Summary: Constituent of striated muscle and liver. It is used therapeutically to stimulate gastric and pancreatic secretions and in the treatment of hyperlipoproteinemias.

Top Publications

  1. pmc Intestinal microbiota metabolism of L-carnitine, a nutrient in red meat, promotes atherosclerosis
    Robert A Koeth
    Department of Cellular and Molecular Medicine, Cleveland Clinic, Cleveland, Ohio, USA
    Nat Med 19:576-85. 2013
  2. pmc Relationships between circulating metabolic intermediates and insulin action in overweight to obese, inactive men and women
    Kim M Huffman
    Physical Medicine and Rehabilitation, Veterans Affairs Medical Center, Durham, North Carolina, USA
    Diabetes Care 32:1678-83. 2009
  3. pmc Unique acyl-carnitine profiles are potential biomarkers for acquired mitochondrial disease in autism spectrum disorder
    R E Frye
    Department of Pediatrics, Arkansas Children s Hospital Research Institute, Little Rock, AR, USA
    Transl Psychiatry 3:e220. 2013
  4. ncbi Enzymology of the carnitine biosynthesis pathway
    Karin Strijbis
    Department of Medical Biochemistry, Academic Medical Center, Amsterdam, The Netherlands
    IUBMB Life 62:357-62. 2010
  5. ncbi L-carnitine supplementation to diet: a new tool in treatment of nonalcoholic steatohepatitis--a randomized and controlled clinical trial
    Mariano Malaguarnera
    Department of Senescence, Urological and Neurological Sciences, University of Catania, Catania, Italy
    Am J Gastroenterol 105:1338-45. 2010
  6. pmc Muscle-specific deletion of carnitine acetyltransferase compromises glucose tolerance and metabolic flexibility
    Deborah M Muoio
    Sarah W Stedman Nutrition and Metabolism Center, Department of Medicine, Duke University, Durham, NC 27704, USA
    Cell Metab 15:764-77. 2012
  7. pmc A common X-linked inborn error of carnitine biosynthesis may be a risk factor for nondysmorphic autism
    Patricia B S Celestino-Soper
    Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, TX 77030, USA
    Proc Natl Acad Sci U S A 109:7974-81. 2012
  8. ncbi Carnitine derivatives: clinical usefulness
    Mariano Malaguarnera
    Research Centre The Great Senescence, University of Catania, Catania, Italy
    Curr Opin Gastroenterol 28:166-76. 2012
  9. ncbi L-carnitine attenuates oxidant injury in HK-2 cells via ROS-mitochondria pathway
    Junsheng Ye
    Department of Organ Transplantation, Nanfang Hospital, Southern Medical University, Guangzhou 510515, China
    Regul Pept 161:58-66. 2010
  10. ncbi Pharmacokinetics of L-carnitine
    Allan M Evans
    Centre for Pharmaceutical Research, School of Pharmaceutical, Molecular and Biomedical Sciences, University of South Australia, Adelaide, South Australia, Australia
    Clin Pharmacokinet 42:941-67. 2003

Detail Information

Publications331 found, 100 shown here

  1. pmc Intestinal microbiota metabolism of L-carnitine, a nutrient in red meat, promotes atherosclerosis
    Robert A Koeth
    Department of Cellular and Molecular Medicine, Cleveland Clinic, Cleveland, Ohio, USA
    Nat Med 19:576-85. 2013
    ..We demonstrate here that metabolism by intestinal microbiota of dietary L-carnitine, a trimethylamine abundant in red meat, also produces TMAO and accelerates atherosclerosis in mice...
  2. pmc Relationships between circulating metabolic intermediates and insulin action in overweight to obese, inactive men and women
    Kim M Huffman
    Physical Medicine and Rehabilitation, Veterans Affairs Medical Center, Durham, North Carolina, USA
    Diabetes Care 32:1678-83. 2009
    ..To determine whether circulating metabolic intermediates are related to insulin resistance and beta-cell dysfunction in individuals at risk for type 2 diabetes...
  3. pmc Unique acyl-carnitine profiles are potential biomarkers for acquired mitochondrial disease in autism spectrum disorder
    R E Frye
    Department of Pediatrics, Arkansas Children s Hospital Research Institute, Little Rock, AR, USA
    Transl Psychiatry 3:e220. 2013
    ..Acyl-carnitine panels were determined to be abnormal if three or more individual acyl-carnitine species were abnormal in the ..
  4. ncbi Enzymology of the carnitine biosynthesis pathway
    Karin Strijbis
    Department of Medical Biochemistry, Academic Medical Center, Amsterdam, The Netherlands
    IUBMB Life 62:357-62. 2010
    The water-soluble zwitterion carnitine is an essential metabolite in eukaryotes required for fatty acid oxidation as it functions as a carrier during transfer of activated acyl and acetyl groups across intracellular membranes...
  5. ncbi L-carnitine supplementation to diet: a new tool in treatment of nonalcoholic steatohepatitis--a randomized and controlled clinical trial
    Mariano Malaguarnera
    Department of Senescence, Urological and Neurological Sciences, University of Catania, Catania, Italy
    Am J Gastroenterol 105:1338-45. 2010
    ..No treatment has been conclusively shown to improve NASH or prevent disease progression. The function of L-carnitine to modulate lipid profile, glucose metabolism, oxidative stress, and inflammatory responses has been shown...
  6. pmc Muscle-specific deletion of carnitine acetyltransferase compromises glucose tolerance and metabolic flexibility
    Deborah M Muoio
    Sarah W Stedman Nutrition and Metabolism Center, Department of Medicine, Duke University, Durham, NC 27704, USA
    Cell Metab 15:764-77. 2012
    ..Here, we identify an essential role for the mitochondrial matrix enzyme, carnitine acetyltransferase (CrAT), in regulating substrate switching and glucose tolerance...
  7. pmc A common X-linked inborn error of carnitine biosynthesis may be a risk factor for nondysmorphic autism
    Patricia B S Celestino-Soper
    Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, TX 77030, USA
    Proc Natl Acad Sci U S A 109:7974-81. 2012
    ..TMLHE maps to the X chromosome and encodes the first enzyme in carnitine biosynthesis, 6-N-trimethyllysine dioxygenase...
  8. ncbi Carnitine derivatives: clinical usefulness
    Mariano Malaguarnera
    Research Centre The Great Senescence, University of Catania, Catania, Italy
    Curr Opin Gastroenterol 28:166-76. 2012
    b>Carnitine and its derivatives are natural substances involved in both carbohydrate and lipid metabolism. This review summarizes the recent progress in the field in relation to the molecular mechanisms.
  9. ncbi L-carnitine attenuates oxidant injury in HK-2 cells via ROS-mitochondria pathway
    Junsheng Ye
    Department of Organ Transplantation, Nanfang Hospital, Southern Medical University, Guangzhou 510515, China
    Regul Pept 161:58-66. 2010
    Oxidative stress has been considered as the possible mechanism of renal ischemia/reperfusion injury. L-carnitine is an endogenous mitochondrial membrane compound and could effectively protect ischemia-reperfusion injury in the kidney...
  10. ncbi Pharmacokinetics of L-carnitine
    Allan M Evans
    Centre for Pharmaceutical Research, School of Pharmaceutical, Molecular and Biomedical Sciences, University of South Australia, Adelaide, South Australia, Australia
    Clin Pharmacokinet 42:941-67. 2003
    L-Carnitine is a naturally occurring compound that facilitates the transport of fatty acids into mitochondria for beta-oxidation...
  11. pmc L-carnitine is an endogenous HDAC inhibitor selectively inhibiting cancer cell growth in vivo and in vitro
    Hongbiao Huang
    Protein Modification and Degradation Lab, Department of Pathophysiology, Guangzhou Medical College, Guangdong, People s Republic of China
    PLoS ONE 7:e49062. 2012
    L-carnitine (LC) is generally believed to transport long-chain acyl groups from fatty acids into the mitochondrial matrix for ATP generation via the citric acid cycle...
  12. ncbi Randomized phase III clinical trial of a combined treatment with carnitine + celecoxib ± megestrol acetate for patients with cancer-related anorexia/cachexia syndrome
    Clelia Madeddu
    Department of Medical Oncology, University of Cagliari, Cagliari, Italy
    Clin Nutr 31:176-82. 2012
    ..e. antioxidants) with carnitine + celecoxib ± megestrol acetate for the treatment of cancer-related anorexia/cachexia syndrome (CACS): the ..
  13. pmc Metabolic profiling of PPARalpha-/- mice reveals defects in carnitine and amino acid homeostasis that are partially reversed by oral carnitine supplementation
    Liza Makowski
    Division of Endocrinology, Metabolism, and Nutrition, Duke University School of Medicine, Durham, North Carolina, USA
    FASEB J 23:586-604. 2009
    ..These results reflect a metabolic bottleneck downstream of carnitine palmitoyltransferase-1, a mitochondrial enzyme that catalyzes the first step in beta-oxidation...
  14. pmc Disorders of carnitine transport and the carnitine cycle
    Nicola Longo
    Division of Medical Genetics, Department of Pediatrics, University of Utah, 2C412 SOM, 50 North Medical Drive, Salt Lake City, UT, USA
    Am J Med Genet C Semin Med Genet 142:77-85. 2006
    b>Carnitine plays an essential role in the transfer of long-chain fatty acids across the inner mitochondrial membrane...
  15. pmc Bioactives from bitter melon enhance insulin signaling and modulate acyl carnitine content in skeletal muscle in high-fat diet-fed mice
    Zhong Q Wang
    Center for the Study of Botanicals and Metabolic Syndrome, Pennington Biomedical Research Center, LSU System, Baton Rouge, LA 70808, USA
    J Nutr Biochem 22:1064-73. 2011
    ..Nutritional supplementation with this extract, if validated for human studies, may offer an adjunctive therapy for diabetes...
  16. ncbi Molecular and functional identification of sodium ion-dependent, high affinity human carnitine transporter OCTN2
    I Tamai
    Faculty of Pharmaceutical Sciences, Kanazawa University, 13 1 Takara machi, Kanazawa 920 0934, Japan
    J Biol Chem 273:20378-82. 1998
    Primary carnitine deficiency, because of a defect of the tissue plasma membrane carnitine transporters, causes critical symptoms. However, the transporter has not been molecularly identified...
  17. ncbi Effects of carnitine on peripheral blood mitochondrial DNA copy number and liver function in non-alcoholic fatty liver disease
    Chae Young Lim
    Department of Internal School of Medicine, Eulji University, Seoul, Korea
    Korean J Gastroenterol 55:384-9. 2010
    Functional and anatomical abnormalities of mitochondria play an important role in developing steatohepatitis. Carnitine is essential for enhanced mitochondrial beta oxidation through the transfer of long-chain fatty acids into the ..
  18. ncbi The ATP-binding cassette transporter Cbc (choline/betaine/carnitine) recruits multiple substrate-binding proteins with strong specificity for distinct quaternary ammonium compounds
    Chiliang Chen
    Department of Plant Pathology, Iowa State University, Ames, IA 50011, USA
    Mol Microbiol 75:29-45. 2010
    We identified a choline, betaine and carnitine transporter, designated Cbc, from Pseudomonas syringae and Pseudomonas aeruginosa that is unusual among members of the ATP-binding cassette (ABC) transporter family in its use of multiple ..
  19. pmc Critical update for the clinical use of L-carnitine analogs in cardiometabolic disorders
    Carmen Mingorance
    Department of Pharmacology, School of Pharmacy, University of Seville, Seville, Spain
    Vasc Health Risk Manag 7:169-76. 2011
    Acetyl-L-carnitine (ALC) and propionyl-L-carnitine (PLC) are two naturally occurring carnitine derivates formed by carnitine acetyltransferase...
  20. ncbi Role of carnitine in the regulation of glucose homeostasis and insulin sensitivity: evidence from in vivo and in vitro studies with carnitine supplementation and carnitine deficiency
    Robert Ringseis
    Institute of Animal Nutrition and Nutrition Physiology, Justus Liebig University Giessen, Heinrich Buff Ring 26 32, 35390 Giessen, Germany
    Eur J Nutr 51:1-18. 2012
    Although carnitine is best known for its role in the import of long-chain fatty acids (acyl groups) into the mitochondrial matrix for subsequent β-oxidation, carnitine is also necessary for the efflux of acyl groups out of the ..
  21. ncbi Carnitine and acylcarnitines: pharmacokinetic, pharmacological and clinical aspects
    Stephanie E Reuter
    School of Pharmacy Medical Sciences, University of South Australia, Adelaide, SA, Australia
    Clin Pharmacokinet 51:553-72. 2012
    L-Carnitine (levocarnitine) is a naturally occurring compound found in all mammalian species...
  22. ncbi L-carnitine reduces doxorubicin-induced apoptosis through a prostacyclin-mediated pathway in neonatal rat cardiomyocytes
    Hung Hsin Chao
    Department of Medicine, Taipei Medical University, Taipei, Taiwan, ROC
    Int J Cardiol 146:145-52. 2011
    Clinical use of doxorubicin is greatly limited by its severe cardiotoxic side effects. L-carnitine is a vitamin-like substance which has been successfully used in many cardiomyopathies, however, the intracellular mechanism(s) remain ..
  23. ncbi Clinical validation of cutoff target ranges in newborn screening of metabolic disorders by tandem mass spectrometry: a worldwide collaborative project
    David M S McHugh
    Mayo Clinic College of Medicine, Rochester, Minnesota, USA
    Genet Med 13:230-54. 2011
    ..To achieve clinical validation of cutoff values for newborn screening by tandem mass spectrometry through a worldwide collaborative effort...
  24. ncbi Carnitine transporter defect diagnosed by newborn screening with electrospray tandem mass spectrometry
    B Wilcken
    New South Wales Newborn Screening and Biochemical Genetics Services, The New Children's Hospital, Westmead, Sydney, Australia
    J Pediatr 138:581-4. 2001
    The carnitine transporter defect is a potentially fatal but treatable disorder...
  25. ncbi L-carnitine prevents the progression of atherosclerotic lesions in hypercholesterolaemic rabbits
    M M Sayed-Ahmed
    Pharmacology Unit, Cancer Biology Department, National Cancer Institute, Cairo, Egypt
    Pharmacol Res 44:235-42. 2001
    This study has been initiated to investigate, in hypercholesterolaemic rabbits, whether L-carnitine deficiency could be an additional risk factor in atherosclerosis, and if so, whether L-carnitine supplementation could prevent the ..
  26. pmc Doxorubicin toxicity can be ameliorated during antioxidant L-carnitine supplementation
    Othman A Alshabanah
    Department of Pharmacology College of Pharmacy, King Saud University, Riyadh Kingdom of Saudi Arabia
    Oxid Med Cell Longev 3:428-33. 2010
    ..The present study investigates whether L-carnitine, antioxidant agent, can reduce the hepatic damage induced by doxorubicin...
  27. ncbi Excitotoxic damage, disrupted energy metabolism, and oxidative stress in the rat brain: antioxidant and neuroprotective effects of L-carnitine
    Daniela Silva-Adaya
    Laboratorio de Aminoácidos Excitadores, Instituto Nacional de Neurologia y Neurocirugia Manuel Velasco Suarez, Mexico, Mexico
    J Neurochem 105:677-89. 2008
    ..Our results suggest that the protective properties of L-CAR in the neurotoxic models tested are mostly mediated by its characteristics as an antioxidant agent...
  28. ncbi Carnitine esters prevent oxidative stress damage and energy depletion following transient forebrain ischaemia in the rat hippocampus
    Abdulhakeem A Al-Majed
    Department of Pharmacology, College of Pharmacy, King Saud University, Riyadh, Saudi Arabia
    Clin Exp Pharmacol Physiol 33:725-33. 2006
    1. The present study investigated whether propionyl-L-carnitine (PLC) has neuroprotective effects, similar to those reported for acetyl-L-carnitine (AC), against transient forebrain ischaemia-induced neuronal damage and biochemical ..
  29. ncbi Primary systemic carnitine deficiency is caused by mutations in a gene encoding sodium ion-dependent carnitine transporter
    J Nezu
    Chugai Research Institute for Molecular Medicine, Ibaraki, Japan
    Nat Genet 21:91-4. 1999
    Primary systemic carnitine deficiency (SCD; OMIM 212140) is an autosomal recessive disorder characterized by progressive cardiomyopathy, skeletal myopathy, hypoglycaemia and hyperammonaemia...
  30. pmc Carnitine insufficiency caused by aging and overnutrition compromises mitochondrial performance and metabolic control
    Robert C Noland
    Sarah W Stedman Nutrition and Metabolism Center, Duke University, Durham, North Carolina 27710, USA
    J Biol Chem 284:22840-52. 2009
    In addition to its essential role in permitting mitochondrial import and oxidation of long chain fatty acids, carnitine also functions as an acyl group acceptor that facilitates mitochondrial export of excess carbons in the form of ..
  31. ncbi The role of carnitine in normal and altered fatty acid metabolism
    Charles Hoppel
    Department of Medicine, Louis Stokes Cleveland Department of Veterans Affairs Medical Center, Case Western Reserve University, Cleveland, OH 44106, USA
    Am J Kidney Dis 41:S4-12. 2003
    b>Carnitine is a low-molecular-weight compound obtained from the diet that also is biosynthesized from the essential amino acids lysine and methionine...
  32. pmc Increased levels of plasma acylcarnitines in obesity and type 2 diabetes and identification of a marker of glucolipotoxicity
    Stephanie J Mihalik
    Department of Pediatrics, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, USA
    Obesity (Silver Spring) 18:1695-700. 2010
    ..Plasma long-chain AcylCN species are increased in obesity and T2DM, suggesting that more fatty acids can enter mitochondria. In T2DM, many shorter species accumulate, suggesting that they have a generalized complex oxidation defect...
  33. ncbi Carnitine: a nutritional, biosynthetic, and functional perspective
    Alison Steiber
    Department of Nutrition, Case Western Reserve University, Medical Research Service, Cleveland, OH 44106, USA
    Mol Aspects Med 25:455-73. 2004
    b>Carnitine status in humans is reported to vary according to body composition, gender, and diet. Plasma carnitine concentration positively correlates with the dietary intake of carnitine...
  34. ncbi Antioxidant and antiradical activities of L-carnitine
    Ilhami Gulcin
    Ataturk University, Faculty of Arts and Sciences, Department of Chemistry, TR 25240 Erzurum Turkey
    Life Sci 78:803-11. 2006
    L-carnitine plays an important regulatory role in the mitochondrial transport of long-chain free fatty acids. In this study, the antioxidant activity of L-carnitine was investigated as in vitro...
  35. ncbi Carnitine revisited: potential use as adjunctive treatment in diabetes
    R A Power
    Division of Nutrition and Chronic Disease, Pennington Biomedical Research Center, Baton Rouge, LA, USA
    Diabetologia 50:824-32. 2007
    This study examined the efficacy of supplemental L: -carnitine as an adjunctive diabetes therapy in mouse models of metabolic disease...
  36. ncbi Supplementation with l-carnitine downregulates genes of the ubiquitin proteasome system in the skeletal muscle and liver of piglets
    J Keller
    Institute of Animal Nutrition and Nutrition Physiology, Justus Liebig Universität Gießen, Heinrich Buff Ring 26 32, 35392 Gießen, Germany
    Animal 6:70-8. 2012
    Supplementation of carnitine has been shown to improve performance characteristics such as protein accretion in growing pigs. The molecular mechanisms underlying this phenomenon are largely unknown...
  37. pmc Carnitine biosynthesis in mammals
    Frederic M Vaz
    Laboratory for Genetic Metabolic Diseases, Departments of Clinical Chemistry and Paediatrics, Emma Children s Hospital, Academic Medical Centre, University of Amsterdam, P O Box 22700, 1100 DE Amsterdam, The Netherlands
    Biochem J 361:417-29. 2002
    b>Carnitine is indispensable for energy metabolism, since it enables activated fatty acids to enter the mitochondria, where they are broken down via beta-oxidation...
  38. ncbi Hepatic expression of malonyl-CoA decarboxylase reverses muscle, liver and whole-animal insulin resistance
    Jie An
    Sarah W Stedman Nutrition and Metabolism Center and Department of Pharmacology and Cancer Biology, Duke University Medical Center, Durham, North Carolina 27710, USA
    Nat Med 10:268-74. 2004
    ..The best explanation for our findings is that hepatic expression of MCD lowered circulating FFA levels, which led to lowering of muscle beta-OH-butyrate levels and improvement of insulin sensitivity...
  39. ncbi Acylcarnitines: role in brain
    Lauren L Jones
    Department of Food Science and Human Nutrition, University of Florida, Gainesville, FL, USA
    Prog Lipid Res 49:61-75. 2010
    l-carnitine is present in mammalian cells as free carnitine and acylcarnitines. The acylcarnitine profile has been shown to be useful in identifying inborn errors of metabolism and to be altered under different metabolic conditions...
  40. pmc Fatty acid oxidation and meiotic resumption in mouse oocytes
    Stephen M Downs
    Department of Biological Sciences, Marquette University, Milwaukee, Wisconsin 53233, USA
    Mol Reprod Dev 76:844-53. 2009
    ..Etomoxir and malonyl CoA, two inhibitors of carnitine palmitoyl transferase-1 (CPT1), and thus FAO, blocked meiotic induction in dbcAMP-arrested cumulus cell-enclosed ..
  41. pmc The brain-specific carnitine palmitoyltransferase-1c regulates energy homeostasis
    Michael J Wolfgang
    Department of Biological Chemistry, Johns Hopkins University School of Medicine, Baltimore, MD 21205, USA
    Proc Natl Acad Sci U S A 103:7282-7. 2006
    ..Malonyl-CoA is an established inhibitor of carnitine palmitoyltransferase-1 (CPT1), an outer mitochondrial membrane enzyme that controls entry of fatty acids into ..
  42. ncbi Expression patterns of the organic cation/carnitine transporter family in adult murine brain
    Anne Marie Lamhonwah
    Division of Neurology, Department of Pediatrics, The Hospital for Sick Children, 555 University Avenue, University of Toronto, Toronto, Ont, Canada M5G 1X8
    Brain Dev 30:31-42. 2008
    Organic cation/carnitine transporters transport carnitine, drugs, and xenobiotics (e.g. choline, acetylcarnitine, betaine, valproic acid), and are expressed in muscle, heart, blood vessels, kidney, gut, etc.
  43. ncbi Prevention of free fatty acid-induced hepatic lipotoxicity by carnitine via reversal of mitochondrial dysfunction
    Dae Won Jun
    Department of Internal Medicine, Hanyang University School of Medicine, Seoul, Korea
    Liver Int 31:1315-24. 2011
    ..Mitochondria are the main sites for fatty acid oxidation and play a central role in lipotoxicity and nonalcoholic steatohepatitis...
  44. pmc Identification of genes required for Pseudomonas aeruginosa carnitine catabolism
    Matthew J Wargo
    The Vermont Lung Center, Department of Medicine, University of Vermont College of Medicine, Burlington, VT 05405, USA
    Microbiology 155:2411-9. 2009
    b>Carnitine is a quaternary amine compound prevalent in animal tissues, and a potential carbon, nitrogen and energy source for pathogens during infection...
  45. ncbi Metabolomic changes and protective effect of (L)-carnitine in rat kidney ischemia/reperfusion injury
    Ye Liu
    Nephrology institute of PLA, Department of Internal Medicine, Changzheng Hospital, Second Military Medical University, Shanghai, China
    Kidney Blood Press Res 35:373-81. 2012
    ..The present study was performed in order to further understand the metabolomic changes of ischemia/reperfusion (I/R)-induced AKI and the protective effect of L-carnitine on AKI.
  46. ncbi Host invasion during rice-blast disease requires carnitine-dependent transport of peroxisomal acetyl-CoA
    Marilou Ramos-Pamplona
    Fungal Genomics Group, Temasek Life Sciences Laboratory, 1 Research Link, National University of Singapore, Singapore 117604
    Mol Microbiol 61:61-75. 2006
    ..We further show that a peroxisome-associated carnitine acetyl-transferase (Crat1) activity is essential for such appressorial function in Magnaporthe...
  47. ncbi MCAD deficiency in Denmark
    Brage Storstein Andresen
    Research Unit for Molecular Medicine, Aarhus University Hospital and Faculty of Health Science, Skejby Sygehus, Aarhus, Denmark
    Mol Genet Metab 106:175-88. 2012
    ..We conclude that MCADD is much more frequent than expected, has a reduced penetrance and that rapid genotyping using the initial blood spot sample is important for correct diagnosis and counseling...
  48. ncbi L-Carnitine: an adequate supplement for a multi-targeted anti-wasting therapy in cancer
    Silvia Busquets
    Cancer Research Group, Departament de Bioquimica i Biologia Molecular, Facultat de Biologia, Universitat de Barcelona, Barcelona, Spain
    Clin Nutr 31:889-95. 2012
    ..Tumour growth is associated with weight loss resulting from both adipose and muscle wasting...
  49. ncbi Pharmacological and pathophysiological roles of carnitine/organic cation transporters (OCTNs: SLC22A4, SLC22A5 and Slc22a21)
    Ikumi Tamai
    Faculty of Pharmaceutical Sciences, Institute of Medical, Pharmaceutical and Health Sciences, Kanazawa University, Kanazawa, 920 1192, Japan
    Biopharm Drug Dispos 34:29-44. 2013
    The carnitine/organic cation transporter (OCTN) family consists of three transporter isoforms, i.e. OCTN1 (SLC22A4) and OCTN2 (SLC22A5) in humans and animals and Octn3 (Slc22a21) in mice...
  50. pmc Expression of genes involved in hepatic carnitine synthesis and uptake in dairy cows in the transition period and at different stages of lactation
    Gloria Schlegel
    Institute of Animal Nutrition and Nutrition Physiology, Justus Liebig Universitat Giessen, Giessen, Germany
    BMC Vet Res 8:28. 2012
    In rodents and pigs, it has shown that carnitine synthesis and uptake of carnitine into cells are regulated by peroxisome proliferator-activated receptor α (PPARA), a transcription factor which is physiologically activated during ..
  51. pmc Oxygen glucose deprivation in rat hippocampal slice cultures results in alterations in carnitine homeostasis and mitochondrial dysfunction
    Thomas F Rau
    Department of Biomedical and Pharmaceutical Sciences, University of Montana, Missoula, Montana, United States of America
    PLoS ONE 7:e40881. 2012
    ..b>Carnitine metabolism directly supports mitochondrial metabolism by shuttling long chain fatty acids across the inner ..
  52. ncbi Propionyl-L-carnitine improves postischemic blood flow recovery and arteriogenetic revascularization and reduces endothelial NADPH-oxidase 4-mediated superoxide production
    Maria Antonietta Stasi
    Dept of Biopathology and Image Diagnostics, Tor Vergata University of Rome, Via Montpellier, 00133 Rome, Italy
    Arterioscler Thromb Vasc Biol 30:426-35. 2010
    The beneficial effect of the natural compound propionyl-l-carnitine (PLC) on intermittent claudication in patients with peripheral arterial disease is attributed to its anaplerotic function in ischemic tissues, but inadequate information ..
  53. ncbi Dialysis-related carnitine disorder
    S Susan Hedayati
    Department of Medicine, Division of Nephrology, Veterans Affairs North Texas Health Care System, Dallas, Texas 75216 7167, USA
    Semin Dial 19:323-8. 2006
    L-carnitine plays an essential role in the beta-oxidation of fatty acids by catalyzing their transport into the mitochondrial matrix...
  54. ncbi Protective effects of l-carnitine and piracetam against mitochondrial permeability transition and PC3 cell necrosis induced by simvastatin
    Rute A P Costa
    Departamento de Patologia Clinica, Universidade Estadual de Campinas, Campinas, Sao Paulo, Brazil
    Eur J Pharmacol 701:82-6. 2013 simvastatin-induced prostate cancer cell death as well as the mechanisms of cell death protection by l-carnitine or piracetam...
  55. ncbi Carnitine, mitochondrial function and therapy
    Victor A Zammit
    University of Warwick, Clinical Sciences Research Institute, Coventry, England, UK
    Adv Drug Deliv Rev 61:1353-62. 2009
    b>Carnitine is important for cell function and survival primarily because of its involvement in the multiple equilibria between acylcarnitine and acyl-CoA esters established through the enzymatic activities of the family of carnitine ..
  56. ncbi Beta-oxidation is essential for mouse oocyte developmental competence and early embryo development
    Kylie R Dunning
    The Robinson Institute, School of Paediatrics and Reproductive Health, The University of Adelaide, Adelaide, South Australia, Australia
    Biol Reprod 83:909-18. 2010
    ..lipids occurs within mitochondria via beta-oxidation of fatty acids, with the rate-limiting step catalyzed by carnitine palmitoyl transferase I (CPT1B), a process also requiring carnitine...
  57. pmc Plasma acylcarnitine profiles suggest incomplete long-chain fatty acid beta-oxidation and altered tricarboxylic acid cycle activity in type 2 diabetic African-American women
    Sean H Adams
    USDA Agricultural Research Service Western Human Nutrition Research Center, USA
    J Nutr 139:1073-81. 2009
    ..MS analyses of 42 carnitine moieties in plasma samples from fasting type 2 diabetics (n = 44) and nondiabetics (n = 12) with or without the ..
  58. ncbi Changes of the plasma metabolome during an oral glucose tolerance test: is there more than glucose to look at?
    Xinjie Zhao
    Key Laboratory of Separation Science for Analytical Chemistry, Dalian Institute of Chemical Physics, Chinese Academy of Sciences, Dalian, China
    Am J Physiol Endocrinol Metab 296:E384-93. 2009
    ..The findings of our metabolomics study reveal detailed insights in the complex physiological regulation of the metabolism during an oGTT offering novel perspectives of this widely used procedure...
  59. pmc The in-depth evaluation of suspected mitochondrial disease
    Richard H Haas
    Department of Neurosciences, University of California San Diego, La Jolla, CA and Rady Children s Hospital San Diego, San Diego, CA, United States
    Mol Genet Metab 94:16-37. 2008
    ..The aim of this work is to facilitate the diagnosis of mitochondrial disease by geneticists, neurologists, and other metabolic specialists who face the challenge of evaluating patients of all ages with suspected mitochondrial disease...
  60. ncbi L-carnitine enhances oocyte maturation and development of parthenogenetic embryos in pigs
    G Q Wu
    Key Laboratory of Animal Genetics, Breeding and Reproduction, Ministry of Agriculture, College of Animal Science and Technology, China Agricultural University, Beijing, People s Republic of China
    Theriogenology 76:785-93. 2011
    The objective was to determine whether adding L-carnitine in IVM/IVC medium enhanced maturation and developmental competence of porcine oocytes in vitro...
  61. ncbi PPAR alpha mediates transcriptional upregulation of novel organic cation transporters-2 and -3 and enzymes involved in hepatic carnitine synthesis
    Alexander Koch
    Institute of Agricultural and Nutritional Sciences, Martin Luther University Halle Wittenberg, D 06108 Halle Saale, Germany
    Exp Biol Med (Maywood) 233:356-65. 2008
    ..05). Moreover, wild-type mice treated with WY 14,643 had greater mRNA abundances of enzymes involved in hepatic carnitine synthesis (4-N-trimethylaminobutyraldehyde dehydrogenase, gamma-butyrobetaine dioxygenase) and increased ..
  62. ncbi Relationship between serum carnitine, acylcarnitines, and renal function in patients with chronic renal disease
    Denis Fouque
    Department of Nephrology, Hopital E Herriot, and JE 2411, University Claude Bernard Lyon 1, Lyon, France
    J Ren Nutr 16:125-31. 2006
    Serum free carnitine is decreased and serum acylcarnitines are increased in maintenance hemodialysis (MHD) patients, and the causes for these abnormalities are not known...
  63. pmc SMA CARNIVAL TRIAL PART II: a prospective, single-armed trial of L-carnitine and valproic acid in ambulatory children with spinal muscular atrophy
    John T Kissel
    Department of Neurology, The Ohio State University, Columbus, Ohio, United States of America
    PLoS ONE 6:e21296. 2011
    ..The SMA CARNIVAL TRIAL was a two part prospective trial to evaluate oral VPA and L-carnitine in SMA children. Part 1 targeted non-ambulatory children ages 2-8 in a 12 month cross over design...
  64. ncbi L-Carnitine supplementation reduces oxidized LDL cholesterol in patients with diabetes
    Mariano Malaguarnera
    Department of Senescence, Urological and Neurological Sciences, University of Catania, Catania, Italy
    Am J Clin Nutr 89:71-6. 2009
    Patients with type 2 diabetes are under high oxidative stress, and levels of hyperglycemia correlate strongly with levels of LDL oxidation. Carnitine favorably modulates oxidative stress.
  65. ncbi Dietary supplementation of herring roe and milt enhances hepatic fatty acid catabolism in female mice transgenic for hTNFα
    Bodil Bjørndal
    Institute of Medicine, University of Bergen, Bergen, Norway
    Eur J Nutr 51:741-53. 2012
  66. ncbi Increased beta-oxidation and improved oocyte developmental competence in response to l-carnitine during ovarian in vitro follicle development in mice
    Kylie R Dunning
    The Robinson Institute, School of Paediatrics and Reproductive Health, University of Adelaide, Adelaide, South Australia, Australia
    Biol Reprod 85:548-55. 2011
    ..we investigated whether upregulation of beta-oxidation by treatment with the fatty acid transport cofactor l-carnitine could improve folliculogenesis and developmental competence of mouse follicles following three-dimensional ..
  67. pmc Chronic oral ingestion of L-carnitine and carbohydrate increases muscle carnitine content and alters muscle fuel metabolism during exercise in humans
    Benjamin T Wall
    Department of Human Movement Sciences, Maastricht University, Maastricht, 6200 MD, The Netherlands
    J Physiol 589:963-73. 2011
    We have previously shown that insulin increases muscle total carnitine (TC) content during acute i.v. l-carnitine infusion...
  68. pmc Propionyl-L-carnitine corrects metabolic and cardiovascular alterations in diet-induced obese mice and improves liver respiratory chain activity
    Carmen Mingorance
    Department of Pharmacology, School of Pharmacy, University of Sevilla, Sevilla, Spain
    PLoS ONE 7:e34268. 2012
    ..The carnitine derivate, propionyl-L-carnitine (PLC), plays a key role in energy control...
  69. ncbi Endothelium-dependent vasorelaxation induced by L-carnitine in isolated aorta from normotensive and hypertensive rats
    Maria Dolores Herrera
    Department of Pharmacology, Faculty of Pharmacy, University of Seville, C Profesor Garcia Gonzalez s n, 41012 Seville, Spain
    J Pharm Pharmacol 54:1423-7. 2002
    The aim of this work was to investigate the mechanism of the vasodilatory effect induced by L-carnitine. Relaxation produced by L-carnitine was studied in rat aortic rings with and without functional endothelium, pre-contracted with ..
  70. ncbi Oligomeric structure of the carnitine transporter CaiT from Escherichia coli
    Kutti Ragunath Vinothkumar
    Max Planck Institute of Biophysics, Max von Laue Strasse 3, D 60438 Frankfurt am Main, Germany
    J Biol Chem 281:4795-801. 2006
    The carnitine transporter CaiT from Escherichia coli belongs to the betaine, choline, and carnitine transporter family of secondary transporters...
  71. ncbi L-carnitine supplementation reduces oocyte cytoskeleton damage and embryo apoptosis induced by incubation in peritoneal fluid from patients with endometriosis
    Gihan Mansour
    Reproductive Research Center, Department of Obstetrics and Gynecology, Cleveland Clinic, Cleveland, Ohio, USA
    Fertil Steril 91:2079-86. 2009
    To investigate the protective effect of L-carnitine (LC) against deleterious substances present in the peritoneal fluid (PF) of patients with endometriosis, which may affect the oocyte cytoskeleton and embryogenesis.
  72. ncbi Regulation of Octn2 transporter (SLC22A5) by peroxisome proliferator activated receptor alpha
    Tomoji Maeda
    Department of Membrane Transport and Pharmacokinetics, Faculty of Pharmaceutical Sciences, Tokyo University of Science, Noda, Chiba, Japan
    Biol Pharm Bull 31:1230-6. 2008
    The tissue distribution and disposition of carnitine, which plays an important role in the transport of long-chain fatty acids across the mitochondrial inner membrane for beta-oxidation, are well controlled by carnitine transporter ..
  73. ncbi Carnitine: transport and physiological functions in the brain
    Katarzyna A Nałęcz
    Department of Molecular and Cellular Neurobiology, Nencki Institute of Experimental Biology, 3 Pasteur Street, 02 093 Warszawa, Poland
    Mol Aspects Med 25:551-67. 2004
    b>Carnitine (4-N-trimethylammonium-3-hydroxybutyric acid), a compound necessary for a transfer of fatty acids for their oxidation within the cell, accumulates in brain although beta-oxidation of fatty acids is very low in neurons...
  74. pmc Altered carnitine homeostasis is associated with decreased mitochondrial function and altered nitric oxide signaling in lambs with pulmonary hypertension
    Shruti Sharma
    Program in Pulmonary Disease, Vascular Biology Center, Medical College of Georgia, Augusta, GA 30912, USA
    Am J Physiol Lung Cell Mol Physiol 294:L46-56. 2008
    ..The purpose of this study was to evaluate the possible role of a disruption in carnitine metabolism in shunt lambs and to determine the effect on NO signaling...
  75. pmc Glycosylation of the OCTN2 carnitine transporter: study of natural mutations identified in patients with primary carnitine deficiency
    Cristina Amat di San Filippo
    Department of Pediatrics and Pathology, University of Utah, Salt Lake City, UT 84132, USA
    Biochim Biophys Acta 1812:312-20. 2011
    Primary carnitine deficiency is caused by impaired activity of the Na(+)-dependent OCTN2 carnitine/organic cation transporter...
  76. ncbi L-carnitine as an ergogenic aid for patients with chronic obstructive pulmonary disease submitted to whole-body and respiratory muscle training programs
    A Borghi-Silva
    Laboratório de Fisioterapia Cardiovascular, Universidade Federal de Sao Carlos, Sao Carlos, SP, Brazil
    Braz J Med Biol Res 39:465-74. 2006
    The effects of adding L-carnitine to a whole-body and respiratory training program were determined in moderate-to-severe chronic obstructive pulmonary disease (COPD) patients...
  77. ncbi Diagnoses of newborns and mothers with carnitine uptake defects through newborn screening
    Ni Chung Lee
    Department of Medical Genetics, National Taiwan University Hospital, Taipei, Taiwan
    Mol Genet Metab 100:46-50. 2010
    b>Carnitine uptake defect (CUD) is an autosomal recessive fatty acid oxidation defect caused by a deficiency of the high-affinity carnitine transporter OCTN2...
  78. ncbi Carnitine synthesis and uptake into cells are stimulated by fasting in pigs as a model of nonproliferating species
    Robert Ringseis
    Institute of Agricultural and Nutritional Sciences, Martin Luther University of Halle Wittenberg, Emil Abderhalden Strasse 26, D 06108 Halle Saale, Germany
    J Nutr Biochem 20:840-7. 2009
    In rodents, fasting increases the carnitine concentration in the liver by an up-regulation of enzymes of hepatic carnitine synthesis and novel organic cation transporter (OCTN) 2, mediated by activation of peroxisome proliferator-..
  79. ncbi Ascorbic acid and carnitine biosynthesis
    C J Rebouche
    Department of Pediatrics, University of Iowa College of Medicine, Iowa City 52242
    Am J Clin Nutr 54:1147S-1152S. 1991
    It has been suggested that early features of scurvy (fatigue and weakness) may be attributed to carnitine deficiency...
  80. ncbi Effect of dietary lipid, carnitine and exercise on lipid profile in rat blood, liver and muscle
    Jyothsna Karanth
    Biochemistry and Nutrition Discipline, Defence Food Research Laboratory, Mysore 570 011, India
    Indian J Exp Biol 47:748-53. 2009
    ..the daily intake of vegetarian diet of either vegetable hydrogenated fat (HF) or peanut oil (PO) with or without carnitine on the lipid profile...
  81. ncbi Carnitine transport by organic cation transporters and systemic carnitine deficiency
    K Lahjouji
    Division of Medical Genetics, , 3175 Cote Sainte-Catherine, Montreal, Quebec H3T 1C5, Canada
    Mol Genet Metab 73:287-97. 2001
    ..Among these molecules, carnitine is an endogenous amine which is an essential cofactor for mitochondrial beta-oxidation...
  82. ncbi Novel localization of OCTN1, an organic cation/carnitine transporter, to mammalian mitochondria
    Anne Marie Lamhonwah
    Division of Neurology, Department of Pediatrics, University of Toronto, Ont, Canada
    Biochem Biophys Res Commun 345:1315-25. 2006
    b>Carnitine is a zwitterion essential for the beta-oxidation of fatty acids. We report novel localization of the organic cation/carnitine transporter, OCTN1, to mitochondria...
  83. ncbi Cancer and anticancer therapy-induced modifications on metabolism mediated by carnitine system
    G Peluso
    Institute of Protein Biochemistry and Enzymology I B P E CNR, Naples, Italy
    J Cell Physiol 182:339-50. 2000
    ..of fuel metabolism in response to internal and environmental stimuli is a vital task that requires an intact carnitine system...
  84. ncbi Effect of L-carnitine on liver cell membranes in ethanol-intoxicated rats
    Izabela Dobrzynska
    Institute of Chemistry, University in Białystok, Al Piłsudskiego 11 4, 15 443 Białystok, Poland
    Chem Biol Interact 188:44-51. 2010
    ..The interaction of food nutrients with ethanol may modulate alcohol toxicity. One such compound is l-carnitine (l-3-hydroxy-4-N,N,N-trimethylaminobutyrate), which is also an antioxidant...
  85. ncbi Carnitine acyltransferases and their influence on CoA pools in health and disease
    Rona R Ramsay
    Centre for Biomolecular Sciences, University of St Andrews, North Haugh, St Andrews KY16 9ST, Scotland, UK
    Mol Aspects Med 25:475-93. 2004
    ..cell compartments without wasting energy on futile cycles of hydrolysis and resynthesis is achieved through the carnitine system...
  86. ncbi Electrospray tandem mass spectrometry for analysis of acylcarnitines in dried postmortem blood specimens collected at autopsy from infants with unexplained cause of death
    D H Chace
    Neo Gen Screening, PO Box 219, Bridgeville, PA 15017, USA
    Clin Chem 47:1166-82. 2001
    ..Tandem mass spectrometry (MS/MS) may reveal disorders of fatty acid oxidation in deaths of previously unknown cause...
  87. ncbi Molecular enzymology of carnitine transfer and transport
    R R Ramsay
    Centre for Biomolecular Sciences, University of St Andrews, North Haugh, St Andrews KY16 9ST, UK
    Biochim Biophys Acta 1546:21-43. 2001
    b>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...
  88. ncbi Effect of genistein with carnitine administration on lipid parameters and obesity in C57Bl/6J mice fed a high-fat diet
    Ji Yeon Yang
    Research Center of Bioactive Materials, Chonbuk National University, Jeonju, Jeonbuk, Republic of Korea
    J Med Food 9:459-67. 2006
    ..We investigated the effect of dietary genistein (the principal soy isoflavone) alone and combined with L-carnitine to evaluate possible synergistic effects on the intentionally induced prediabetic state characterized by insulin ..
  89. ncbi Altered brain phospholipid and acylcarnitine profiles in propionic acid infused rodents: further development of a potential model of autism spectrum disorders
    Raymond H Thomas
    The Kilee Patchell Evans Autism Research Group, Department of Psychology and Biology, University of Western Ontario, London, Ontario, Canada
    J Neurochem 113:515-29. 2010
    ..longchain (C12-C24) acylcarnitines, total short-chain (C2 to C9) acylcarnitines, and the ratio of bound to free carnitine were increased following infusions with PPA and BUT...
  90. ncbi Carnitine and type 2 diabetes
    Randall L Mynatt
    Pennington Biomedical Research Center, Baton Rouge, LA 70808, USA
    Diabetes Metab Res Rev 25:S45-9. 2009
    ..Hyperinsulemic/euglycemic clamp studies in humans and carnitine supplementation studies in rodents provide "proof-of-concept" that carnitine is effective at improving insulin-..
  91. pmc High heritability of metabolomic profiles in families burdened with premature cardiovascular disease
    Svati H Shah
    Department of Medicine, Duke University Medical Center, Durham, NC 27710, USA
    Mol Syst Biol 5:258. 2009
    ..27). We report a novel finding of high heritabilities of metabolites in premature CAD, establishing a possible genetic basis for these profiles. These results have implications for understanding CAD pathophysiology and genetics...
  92. ncbi Early nerve ending rescue from oxidative damage and energy failure by L: -carnitine as post-treatment in two neurotoxic models in rat: recovery of antioxidant and reductive capacities
    Diana Elinos-Calderón
    Laboratorio de Aminoácidos Excitadores, Instituto Nacional de Neurologia y Neurocirugia Manuel Velasco Suarez, Insurgentes Sur 3877, C P 14269, Mexico D F, Mexico
    Exp Brain Res 197:287-96. 2009
    ..In this work, the effects of the well-known antioxidant and energy precursor, L: -carnitine (L: -CAR), were tested as a post-treatment in two neurotoxic models under in vitro and in vivo conditions...
  93. ncbi Neuroprotective effects of L-carnitine in a transgenic animal model of Huntington's disease
    Eniko Vamos
    Department of Neurology, Albert Szent Gyorgyi Clinical Centre, University of Szeged, Semmelweis str u 6, 6725 Szeged, Hungary
    Biomed Pharmacother 64:282-6. 2010
    ..L-carnitine plays a role in facilitating the mitochondrial transport of fatty acids, but it also protects the cells from ..
  94. pmc Biochemical correction of short-chain acyl-coenzyme A dehydrogenase deficiency after portal vein injection of rAAV8-SCAD
    Stuart G Beattie
    University of Massachusetts Medical School, Worcester, MA 01655, USA
    Hum Gene Ther 19:579-88. 2008
    ..These results demonstrate biochemical correction of SCAD deficiency after AAV8-mediated SCAD gene delivery...
  95. ncbi Quantitative analysis of the dynamic signaling pathway involved in the cAMP mediated induction of l-carnitine biosynthesis in E. coli cultures
    José Hormiga
    Grupo de Tecnología Bioquímica, Departamento de Bioquimica y Biologia Molecular, Facultad de Biologia, Universidad de La Laguna, 38206, La Laguna, Tenerife, Islas Canarias, Spain
    Mol Biosyst 6:699-710. 2010
    L-(-)-carnitine can be synthesized from waste bioprecursors in the form of crotonobetaine. Such biotransformation is carried out in E. coli by the enzymes encoded by operons regulated by the cAMP receptor proteins...
  96. ncbi Expanded newborn screening identifies maternal primary carnitine deficiency
    Lisa A Schimmenti
    University of Minnesota, Department of Pediatrics, Division of Genetics and Metabolism, Institute of Human Genetics, Minneapolis, MN, USA
    Mol Genet Metab 90:441-5. 2007
    Primary carnitine deficiency impairs fatty acid oxidation and can result in hypoglycemia, hepatic encephalopathy, cardiomyopathy and sudden death...
  97. ncbi Growth characteristics, blood metabolites, and insulin-like growth factor system components in maternal tissues of gilts fed L-carnitine through day seventy of gestation
    K R Brown
    Department of Animal Sciences and Industry, Kansas State University, Manhattan, KS 66506 0201, USA
    J Anim Sci 85:1687-94. 2007
    A total of 59 gilts (BW = 137.7 kg) from 3 breeding groups were used to assess the effects of feeding l-carnitine during gestation on gilt growth characteristics, blood metabolites, and uterine and chorioallantoic expression of IGF axis ..
  98. ncbi L-carnitine inhibits hepatocarcinogenesis via protection of mitochondria
    BaoJun Chang
    Department of Biochemistry and Molecular Pathology, Osaka City University Medical School, Osaka, Japan
    Int J Cancer 113:719-29. 2005
    ..Furthermore, we proved that L-carnitine effectively protects mitochondrial function in vivo...
  99. ncbi Urinary excretion of L-carnitine and its short-chain acetyl-L-carnitine in patients undergoing carboplatin treatment
    Angelo Mancinelli
    Scientific Department, Sigma Tau, Via Pontina Km 30, 400, Pomezia, Italy
    Cancer Chemother Pharmacol 60:19-26. 2007
    To evaluate the effect of the anti-cancer drug carboplatin on plasma concentrations and urinary excretion of L-carnitine (LC) and its main ester, acetyl-L-carnitine (ALC), in cancer patients.
  100. ncbi Plasma and urinary carnitine and acylcarnitines in chronic fatigue syndrome
    Mark G Jones
    St George s Hospital Medical School, Cranmer Terrace, London, SW17 0RE, UK
    Clin Chim Acta 360:173-7. 2005
    Contradictory reports have suggested that serum free carnitine and acylcarnitine concentrations are decreased in patients with chronic fatigue syndrome (CFS) and that this is a cause of the muscle fatigue observed in these patients...
  101. ncbi Effects of simvastatin and carnitine versus simvastatin on lipoprotein(a) and apoprotein(a) in type 2 diabetes mellitus
    Fabio Galvano
    University of Catania, Department of Biological Chemistry, Medical Chemistry and Molecular Biology, Viale A Doria 6, 95125 Catania, Italy
    Expert Opin Pharmacother 10:1875-82. 2009
    The aim of the present study was to compare the effects of simvastatin and L-carnitine coadministration versus simvastatin monotherapy on lipid profile, lipoprotein(a) (Lp(a)) and apoprotein(a) (Apo(a)) levels in type II diabetic patients.

Research Grants63

  1. Prevention of Complications in Thalassemia
    Elliott P Vichinsky; Fiscal Year: 2011
    ..with thalassemia intermedia, 4) Antioxidant therapy with a combination of RS-Alpha-Lipoic Acid (LA) and L-Carnitine (ALCAR) will produce a measurable decline in oxidative stress in patients with thalassemia, 5) Increased ..
  2. Impact of the P479L Variant in CPT1A on Infant Mortality in Alaska
    David M Koeller; Fiscal Year: 2011 tandem mass spectrometry (MS/MS), which led to the identification of an unexpectedly high incidence of carnitine palmitoyltransferase 1A (CPT1A) deficiency, a rare disorder of fatty acid oxidation...
  3. Structural biology of enzymes in fatty acid metabolism
    Liang Tong; Fiscal Year: 2012
    Acetyl-coenzyme A (acetyl-CoA) carboxylases (ACCs), pyruvate carboxylase (PC), carnitine acyltransferases, and AMP-activated protein kinase (AMPK) play crucial roles in the metabolism of fatty acids and/or carbohydrates, as well as ..
  4. Metabolic Dysfunction and Neuroprotection after Pediatric Head Injury
    Susanna Scafidi; Fiscal Year: 2013
    ..e. 2-hydroxybutyrate (BHB) and acetyl-L-carnitine (ALCAR), that bypass the inhibition of pyruvate metabolism...
  5. Mechanisms of tubular atrophy in renal disease
    Jeffrey R Schelling; Fiscal Year: 2012
    ..The impact of rate-limiting, NEFA-metabolizing enzymes acyl CoA synthetase, acyl CoA thioesterase and carnitine palmitoyl transferase will be addressed using pharmacologic inhibitors and knockout mice...
  6. L-carnitine Treatment for Vasopressor Dependent Septic Shock
    Alan E Jones; Fiscal Year: 2013
    ..L-carnitine provides the key elements of a novel therapy to ameliorate the adverse hemodynamic effects of sepsis...
    GEORGIRENE VLADUTIU; Fiscal Year: 2012
    ..04). Up to 16- and 10-fold increases in mutant allele frequencies exist for McArdle disease and carnitine palmitoyltransferase II deficiency, respectively...
  8. Effects of Dietary Fat on Contractile and Mitochondrial Function in Heart Failure
    Margaret P Chandler; Fiscal Year: 2010
    ..mRNA and protein expression and activities of enzymes regulating FA metabolic pathways (eg PPAR alpha, PGC-1 alpha, carnitine palmitoyl transferase-l) and activities and modifications of the individual ETC complexes will be examined.
  9. Role of microRNAs in lipid metabolism and cardiovascular disease
    Carlos Fernandez Hernando; Fiscal Year: 2013
    ..For example, miR-33 targets carnitine O- octaniltransferase (CROT), Carnitine palmitoyltransferase 1A (CPT1a) and hydroxyacyl-CoA- dehydrogenase (..
  10. Cardiac metabolism and heart failure
    Rong Tian; Fiscal Year: 2010
    ..First, we seek to enhance fatty acid entry into mitochondria via muscle carnitine palmitoyl transferease-1 (mCPT-1), the rate-limiting step of long chain fatty acid oxidation, by lowering ..
  11. Metabolic &Developmental Aspects of Intellectual Disability
    Mary C McKenna; Fiscal Year: 2013
    ..achieved by avoiding unnecessary hyperoxia, stimulating aerobic energy metabolism by administration of acetyl-L-carnitine, protecting lipid rafts, genomic post-conditioning against secondary oxidative stress by administration of ..
  12. Regulated Expression of Hypothalamic CPT1 Using Gutless Adenovirus
    Silvana Obici; Fiscal Year: 2010
    ..state malonyl CoA acts as a sensor of fuel abundance because of its potent allosteric inhibition of the enzyme carnitine palmitoyltransferase 1 (CPT1), which controls the entry of fatty acids into mitochondria where they undergo ..
    Philip A Wood; Fiscal Year: 2010
    ..specific aims of this competing continuation project include: (1) Develop and characterize new mouse models for carnitine palmitoyltransferase-1b (muscle isoform) deficiency...
  14. The Role of Mitochondrial Dysfunction in Non-Alcoholic Fatty Liver Disease
    Jeffrey D Browning; Fiscal Year: 2013 subjects with NAFLD, implying that mitochondrial FA 2-oxidation is inhibited via the effect of malonyl-CoA on carnitine palmitoyl transferase...
    John H Griffin; Fiscal Year: 2013
    ..The anticoagulant soluble lipids include palmitoyl carnitine and related long chain acyl carnitines and lyso-sulfatide...
  16. Pathogenesis of Ketosis Prone Diabetes
    Ashok Balasubramanyam; Fiscal Year: 2010
    ..patients have lower concentrations of total fatty acids and acyl carnitines, higher concentrations of an acyl carnitine marker of beta-hydroxybutyrate, and markedly higher concentrations of glutamine / glutamate, together with ..
  17. Inhibition of CPT-1b in muscle: effects on glucose homeostasis
    RANDALL LEE MYNATT; Fiscal Year: 2013
    ..Others report lipid oversupply via a high fat diet can actually increase FAO to the extent that carnitine and TCA cycle intermediates are limiting, leading to mitochondrial abnormalities and skeletal muscle insulin ..
  18. Carnitine Acetyltransferase in Defending Mitochondrial and Metabolic Function
    Deborah M Muoio; Fiscal Year: 2013
    DESCRIPTION (provided by applicant): This project seeks to elucidate the role of carnitine acetyltransferase (CrAT) as an important regulator of mitochondrial function and glucose tolerance, and the principal mediator of the ..
  19. Perinatal Pulmonary Hypertension: Developmental Mediators
    Jeffrey R Fineman; Fiscal Year: 2013
    ..relevant lamb model of CHD, we have generated compelling data demonstrating that increased PBF impairs carnitine homeostasis, in association with mitochondrial dysfunction, decreased HSP90/eNOS interactions, increased ..
    Tory M Hagen; Fiscal Year: 2011
    ..Feeding old rats acetyl-L-carnitine (ALCAR) reverses IFM dyslipidemia, while (R)-a-lipoic acid (LA) lowers oxidative stress in IFM and the aging ..
  21. Mechanisms of Neuroprotection by Astrocytes in Alcohol Abuse
    James Haorah; Fiscal Year: 2012
    ..of acetaldehyde as transducer for oxidative stress, a therapeutic stabilization of astrocytic ALDHs by acetyl-L-carnitine (ALC) is an innovative approach, which has not been studied so far...
  22. Coenzyme A Regulation of Metabolism
    Suzanne Jackowski; Fiscal Year: 2013
    ..metabolism, employed a chemical biology approach to identify small molecule PanK inhibitors, and identified acyl-carnitine as a PanK activator...
  23. MiR-33 and Aging: Implications for Metabolic Syndrome
    Leigh Goedeke; Fiscal Year: 2013
    ..miR-33 binds and represses key enzymes involved in the regulation of fatty acid oxidation, including carnitine O-octaniltransferase (CROT), carnitine palmitoyltransferase 1A (CPT1a), and hydroxyacyl-CoA-dehydrogenase (HADHB)..
  24. Aerobic fitness, mitochondrial dysfunction, and fatty liver disease
    John P Thyfault; Fiscal Year: 2013
    ..of peroxisome proliferator gamma co-activator 1 alpha (PGC-1, to stimulate mitochondrial biogenesis) or carnitine palmitoyltransferase-1 (CPT-1, to enhance FAOX in existing mitochondria) in LCR livers or primary hepatocytes ..
  25. Acyl-CoA Synthetase: Structure, Function and Regulation
    ROSALIND ANNE COLEMAN; Fiscal Year: 2013 to direct FA towards [unreadable]-oxidation in highly oxidative tissues because the enzyme interacts with carnitine acyltranferase to hand off its acyl-CoA product;2) that the function of ACSL1 differs in liver because at least ..
  26. Mechanisms of Peripheral mGluR Modulation of Nociception
    Robert W Gereau; Fiscal Year: 2013
    ..demonstrated that enhancing this pathway by promoting epigenetic upregulation of mGlu2 expression with L-acetyl Carnitine (LAC) therapy or using histone deacetylase (HDAC) inhibitors like SAHA (Vorinostat) can promote resolution of ..
    E Douglas Lewandowski; Fiscal Year: 2013
    ..and storage, with apparent effects on contractility;2) increased expression of the liver (L) isoform of carnitine palmitoyltransferase I (L-CPT1) in hypertrophied hearts that coincides with reduced fatty acid oxidation;3) ..
  28. Role of Carnitine Acetyltansferase in Combatting Nutrient Stress
    Michael Davies; Fiscal Year: 2013
    DESCRIPTION (provided by applicant): Carnitine acetyltransferase (CrAT) is a freely reversible mitochondrial matrix enzyme that catalyzes the exchange of short-chain acyl groups between CoA and carnitine...
  29. Mitochondrial-Targeted Therapeutics for Treatment of Spinal Cord Injury
    Patrick G Sullivan; Fiscal Year: 2013
    ..amide form of N-acetylcysteine), as well as an alternative biofuel substrate for energy production, acetyl-l-carnitine (ALC), following SCI...
  30. Can L-carnitine prevent drug-induced hearing loss?
    Gilda Kalinec; Fiscal Year: 2004
    ..In preliminary experiments, we found that the natural micronutrient L-carnitine (LCAR), which is required for normal mitochondrial function, prevented perinatal cisplatin-induced sensorineural ..
  31. The effect of ACC2 deletion on cardiac metabolism and function
    Stephen C Kolwicz; Fiscal Year: 2011
    ..As malonyl CoA levels rise, fatty acid transport through carnitine palmitoyl transferase I (CPTI) is inhibited...
    William Heird; Fiscal Year: 2001
    ..e., glycerol-3-phosphate acyltransferase), increase the rate-limiting enzymes of mitochondrial (i.e., carnitine palmitoyl transferase) and peroxisomal fatty acid oxidation (i.e...
    David Otto; Fiscal Year: 1990
    ..A corollary to this hypothesis is that omega-3 fatty acids also cause an adaptive decrease in the sensitivity of carnitine palmitoyltransferase I to inhibition by malony1-CoA, and an increase in the capacity for peroxisomal fatty acid ..
  34. Mutations Associated with Carnitine Deficiency: Risk Factor for Regression in ASD
    ROBIN P KOCHEL; Fiscal Year: 2013
    ..In the search for an ASD etiological mechanism, researchers have noted significantly reduced levels of carnitine among those with ASD...
  35. Parenteral Medium Chain Triglycerides in the Premature
    Eberhard Schmidt Sommerfeld; Fiscal Year: 2002
    ..beneficial for brain development, but could also cause toxicity; and (2) MCT decrease the availability of free carnitine, resulting in an accumulation of short- and medium-chain acyl-coenzyme A and impairment of energy metabolism if ..
    Linda Brady; Fiscal Year: 1993
    b>Carnitine palmitoyltransferase (CPT) is a regulatory membrane enzyme which controls the entry of long-chain fatty acids into the mitochondria for beta-oxidation...
    LORAN BIEBER; Fiscal Year: 1993
    A role for carnitine palmitoyltransferase, CPT, in mitochondrial beta- oxidation of long-chain fatty acids is well-established and the evidence for a role for mitochondrial carnitine acetyltransferase, CAT, in modulation of the short-..
    HOWARD GLAUERT; Fiscal Year: 1993
    ..increased expression of several proteins, including the enzymes of the peroxisomal beta-oxidation pathway, the carnitine acyltransferases, and cytochrome P-450IVA1...
    Stanley Blumenthal; Fiscal Year: 1980
    ..long-chain fatty acids may augment gluconeogenesis, even when their oxidation is blocked by octanoyl - D (plus) carnitine. Therefore it is conceivable that the intact fatty acid molecule, itself - or its CoA ester - stimulated ..
    William Rhead; Fiscal Year: 1991
    ..After carnitine (CN) loading, we will measure urine and plasma organic acids and acyl-CNs in EBO patients and quantitate acyle-..
    Victoria Esser; Fiscal Year: 2004
    The mitochondrial oxidation of long chain fatty acids is regulated in large part at the level of carnitine palmitoyltransferase I (CPT I) located on the mitochondrial outer membrane...
    Donghai Wu; Fiscal Year: 2000
    ..For instance, carnitine palmitoylransferase I and II are essential for b-oxidation of long chain fatty acids in mitochondria and are ..
    DIANE HICKSON BICK; Fiscal Year: 2002
    ..Patients with genetic carnitine palmitoyltransferase (CPT) deficiencies die in cardiac arrhythmia...
    Owen Griffith; Fiscal Year: 1990
    ..S. Grisolia et al. recently reported that ammonia is much less toxic to mice previously given L-carnitine, a mitochondrial transport carrier for long-chain fatty acids. The protective mechanism is unknown...
  45. Phase II Developmental Study on Fatigue in AIDS Patients
    RICARDO CRUCIANI; Fiscal Year: 2003
    ..Our preliminary data suggest that micronutrient deficiencies, specifically carnitine deficiency, may be an important factor in fatigue. Carnitine plays a major role in energy metabolism...
    GEORGE COOK; Fiscal Year: 2003
    ..Fatty acid oxidation inhibitors [especially carnitine palmitoyltransferase-I (CPT-I) inhibitors] increase glucose oxidation and limit fatty acid oxidation in the ..
  47. Activators of Adipocyte Fatty Acid Oxidation
    CYDNEY BROOKS; Fiscal Year: 2003
    ..The enzyme carnitine palmitoyltransferase I (CPT I) is the critical control point for fatty acid metabolism in the cell and provides ..
    William Stanley; Fiscal Year: 2003
    ..The transport of long-chain fatty acids into the mitochondrion is regulated by the activity of carnitine palmitoyl transferase I (CPT-I), which is inhibited by malonyl-CoA...
  49. Role of altered carnitine metabolism in perinatal endothelial dysfunction
    Stephen Black; Fiscal Year: 2009
    ..Recently we have found that decreases in NO signaling correlate with altered carnitine metabolism and mitochondrial dysfunction...
  50. Epidemiology of SBCAD Deficiency in Hmong-Americans
    Maureen Durkin; Fiscal Year: 2009
    ..routine newborn screening for SBCADD, neither the natural history of this deficiency nor the utility of early l-carnitine treatment and dietary intervention are known...
    Tod Gulick; Fiscal Year: 2005
    DESCRIPTION (Applicant's abstract): Carnitine palmitoyltransferase I (CPT-1) catalyzes the rate-limiting step in mitochondrial FA oxidation...
    ARUN TAHILIANI; Fiscal Year: 1990
    ..Because Coenzyme A (CoA) and carnitine are essential cofactors of fatty acid metabolism, the work has, in recent years, been directed toward ..
  53. Regulation of Cardiac Carnitine Palmitoyltransferase I
    Gebre Woldegiorgis; Fiscal Year: 2004
    ..Fatty acid-induced injury to the ischemic myocardium can be reduced by inhibition of carnitine palmitoyltransferase I (CPTI), a rate-limiting enzyme in beta-oxidation...
    Vadivel Ganapathy; Fiscal Year: 2003
    ..are to investigate the structure, function, and regulation of plasma membrane transporters responsible for carnitine homeostasis and to understand the relevance of these transporters to the genetic disorders ofcarnitine ..
    PAUL WIESER; Fiscal Year: 1980
    Previous studies in our laboratory have shown that carnitine is able to stimulate glycerol release from adipose tissue of human newborns and to potentiate glycerol release in the presence of lipolytic agents in adult adipose tissue and ..
    Robert Wolfe; Fiscal Year: 2002
    ..The capacity for transport of fatty acid into the mitochondria is determined by the activity of the enzyme carnitine palmitoyltransferase 1 (CPT-I), and at any given activity of CPT-I, the availability of fatty acids within the ..
  57. Liposomal L-Carnitine
    HANS HOFLAND; Fiscal Year: 2002
    ..Given orally, L-carnitine has shown great promise in clinical trials, however, its bloavailability is poor...
  58. Functional analysis and regulation of epididymal OCTN2
    BARRY HINTON; Fiscal Year: 2007
    ..g. sodium-myo inositol cotransporter which transports the osmolyte myo-inositol. L-carnitine is another osmolyte that plays a key role in the adaption of hyperosmolality and is found in high concentrations ..
    CHRISTINE WILCOX; Fiscal Year: 1993
    ..The initial focus will be the study of the effects of acetyl-L-carnitine (ALC) and nerve growth factor (NGF)...
    KURT SAUPE; Fiscal Year: 1999
    ..Many age-associated changes in mitochondrial function can be reversed with dietary supplementation of carnitine derivatives such as acetyl-L- carnitine, a critical co-factor in fatty acid metabolism...
    CHARLES REBOUCHE; Fiscal Year: 1990
    Normal term infants will be studied to determine whether consumption of a carnitine-free diet is associated with evidence of impaired ability to oxidize long-chain fatty acids...
    LORRAINE TURCOTTE; Fiscal Year: 2002
    ..Malonyl-CoA, a potent inhibitor of carnitine palmitoyl transferase, is formed from acetyl-CoA in a carboxylation reaction catalyzed by acetyl-CoA carboxylase...
    William Stanley; Fiscal Year: 2001
    ..The rate of fatty acid oxidation in cardiomyocytes is primarily controlled by the activity of carnitine palmitoyl transferase I (CPT I)...