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Critical Reviews in Clinical Laboratory Sciences Volume 29, 1992 - Issue 3-4
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Research Article

Clinical and Biochemical Aspects of Carnitine Deficiency and Insufficiency: Transport Defects and Inborn Errors of β-Oxidation

Corrado Angelini Regional Neuromuscular Center, Neurological Clinic, University of Padova, Italy
,
Lodovica Vergani Regional Neuromuscular Center, Neurological Clinic, University of Padova, Italy
&
Andrea Martinuzzi Regional Neuromuscular Center, Neurological Clinic, University of Padova, Italy
Pages 217-242 | Published online: 27 Sep 2008

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Miroslav Cuturic, Ruth K. Abramson, Robert J. Breen, Alfred C. Edwards & Elliott E. Levy. (2016) Comparison of serum carnitine levels and clinical correlates between outpatients and acutely hospitalised individuals with bipolar disorder and schizophrenia: A cross-sectional study. The World Journal of Biological Psychiatry 17:6, pages 475-479.
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Miroslav Cuturic, Ruth K. Abramson, Robert R. Moran, James W. Hardin & Alicia V. Hall. (2011) Clinical correlates of low serum carnitine levels in hospitalized psychiatric patients. The World Journal of Biological Psychiatry 12:1, pages 73-79.
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Articles from other publishers (33)

Shin-ichi Uchiyama, Seigo Korematsu, Rieko Wasada, Kazuhide Imai, Atsumi Uemura, Misako Hiramatsu & Kazuya Goto. (2023) A case of Fukuyama-type congenital muscular dystrophy with acute carnitine deficiency triggered by fever, vomiting, and gastrointestinal bleeding. Nutrition 110, pages 112011.
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Venugopalan Rajesh, Annadurai Kokilavani, Subramanian Jayaseelan, Swaminathan Gomathi, Korrapati Vishali & Manni Venkatachari Kumudhavalli. (2023) Embryonic exposure to acetyl-L-carnitine protects against valproic acid-induced cardiac malformation in zebrafish model. Amino Acids 55:5, pages 619-638.
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Sijia Chen, Weidong Ye, Kendall D. Clements, Ziye Zan, Weishan Zhao, Hong Zou, Guitang Wang & Shangong Wu. (2022) Bacillus licheniformis FA6 Affects Zebrafish Lipid Metabolism through Promoting Acetyl-CoA Synthesis and Inhibiting β-Oxidation. International Journal of Molecular Sciences 24:1, pages 673.
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Akira Takahashi. (2021) The pathophysiology of leg cramping during dialysis and the use of carnitine in its treatment. Physiological Reports 9:21.
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Alina Kępka, Agnieszka Ochocińska, Sylwia Chojnowska, Małgorzata Borzym-Kluczyk, Ewa Skorupa, Małgorzata Knaś & Napoleon Waszkiewicz. (2021) Potential Role of L-Carnitine in Autism Spectrum Disorder. Journal of Clinical Medicine 10:6, pages 1202.
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Satoshi ODOKoji TANABEMasafumi YOHDA & Masamitsu YAMAUCHI. (2020) Liver-Oriented Acute Metabolic Effects of A Low Dose of L-Carnitine under Fat-Mobilizing Conditions: Pilot Human Clinical Trial. Journal of Nutritional Science and Vitaminology 66:2, pages 136-149.
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Goutham Vasam, Kimberly Reid, Yan Burelle & Keir J. Menzies. 2019. Mitochondria in Obesity and Type 2 Diabetes. Mitochondria in Obesity and Type 2 Diabetes 93 126 .
Carine Lindquist, Bodil Bjørndal, Christine Renate Rossmann, Asbjørn Svardal, Seth Hallström & Rolf Kristian Berge. (2018) A fatty acid analogue targeting mitochondria exerts a plasma triacylglycerol lowering effect in rats with impaired carnitine biosynthesis. PLOS ONE 13:3, pages e0194978.
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Stephanie E. Reuter & Allan M. Evans. (2012) Carnitine and Acylcarnitines. Clinical Pharmacokinetics 51:9, pages 553-572.
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Stephanie E. Reuter & Allan M. Evans. (2012) Carnitine and Acylcarnitines. Clinical Pharmacokinetics 51:9, pages 553-572.
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Yu Cao, Hai-jun Qu, Ping Li, Chun-bo Wang, Le-xin Wang & Zhi-wu Han. (2011) Single Dose Administration of L-Carnitine Improves Antioxidant Activities in Healthy Subjects. The Tohoku Journal of Experimental Medicine 224:3, pages 209-213.
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Nils Erik Gilhus, Michael P. Barnes & Michael BraininC. Angelini, A. Federico, H. Reichmann, A. Lombes, C. Verney Saban, P. Chinnery & J. Vissing. 2010. European Handbook of Neurological Management. European Handbook of Neurological Management 501 511 .
Miroslav Cuturic, Ruth K. Abramson, Robert R. Moran & James W. Hardin. (2010) Clinical Outcomes and Low-Dose Levocarnitine Supplementation in Psychiatric Inpatients With Documented Hypocarnitinemia: A Retrospective Chart Review. Journal of Psychiatric Practice 16:1, pages 5-14.
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Sherifa A. Hamed & Mostafa M. Abdella. (2009) The risk of asymptomatic hyperammonemia in children with idiopathic epilepsy treated with valproate: Relationship to blood carnitine status. Epilepsy Research 86:1, pages 32-41.
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Maren Fischer, Janine Keller, Frank Hirche, Holger Kluge, Robert Ringseis & Klaus Eder. (2009) Activities of γ-butyrobetaine dioxygenase and concentrations of carnitine in tissues of pigs. Comparative Biochemistry and Physiology Part A: Molecular & Integrative Physiology 153:3, pages 324-331.
Crossref
Gianfranco Fornasini, Richard N. Upton & Allan M. Evans. (2007) A pharmacokinetic model for L-carnitine in patients receiving haemodialysis. British Journal of Clinical Pharmacology 64:3, pages 335-345.
Crossref
Corrado Angelini. 2007. Myopathies. Myopathies 183 191 .
C. Angelini, A. Federico, H. Reichmann, A. Lombes, P. Chinnery & D. Turnbull. (2006) Task force guidelines handbook: EFNS guidelines on diagnosis and management of fatty acid mitochondrial disorders. European Journal of Neurology 13:9, pages 923-929.
Crossref
Georgirene D. Vladutiu, Zachary Simmons, Paul J. Isackson, Mark Tarnopolsky, Wendy L. Peltier, Alexandru C. Barboi, Naganand Sripathi, Robert L. Wortmann & Paul S. Phillips. (2006) Genetic risk factors associated with lipid-lowering drug-induced myopathies. Muscle & Nerve 34:2, pages 153-162.
Crossref
Tom L Broderick. (2006) Hypocarnitinaemia Induced by Sodium Pivalate in the Rat is Associated with Left Ventricular Dysfunction and Impaired Energy Metabolism. Drugs in R & D 7:3, pages 153-161.
Crossref
C. Angelini, A. Federico, H. Reichmann, A. Lombes, P. Chinnery & D. Turnbull. 2006. European Handbook of Neurological Management. European Handbook of Neurological Management 526 533 .
Werner Klingler, Frank Lehmann-Horn & Karin Jurkat-Rott. (2005) Complications of anaesthesia in neuromuscular disorders. Neuromuscular Disorders 15:3, pages 195-206.
Crossref
Barbara Marriage, M.Thomas Clandinin & D.Moira Glerum. (2003) Nutritional cofactor treatment in mitochondrial disorders. Journal of the American Dietetic Association 103:8, pages 1029-1038.
Crossref
Allan M Evans & Gianfranco Fornasini. (2003) Pharmacokinetics of L-Carnitine. Clinical Pharmacokinetics 42:11, pages 941-967.
Crossref
Simona Berardi, Bruno Stieger, Bruno Hagenbuch, Ernesto Carafoli & Stephan Krähenbühl. (2001) Characterization of L‐carnitine transport into rat skeletal muscle plasma membrane vesicles. European Journal of Biochemistry 267:7, pages 1985-1994.
Crossref
Juan P Infante & Virginia A Huszagh. (2000) Secondary carnitine deficiency and impaired docosahexaenoic (22:6 n -3) acid synthesis: a common denominator in the pathophysiology of diseases of oxidative phosphorylation and β-oxidation . FEBS Letters 468:1, pages 1-5.
Crossref
Aida N. Lteif & W.F. Schwenk. (1999) HYPOGLYCEMIA IN INFANTS AND CHILDREN. Endocrinology and Metabolism Clinics of North America 28:3, pages 619-646.
Crossref
Fernando Scaglia & Nicola Longo. (1999) Primary and secondary alterations of neonatal carnitine metabolism. Seminars in Perinatology 23:2, pages 152-161.
Crossref
Janos KernerCharles Hoppel. (1998) GENETIC DISORDERS OF CARNITINE METABOLISM AND THEIR NUTRITIONAL MANAGEMENT. Annual Review of Nutrition 18:1, pages 179-206.
Crossref
Dennis J. Paulson. 1998. Cardiac Metabolism in Health and Disease. Cardiac Metabolism in Health and Disease 33 41 .
A. Lee Carter, Tom O. Abney & David F. Lapp. (2016) Biosynthesis and Metabolism of Carnitine. Journal of Child Neurology 10:2_suppl, pages 2S3-2S7.
Crossref
. 1995. Metabolic and Degenerative Diseases of the Central Nervous System. Metabolic and Degenerative Diseases of the Central Nervous System 707 850 .
Shri V. Pande & Madiraju S.R. Murthy. (1994) Carnitine-acylcarnitine translocase deficiency: implications in human pathology. Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease 1226:3, pages 269-276.
Crossref

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