2014" (PDF). Retrieved 2019-11-28.[dead link] Online results Official results (PDF) Mixed team event results (PDF) (All articles with dead external links, Articles with dead external links from April 2022, Ju-Jitsu World Championships, 2014 in French sport ...
Inhibition by acetyl-CoA of hepatic carnitine acyltransferase and fatty acid oxidation
Evidence of altered fatty acid metabolism in dogs with naturally occurring valvular heart disease and congestive heart failure ...
CPT1A gene: MedlinePlus Genetics
The CPT1A gene provides instructions for making an enzyme called carnitine palmitoyltransferase 1A, which is found in the liver ... Carnitine acyltransferases and their influence on CoA pools in health and disease. Mol Aspects Med. 2004 Oct-Dec;25(5-6):475-93 ... Carnitine palmitoyltransferase I deficiency. More than 20 mutations in the CPT1A gene have been found to cause carnitine ... Carnitine palmitoyltransferase 1A connects carnitine to long-chain fatty acids so they can cross the inner membrane of ...
PDF] Effects of dietary fatty acid composition on the regulation of carnitine palmitoyltransferase (CPT) I in rainbow trout ...
Effects of dietary fatty acid composition on the regulation of carnitine palmitoyltransferase (CPT) I in rainbow trout ( ... Co-ordinate induction of hepatic mitochondrial and peroxisomal carnitine acyltransferase synthesis by diet and drugs.. *P. ... studies using both diet and drugs to cause enzyme induction suggest that the synthesis of the carnitine acyltransferases ( ... Intertissue regulation of carnitine palmitoyltransferase I (CPTI): mitochondrial membrane properties and gene expression in ...
Frontiers | Calorie Restriction Rescues Mitochondrial Dysfunction in Adck2-Deficient Skeletal Muscle
Acyl-carnitine (C0), (C2), (C16), (C18) measurements in plasma. Data represent the mean ± SD (N = 3). (D) Acetyl-CoA ... protein levels of Acetyl-CoA Acyltransferase 2 (ACAA2), which catalyzes the last step of β-oxidation, were elevated only in ... Adck2+/− mice on ad libitum showed an accumulation of acyl-carnitines C2, C16, and C18 and the corresponding decrease of free ... The β-oxidation of fatty acids assay (BOX) was carried out in the presence of palmitoyl-l-carnitine and malate. Substrates and ...
SCOPe 2.07: Domain d1q6xa2: 1q6x A:402-617
YRC Public Data Repository - Gene Ontology - carnitine O-octanoyltransferase activity
IJMS | Free Full-Text | Role of 3-Hydroxy Fatty Acid-Induced Hepatic Lipotoxicity in Acute Fatty Liver of Pregnancy
Carnitine acyl transferase I converts acyl-CoA into fatty acyl carnitine, which is translocated across the inner mitochondrial ... Carnitine acyl transferase II in the inner mitochondrial membrane catalyzes the formation of fatty acyl-CoA for the initiation ... For instance, a published report linked maternal AFLP with pediatric carnitine palmitoyl transferase I deficiency [88]. A ... and carnitine fatty acyl-CoA translocase and MTP deficiency [20,71]. MTP−/− mice were also reported to develop intrauterine ...
The Role of PPAR
Activation in Liver and Muscle
in carnitine metabolism in mice [37, 38]. No similar indications of PPAR. -induced carnitine synthesis have been described in ... In addition, the expression of the gene encoding the enzyme acetyl-CoA acyltransferase 2 (ACAA2) involved in the final step of ... Furthermore, hepatic carnitine synthesis is enhanced by PPAR. activation in mice [34, 35]. Carnitine is a conditionally ... α-activation results in enhanced carnitine biosynthesis and OCTN2-mediated hepatic carnitine accumulation," Biochimica et ...
Other Transferase Inhibitors | Other Transferases | Tocris Bioscience
DeCS
Carnitine Acyltransferases Entry term(s). Acylcarnitine Translocase Acyltransferases, Carnitine Carnitine Acylcarnitine ... Carnitine acyltransferases Entry term(s):. Acylcarnitine Translocase. Acyltransferases, Carnitine. Carnitine Acetylcarnitine ... Carnitine Acyltransferases - Preferred Concept UI. M0003496. Scope note. Acyltransferases in the inner mitochondrial membrane ... Carnitine Acylcarnitine Translocase. Carnitine Translocase. Carnitine-Acetylcarnitine Translocase. Carnitine-Acylcarnitine ...
QualityCounts.com Newsletter of 10/19/11
... reduced glycerol-3-phosphate acyl transferase-1, and increased carnitine palmitoyltransferase-1 expression, which led to ... The Benefits of Carnitine and DHEA for Fat Metabolism - Life Extension Magazine, 8/02 - Carnitine is an amino acid thats ... However, the mRNA levels of carnitine palmitoyl transferase-1 (CPT-1) and uncoupling protein 2 (UCP2), as well as lipolytic ...
MK MetabolismeLIPID 2 PDF | PDF
The glucose dependence of Akt-transformed cells can be reversed by pharmacologic activation of fatty acid beta-oxidation. -...
Code System Concept
Remarkable metabolic reorganization and altered metabolic requirements in frog metamorphic climax | Frontiers in Zoology | Full...
Three FFAs (Δ18:1, Δ18:2, and Δ16:0) and two acyl-carnitines (Δ16:0-carnitine and Δ18:0-carnitine) decreased and increased in ... 4b). At the transcriptional level, T3-treated tadpoles showed upregulated glycerolipid synthesis (DAG/MAG O-acyltransferase), ... and two aryl-carnitines (Δ18:0-carnitine and Δ10:0-carnitine; active form of FFAs) decreased and increased in content, ... 3c). Other FFAs or acyl-carnitines were unaffected. At the transcriptional level, T3-treated tadpoles showed upregulated ...
Bio2Vec
An increase in the number of peroxisomes is coupled to the initial infection stage and stress response of Botrytis cinerea |...
Peroxisomal carnitine acetyl transferase is required for elaboration of penetration hyphae during plant infection by ... ABCD, ATP-binding cassette, subfamily D; ACAA, Acetyl-CoA acyltransferase; ACOX, Acyl-CoA oxidase; ACSL, Long-chain acyl-CoA ... Functional divergence of diacylglycerol acyltransferases in the unicellular green alga Haematococcus pluvialis. J Exp Bot. 2021 ... of root waxes and can be produced in the aerial cuticle by ectopic expression of a suberin-associated acyltransferase. Plant ...
Dietary effect of pomegranate seed oil rich in 9cis, 11trans, 13cis conjugated linolenic acid on lipid metabolism in obese,...
Phosphatidylcholine-Sterol O-Acyltransferase | Harvard Catalyst Profiles | Harvard Catalyst
Carnitine Acyltransferases. *Citrate (si)-Synthase. *Diacylglycerol O-Acyltransferase. *Glycerol-3-Phosphate O-Acyltransferase ... Phosphatidylcholine-Sterol O-Acyltransferase*Phosphatidylcholine-Sterol O-Acyltransferase. *O-Acyltransferase, ... "Phosphatidylcholine-Sterol O-Acyltransferase" is a descriptor in the National Library of Medicines controlled vocabulary ... In familial lecithin:cholesterol acyltransferase deficiency disease, the absence of the enzyme results in an excess of ...
SMPDB
In mitochondrial oxidation similar but different enzymes are used called carnitine acyltransferase I and II. Difference number ... peroxisomal carnitine acyltransferase, which is needed to move an activated acyl group from outside the peroxisome to inside it ... The enzyme 1-acyl-sn-glycerol-3-phosphate acyltransferase converts LPA into phosphatidic acid (1,2-diacyl-sn-glycerol 3- ... In particular, both pathways make use of acetyl-CoA acyltransferase, 3-hydroxyacyl-CoA dehydrogenase and enoyl-CoA hydratase. ...
l-Carnitine ameliorates congenital myopathy in a tropomyosin 3 de novo mutation transgenic zebrafish | Journal of Biomedical...
Interestingly, l-carnitine treatment on TPM3(E151G) transgenic larvae significantly improves the muscle endurance by restoring ... which can be rescued by l-carnitine treatment, fatty acid metabolism was differentially dysregulated in TPM3(E151G) fish and ... rescued by l-carnitine treatment. These results demonstrate that TPM3(E151G) and TPM3(E151A) exhibit different pathogenicity, ... enhances l-carnitine uptake inside cells. Carnitine acyltransferases are a family of enzymes that catalyze the reversible ...
Tang, Yi<...
"Reversible Product Release and Recapture by a Fungal Polyketide Synthase Using a Carnitine Acyltransferase Domain." Angew. Chem ... Tang, Y., Koppisch, A., Khosla, C. "The Acyltransferase Homolog Found in Initiation Modules of Type II PKS is an Acetyl-ACP ... Xie, X., Meehan, M. J. Xu, W., Dorrestein, P. C., Tang, Y.* "Acyltransferase Mediated Polyketide Release From Fungal ... "Biosynthesis of lovastatin analogs with a broadly specific acyltransferase." Chem. Biol. 2006, 13, 1161-1169. Link ...
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Pesquisa | Prevenção e Controle de Câncer
Increasing fatty acid oxidation remodels the hypothalamic neurometabolome to mitigate stress and inflammation<...
We have shown previously that C75, a stimulator of carnitine palmitoyl transferase-1 (CPT-1) and fatty acid oxidation (FAOx), ... the glycerol-3-phosphate acyltransferase (GPAT) inhibitor FSG67, on FA metabolism, metabolomics profiles, and metabolic stress ... We have shown previously that C75, a stimulator of carnitine palmitoyl transferase-1 (CPT-1) and fatty acid oxidation (FAOx), ... We have shown previously that C75, a stimulator of carnitine palmitoyl transferase-1 (CPT-1) and fatty acid oxidation (FAOx), ...
DeCS 2008 - versión 17 de Marzo de 2008
Pesquisa | Portal Regional da BVS
This occurs enzymatically via carnitine acyltransferases. Specific acylcarnitines accumulate as a result of various organic ... Plasma samples revealed low levels of free carnitine (C0), long-chain acyl-carnitines and total carnitine. In particular, C0 ... To measure total carnitine, samples are spiked with deuterated carnitine (internal standard) and hydrolyzed with potassium ... Abnormal levels of free carnitine, total carnitine, and acylcarnitines in serum can be indicative of a metabolic disorder ...
Glycerol-3-phosphate acyltransferaMitochondrialPalmitoyltransferase 1APhosphatidylcholine-sterol O-acyltrHepatic carnitineCPT1ATranslocasePalmitoyl-carnitineDiacylglycerolMitochondriaEnzyme calledProteinLipoproteinFatty acidsCPT2LIVERDeficiencyAmino acidsConcentrationsGeneRegulationLong-chainHumanMuscleLevelsHighEffect
Glycerol-3-phosphate acyltransfera3
- Second, the mitochondrial outer membrane enzyme glycerol-3-phosphate acyltransferase esterifies an acyl-group to the sn-1 position of sn-glycerol 3-phosphate to form 1-acyl-sn-glycerol 3-phosphate (lysophosphatidic acid or LPA). (smpdb.ca)
- The enzyme 1-acyl-sn-glycerol-3-phosphate acyltransferase converts LPA into phosphatidic acid (1,2-diacyl-sn-glycerol 3-phosphate) by esterifying an acyl-group to the sn-2 position of the glycerol backbone. (smpdb.ca)
- In the present work, we characterized the effects of C75 and another anorexigenic compound, the glycerol-3-phosphate acyltransferase (GPAT) inhibitor FSG67, on FA metabolism, metabolomics profiles, and metabolic stress responses in cultured hypothalamic neurons and hypothalamic neuronal cell lines during lipid excess with palmitate. (elsevier.com)
Mitochondrial4
- At micromolar concentrations, acetyl-CoA inhibited hepatic carnitine acyltransferase activity and mitochondrial fatty acid oxidation. (uab.edu)
- 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. (bvsalud.org)
- L-Carnitine may play an important role in male reproductive health, mainly due to increased mitochondrial fatty acid oxidation, which results in improved motility of epididymal sperm. (supplemena.com)
- L-Carnitine is essential for transporting long-chain fatty acids across the mitochondrial membrane, for subsequent fat breakdown and energy generation. (supplemena.com)
Palmitoyltransferase 1A4
- The CPT1A gene provides instructions for making an enzyme called carnitine palmitoyltransferase 1A, which is found in the liver. (medlineplus.gov)
- Carnitine palmitoyltransferase 1A connects carnitine to long-chain fatty acids so they can cross the inner membrane of mitochondria. (medlineplus.gov)
- Most of these mutations change single protein building blocks (amino acids) within carnitine palmitoyltransferase 1A. (medlineplus.gov)
- Akkaoui M, Cohen I, Esnous C, Lenoir V, Sournac M, Girard J, Prip-Buus C. Modulation of the hepatic malonyl-CoA-carnitine palmitoyltransferase 1A partnership creates a metabolic switch allowing oxidation of de novo fatty acids. (medlineplus.gov)
Phosphatidylcholine-sterol O-acyltr3
- Phosphatidylcholine-Sterol O-Acyltransferase" is a descriptor in the National Library of Medicine's controlled vocabulary thesaurus, MeSH (Medical Subject Headings) . (harvard.edu)
- This graph shows the total number of publications written about "Phosphatidylcholine-Sterol O-Acyltransferase" by people in Harvard Catalyst Profiles by year, and whether "Phosphatidylcholine-Sterol O-Acyltransferase" was a major or minor topic of these publication. (harvard.edu)
- Below are the most recent publications written about "Phosphatidylcholine-Sterol O-Acyltransferase" by people in Profiles. (harvard.edu)
Hepatic carnitine1
- Bennett MJ, Boriack RL, Narayan S, Rutledge SL, Raff ML. Novel mutations in CPT 1A define molecular heterogeneity of hepatic carnitine palmitoyltransferase I deficiency. (medlineplus.gov)
CPT1A1
- More than 20 mutations in the CPT1A gene have been found to cause carnitine palmitoyltransferase I (CPT I) deficiency. (medlineplus.gov)
Translocase1
- Downregulation of carnitine acyl-carnitine translocase by miRNAs 132 and 212 amplifies glucose-stimulated insulin secretion. (duke.edu)
Palmitoyl-carnitine2
- Exogenous LCACs, especially palmitoyl-carnitine and stearoyl-carnitine, inhibited iNKT cell expansion and promoted senescence. (bvsalud.org)
- A nonhydrolyzable ether analog of palmitoyl-carnitine stimulated IS, showing that β-oxidation of palmitoyl-carnitine is not required for its stimulation of IS. (duke.edu)
Diacylglycerol3
- Last, the enzyme diacylglycerol O-acyltransferase synthesizes triacylglycerol from diacylglycerol and a fatty acyl-CoA. (smpdb.ca)
- Diacylglycerol O-Acyltransferase" is a descriptor in the National Library of Medicine's controlled vocabulary thesaurus, MeSH (Medical Subject Headings) . (jefferson.edu)
- Below are the most recent publications written about "Diacylglycerol O-Acyltransferase" by people in Profiles. (jefferson.edu)
Mitochondria4
- A group of fats called long-chain fatty acids cannot enter mitochondria unless they are attached to a substance known as carnitine. (medlineplus.gov)
- Once these fatty acids are inside mitochondria, carnitine is removed and they can be metabolized to produce energy. (medlineplus.gov)
- Interestingly, l -carnitine treatment on TPM3 (E151G) transgenic larvae significantly improves the muscle endurance by restoring the basal respiration and ATP levels in mitochondria. (biomedcentral.com)
- CACT is responsible for transporting long-chain acyl-carnitines into the mitochondria for β-oxidation. (duke.edu)
Enzyme called1
- Essentially, this switch is controlled by an enzyme called Carnitine acyltransferases protein or Crat in short. (thecytoburns.com)
Protein5
- This switch is activated by the energy sensor adenosine monophosphate-activated protein kinase (AMPK), in response to the increase in the AMP/ATP ratio, while carnitine palmitoyl transferase-1 (CPT-1) is also activated by AMPK inhibition of acetyl-CoA carboxylase (ACC). (frontiersin.org)
- Caco2 cell culture and animal experiment confirmed IMZ affected carnitine absorption through the organic cation transporter type-2 (OCTN2) protein encoded by solute carrier family 22A member 5 (SLC22A5) gene in colon. (bvsalud.org)
- These results suggested that maternal IMZ exposure affected carnitine absorption through OCTN2 protein, which led to the decline of anaerobic bacteria and unbalanced intestinal homeostasis. (bvsalud.org)
- Normal protein content but abnormally inhibited enzyme activity in muscle carnitine palmitoyltransferase II deficiency. (dbcls.jp)
- Lethal neonatal and severe late infantile forms of carnitine palmitoyltransferase II deficiency associated with compound heterozygosity for different protein truncation mutations. (dbcls.jp)
Lipoprotein2
- Structural analysis of lecithin:cholesterol acyltransferase bound to high density lipoprotein particles. (harvard.edu)
- Expression of the PPAR-α and γ, lipoprotein lipase (LPL), hormone sensitive lipase (HSL), carnitine palmitoyl acyltransferase-1 (CPT-1), insulin receptor, GLUT-4 and adipokines were assessed in epidydimal fat. (wordpress.com)
Fatty acids1
- Without enough of this enzyme, carnitine is not attached to long-chain fatty acids. (medlineplus.gov)
CPT21
- CPT2 gene mutations resulting in lethal neonatal or severe infantile carnitine palmitoyltransferase II deficiency. (dbcls.jp)
LIVER1
- Addition of recombinant PTE-2 to incubations containing isolated mouse liver peroxisomes strongly inhibited bile acid-CoA:amino acid N-acyltransferase activity, suggesting that this thioesterase can interfere with CoASH-dependent pathways. (rhea-db.org)
Deficiency25
- In familial lecithin:cholesterol acyltransferase deficiency disease, the absence of the enzyme results in an excess of unesterified cholesterol in plasma. (harvard.edu)
- Cause of recurrent rhabdomyolysis, carnitine palmitoyltransferase II deficiency and novel pathogenic mutation. (dbcls.jp)
- Exercise, Nutrition, and Supplements in the Muscle Carnitine Palmitoyl-Transferase II Deficiency: New Theoretical Bases for Potential Applications. (dbcls.jp)
- Need for strict clinical management of patients with carnitine palmitoyltransferase II deficiency: Experience with two cases detected by expanded newborn screening. (dbcls.jp)
- Severe rhabdomyolysis in homozygote carnitine palmitoyltransferase II deficiency. (dbcls.jp)
- Recurrent Myalgia since Early Infancy-Misleading Clinical Course in a Child with Carnitine Palmitoyltransferase-II Deficiency. (dbcls.jp)
- An ignored cause of red urine in children: rhabdomyolysis due to carnitine palmitoyltransferase II (CPT-II) deficiency. (dbcls.jp)
- First Japanese Case of Carnitine Palmitoyltransferase II Deficiency with the Homozygous Point Mutation S113L. (dbcls.jp)
- Missed Newborn Screening Case of Carnitine Palmitoyltransferase-II Deficiency. (dbcls.jp)
- Neonatal Carnitine Palmitoyltransferase II Deficiency: A Lethal Entity. (dbcls.jp)
- Carnitine palmitoyl transferase II deficiency in an adolescent presenting with rhabdomyolysis and acute renal failure. (dbcls.jp)
- Carnitine palmitoyltransferase II (CPT II) deficiency: genotype-phenotype analysis of 50 patients. (dbcls.jp)
- Novel mutations in myopathic form of carnitine palmitoyltransferase II deficiency in a Chinese patient. (dbcls.jp)
- Clinically symptomatic heterozygous carnitine palmitoyltransferase II (CPT II) deficiency. (dbcls.jp)
- Neonatal carnitine palmitoyltransferase II deficiency associated with Dandy-Walker syndrome and sudden death. (dbcls.jp)
- Carnitine palmitoyltransferase II deficiency: successful anaplerotic diet therapy. (dbcls.jp)
- Mutations of carnitine palmitoyltransferase II (CPT II) in Japanese patients with CPT II deficiency. (dbcls.jp)
- Clinical features and new molecular findings in Carnitine Palmitoyltransferase II (CPT II) deficiency. (dbcls.jp)
- Severe infantile type of carnitine palmitoyltransferase II (CPT II) deficiency due to homozygous R503C mutation. (dbcls.jp)
- A novel splice site mutation in neonatal carnitine palmitoyl transferase II deficiency. (dbcls.jp)
- Effect of diet on exercise tolerance in carnitine palmitoyltransferase II deficiency. (dbcls.jp)
- Mutation and biochemical analysis in carnitine palmitoyltransferase type II (CPT II) deficiency. (dbcls.jp)
- Exercise tolerance in carnitine palmitoyltransferase II deficiency with IV and oral glucose. (dbcls.jp)
- Screening for carnitine palmitoyltransferase II deficiency by tandem mass spectrometry. (dbcls.jp)
- Identification of novel mutations in Spanish patients with muscle carnitine palmitoyltransferase II deficiency. (dbcls.jp)
Amino acids1
- l -Carnitine is an amino acid that is widely present in human tissues and can be produced from essential amino acids lysine and methionine. (biomedcentral.com)
Concentrations1
- High-performance liquid chromatography-tandem mass spectrometry (HPLC-MS/MS) and next-generation sequencing (NGS) were used to detect the concentrations of carnitine in the blood spots and for diagnosis. (bvsalud.org)
Gene1
- A variable myopathy associated with heterozygosity for the R503C mutation in the carnitine palmitoyltransferase II gene. (dbcls.jp)
Regulation2
- PDF] Effects of dietary fatty acid composition on the regulation of carnitine palmitoyltransferase (CPT) I in rainbow trout (Oncorhynchus mykiss). (semanticscholar.org)
- These studies establish a link between miRNA-dependent regulation of CACT and fatty acyl-carnitine-mediated regulation of IS. (duke.edu)
Long-chain2
- Plasma samples revealed low levels of free carnitine (C0), long-chain acyl-carnitines and total carnitine. (bvsalud.org)
- The addition of long-chain fatty acyl-carnitines promoted IS from rat insulinoma β-cells (INS-1) as well as primary mouse islets. (duke.edu)
Human2
- Randomized, Placebo-Controlled Phase 2b Study to Evaluate the Safety and Efficacy of Recombinant Human Lecithin Cholesterol Acyltransferase in Acute ST-Segment-Elevation Myocardial Infarction: Results of REAL-TIMI 63B. (harvard.edu)
- Recombinant human lecithin-cholesterol acyltransferase in patients with atherosclerosis: phase 2a primary results and phase 2b design. (harvard.edu)
Muscle1
- With RNAseq transcriptomic analysis of the expression profiling from the muscle specimens, it surprisingly discloses large downregulation of genes involved in pathways of sodium, potassium, and calcium channels, which can be rescued by l -carnitine treatment, fatty acid metabolism was differentially dysregulated in TPM3 (E151G) fish and rescued by l -carnitine treatment. (biomedcentral.com)
Levels3
- Then, we assessed the changes in growth phenotypes, carnitine levels, and gut microbiota in F0, F1 or F2 generations. (bvsalud.org)
- Fertilmas is a capsule-based product for men with general infertility, and has lower levels of L-Carnitine & L-Arginine than EROSMAS. (supplemena.com)
- The resulting reduction in energy metabolism due to lower L-Carnitine levels can be restored by L-Carnitine supplementation. (supplemena.com)
High1
- The Carnipure® product is a high quality L-Carnitine ingredient manufactured by the Swiss company Lonza. (supplemena.com)
Effect1
- Maternal IMZ exposure also had a greater effect on gut microbiota in offspring, especially anaerobic bacteria, which positively correlated with C0 and acyl-carnitines. (bvsalud.org)