• Other names in common use include medium-chain/long-chain carnitine acyltransferase, carnitine medium-chain acyltransferase, easily solubilized mitochondrial carnitine palmitoyltransferase, and overt mitochondrial carnitine palmitoyltransferase. (wikipedia.org)
  • The glucose-dependent inhibition of glucagon secretion relies on pyruvate dehydrogenase and carnitine palmitoyl transferase 1a activity and lowering of mitochondrial fatty acid oxidation by increases in glucose. (diabetesjournals.org)
  • 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)
  • Acyl carnitine crosses the inner mitochondrial membrane and enters the matrix. (prepladder.com)
  • Carnitine palmitoyltransferase II precursor (CPT2) is a nuclear protein which is transported to the mitochondrial inner membrane. (thermofisher.com)
  • The higher level of plasma acetylcarnitine may reflect a stimulated mitochondrial and peroxisomal β-oxidation of fatty acids, as the hepatic activities of peroxisomal acyl-CoA oxidase 1 and mitochondrial carnitine palmitoyltransferase-II were higher in the FPH-fed mice. (biomedcentral.com)
  • Meanwhile, Hypo-EVs enhanced mitochondrial FAO in kidney by restoring the expression of a FAO key rate-limiting enzyme carnitine palmitoyl-transferase 1A (CPT1A). (biomedcentral.com)
  • As may be expected from members of the same enzymatic family, there is strong similarity between the structures of carnitine acetyltransferase (CRAT) and CROT, as these enzymes have 36% sequence homology. (wikipedia.org)
  • In the absence of carnitine acetyltransferase (CRAT), acyltransferases such as CROT can catalyze the acetyl group transfer from acetylcarnitine to coenzyme A. Rescue experiments with CROT gene knockout cells have shown that peroxisomal CROT can mediate acetyl-CoA production under glucose-limited conditions. (wikipedia.org)
  • To help define in molecular detail the catalytic mechanism of these enzymes, we report here the high resolution crystal structure of wild-type murine carnitine acetyltransferase (CrAT) in a ternary complex with its substrates acetyl-CoA and carnitine, and the structure of the S554A/M564G double mutant in a ternary complex with the substrates CoA and hexanoylcarnitine. (proteopedia.org)
  • Crystal structures of murine carnitine acetyltransferase in ternary complexes with its substrates. (proteopedia.org)
  • 15. Inhibition of carnitine palmitoyl transferase 1A-induced fatty acid oxidation suppresses cell progression in gastric cancer. (nih.gov)
  • CROT may play a role in converting peroxisomal medium-chain acylcarnitine derivatives to medium-chain acyl-CoA derivatives. (wikipedia.org)
  • Acyl CoA with carnitine gives acylcarnitine, and the enzyme is carnitine acyl transferase 1 (rate-limiting enzyme). (prepladder.com)
  • Carnitine acyltransferase 2 releases the carnitine back and converts acylcarnitine to acyl CoA. (prepladder.com)
  • Plasma carnitine and the carnitine precursor γ-butyrobetaine was higher in the FPH-group compared to control, as was plasma short-chained and medium-chained acylcarnitine esters. (biomedcentral.com)
  • Carnitine O-octanoyltransferase (CROT or COT) is a member of the transferase family, more specifically a carnitine acyltransferase, a type of enzyme which catalyzes the transfer of acyl groups from acyl-CoAs to carnitine, generating CoA and an acyl-carnitine. (wikipedia.org)
  • EC 2.3.1.137) The systematic name of this enzyme is octanoyl-CoA:L-carnitine O-octanoyltransferase. (wikipedia.org)
  • Specifically, CROT catalyzes the chemical reaction: octanoyl-CoA + L-carnitine ⇌ {\displaystyle \rightleftharpoons } CoA + L-octanoylcarnitine Thus, the two substrates of this enzyme are octanoyl-CoA and L-carnitine and its two products are CoA and L-octanoylcarnitine. (wikipedia.org)
  • This enzyme belongs to the family of transferases, specifically those acyltransferases transferring groups other than aminoacyl groups. (wikipedia.org)
  • Interestingly, the trend for a related enzyme, carnitine palmitoyl transferase (CPT), the opposite trend was found. (wikipedia.org)
  • The CPT1A gene provides instructions for making an enzyme called carnitine palmitoyltransferase 1A, which is found in the liver. (medlineplus.gov)
  • Without enough of this enzyme, carnitine is not attached to long-chain fatty acids. (medlineplus.gov)
  • Gene expression and enzyme activity was analysed in liver, fatty acid composition was analyzed in liver and ovarian white adipose tissue, and inflammatory parameters, carnitine, and acylcarnitines were analyzed in plasma. (biomedcentral.com)
  • Integrated transcriptome and metabolome network analysis showed 2 major gene-metabolite clusters, 1 centered on the transcript for the bidirectional glucose transporter 2 (Glut2) and the other centered on the transcripts for carnitine-palmitoyl transferase 2 (Cpt2) and acetyl-CoA acyltransferase (Acaa1). (nih.gov)
  • The change of fat deposit was accompanied with the reduction in serum lipids, the increase in the activity of enzymes involved in lipid metabolism in the liver, the suppression of fatty acid synthase and the enhance of carnitine fatty acyl transferase Ⅰ (CPT1) expression. (authorea.com)
  • 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)
  • 14. Carnitine palmitoyltransferase 1A functions to repress FoxO transcription factors to allow cell cycle progression in ovarian cancer. (nih.gov)
  • A key difference between these enzymes that may explain their selectivities between short and medium-chain acyl-CoAs hinges on a glycine residue which is present in the acyl binding site in CROT, Gly-553. (wikipedia.org)
  • Sets of 4 enzymes (an acyl dehydrogenase, a hydratase, a hydroxyacyl dehydrogenase, and a lyase) specific for different chain lengths (very long chain, long chain, medium chain, and short chain) are required to catabolize fatty acids completely. (msdmanuals.com)
  • Carnitine acetylase seems to affect the flux through the pyruvate dehydrogenase complex. (proteopedia.org)
  • More than 20 mutations in the CPT1A gene have been found to cause carnitine palmitoyltransferase I (CPT I) deficiency. (medlineplus.gov)
  • People with carnitine/acyltransferase deficiency can't use LCFA for fuel. (flashcardmachine.com)
  • 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)
  • In CRAT, however, the residue in the same position in the acyl binding site is a methionine residue, Met-564. (wikipedia.org)
  • M564G CRAT mutants have been shown to accept a wider variety of acyl-CoA substrates. (wikipedia.org)
  • The structural information provides significant new insights into the catalytic mechanism of CrAT and possibly carnitine acyltransferases in general. (proteopedia.org)
  • While CROT's canonical substrate is octanoyl-CoA, CROT is also known to be able to catalyze the deacylation of numerous acyl-CoAs, such as acetyl-CoA, propionyl-CoA, butyryl-CoA, and hexanoyl-CoA. (wikipedia.org)
  • Acyl-CoA and acetyl-CoA should not be confused with each other. (amboss.com)
  • Acetyl-CoA is the acyl-CoA of acetic acid (also known as acetate). (amboss.com)
  • A maximum of 60% inhibition was observed in purified CROT, similar to what was seen with carnitine palmitoyltransferase (CPT). (wikipedia.org)
  • 9. Carnitine-acyltransferase system inhibition, cancer cell death, and prevention of myc-induced lymphomagenesis. (nih.gov)
  • Lipid vacuoles to Acyl CoA (Nathan) (modeling in process) g. (nih.gov)
  • This reaction is easily chemically reversible, and does not require any energy input, as both fatty acyl-CoAs and fatty acylcarnitines are considered chemically "activated" forms of fatty acyl groups. (wikipedia.org)
  • Palmityl-CoA, also known as palmitoyl CoA or CoA, palmitoyl, belongs to the class of organic compounds known as long-chain fatty acyl coas. (ymdb.ca)
  • CoA is also involved in acyl group transfers, and these reactions are important in many metabolic processes, including the synthesis of ketones, amino acids and neurotransmitters. (coalitionbrewing.com)
  • Similarly, G553M CROT mutants show marked inactivity with octanoyl-CoA, while maintaining activity with short-chain acyl-CoAs. (wikipedia.org)
  • the putative mechanisms involved may be its action in reducing the lipase lipoprotein activity and to increase the carnitine-palmitoil-transferase-1 (CAT-1) activity, its interaction with PPARγ, and to raise the expression of UCP-1. (gpni.fit)