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In vivo neuroprotective effect of L-carnitine against oxidative stress in maple syrup urine disease

Content Provider	Springer Nature Link
Author	Mescka, Caroline Moraes, Tarsila Rosa, Andrea Mazzola, Priscila Piccoli, Bruna Jacques, Carlos Dalazen, Giovana Coelho, Juliana Cortes, Marcelo Terra, Melaine Regla Vargas, Carmen Dutra Filho, Carlos S.
Copyright Year	2011
Abstract	Maple syrup urine disease (MSUD) is an autosomal recessive inborn error of metabolism caused by deficiency of the activity of the mitochondrial enzyme complex branched-chain α-keto acid dehydrogenase (BCKAD) leading to accumulation of the branched-chain amino acids (BCAA) leucine, isoleucine and valine and their corresponding branched-chain α-keto acids. Affected patients present severe brain dysfunction manifested such as ataxia, seizures, coma, psychomotor delay and mental retardation. The mechanisms of brain damage in this disease remain poorly understood. Recent studies have shown that oxidative stress may be involved in neuropathology of MSUD. L-Carnitine (L-Car) is considered a potential antioxidant through its action against peroxidation as a scavenger of reactive oxygen species and by its stabilizing effect of damage to cell membranes. In this study we evaluate the possible neuroprotective in vivo effects of L-Car against pro-oxidative effects of BCAA in cerebral cortex of rats. L-Car prevented lipoperoxidation, measured by thiobarbituric acid-reactive substances, protein damage, measured by sulfhydryl and protein carbonyl content and alteration on catalase and glutathione peroxidase activity in rat cortex from a chemically-induced model of MSUD. Our data clearly show that L-Car may be an efficient antioxidant, protecting against the oxidative stress promoted by BCAA. If the present results are confirmed in MSUD patients, this could represent an additional therapeutic approach to the patients affected by this disease.
Starting Page	21
Ending Page	28
Page Count	8
File Format	PDF
ISSN	08857490
Journal	Metabolic Brain Disease
Volume Number	26
Issue Number	1
e-ISSN	15737365
Language	English
Publisher	Springer US
Publisher Date	2011-03-05
Publisher Place	Boston
Access Restriction	One Nation One Subscription (ONOS)
Subject Keyword	Maple syrup urine disease L-carnitine Branched-chain amino acids Oxidative stress Neuroprotection Antioxidant Biochemistry Metabolic Diseases Neurology Oncology Neurosciences
Content Type	Text
Resource Type	Article
Subject	Biochemistry Neurology (clinical) Cellular and Molecular Neuroscience

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L-carnitine Prevents Oxidative Stress in the Brains of Rats Subjected to a Chemically Induced Chronic Model of MSUD

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In vivo neuroprotective effect of L-carnitine against oxidative stress in maple syrup urine disease

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Evidence that oxidative stress is increased in plasma from patients with maple syrup urine disease

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Inhibition of Brain Energy Metabolism by the Branched-chain Amino Acids Accumulating in Maple Syrup Urine Disease

In vivo neuroprotective effect of L-carnitine against oxidative stress in maple syrup urine disease

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