ArticleMolecular neurobiology2016
L-carnitine Prevents Oxidative Stress in the Brains of Rats Subjected to a Chemically Induced Chronic Model of MSUD.
Article in Molecular neurobiology, 2016. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 23 papers.
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Who cites it
23 citing papers in PubMed, 44 citations in OpenAlex.
- Neuroprotective, antioxidant and anti-inflammatory effect of carnitine in patients with Maple syrup urine disease: branched-chain amino acids and branched-chain keto acids levels.Metabolic brain disease · 2025Article
- Minocycline Protects Against Oxidative Stress in a Model of Maple Syrup Urine Disease.Neurochemical research · 2025Article
- Neuroprotective effects of L-carnitine towards oxidative stress and inflammatory processes: a review of its importance as a therapeutic drug in some disorders.Metabolic brain disease · 2025Review
- α-Ketoisocaproic Acid Disrupts Mitochondrial Bioenergetics in the Brain of Neonate Rats: Molecular Modeling Studies of α-ketoglutarate Dehydrogenase Subunits Inhibition.Neurochemical research · 2025Article
- Combination of Gold Nanoparticles with Carnitine Attenuates Brain Damage in an Obesity Animal Model.Molecular neurobiology · 2024Article
- In Vivo Intracerebral Administration of α-Ketoisocaproic Acid to Neonate Rats Disrupts Brain Redox Homeostasis and Promotes Neuronal Death, Glial Reactivity, and Myelination Injury.Molecular neurobiology · 2024Article
- Memantine Improves Memory and Neurochemical Damage in a Model of Maple Syrup Urine Disease.Neurochemical research · 2024Article
- L-Carnitine Prevents Behavioural Alterations in Ketamine-Induced Schizophrenia in Mice: Possible Involvement of Oxidative Stress and Inflammation Pathways.Journal of toxicology · 2023Article
- Exposure to leucine induces oxidative stress in the brain of zebrafish.Metabolic brain disease · 2022Article
- L-carnitine protects DNA oxidative damage induced by phenylalanine and its keto acid derivatives in neural cells: a possible pathomechanism and adjuvant therapy for brain injury in phenylketonuria.Metabolic brain disease · 2021Article
- Preliminary results of PBA-loaded nanoparticles development and the effect on oxidative stress and neuroinflammation in rats submitted to a chemically induced chronic model of MSUD.Metabolic brain disease · 2021Article
- Administration of branched-chain amino acids alters epigenetic regulatory enzymes in an animal model of Maple Syrup Urine Disease.Metabolic brain disease · 2021Article
- Brain Branched-Chain Amino Acids in Maple Syrup Urine Disease: Implications for Neurological Disorders.International journal of molecular sciences · 2020Review
- Melatonin ameliorates oxidative stress and DNA damage of rats subjected to a chemically induced chronic model of Maple Syrup Urine Disease.Metabolic brain disease · 2020Article
- The effects of L-carnitine supplementation on lipid concentrations inpatients with type 2 diabetes: A systematic review and meta-analysis of randomized clinical trials.Journal of cardiovascular and thoracic research · 2020Review
- Effects of Co-Administration of Bone Marrow Stromal Cells and L-Carnitine on The Recovery of Damaged Ovaries by Performing Chemotherapy Model in Rat.International journal of fertility & sterility · 2019Article
- Investigation of L - Carnitine Concentrations in Treated Patients with Maple Syrup Urine Disease.Journal of pediatric genetics · 2019Article
- Silico analysis of a novel mutation c.550delT in a Chinese patient with maple syrup urine disease.Clinical case reports · 2018Article
- Branched-chain amino acids promote endothelial dysfunction through increased reactive oxygen species generation and inflammation.Journal of cellular and molecular medicine · 2018Article
- Chronic Exposure to β-Alanine Generates Oxidative Stress and Alters Energy Metabolism in Cerebral Cortex and Cerebellum of Wistar Rats.Molecular neurobiology · 2018Article
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Authors and funding
10 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Maple syrup urine disease (MSUD), or branched-chain α-keto aciduria, is an inherited disorder that is caused by a deficiency in branched-chain α-keto acid dehydrogenase complex (BCKAD) activity. Blockade of this pathway leads to the accumulation of the branched-chain amino acids (BCAAs), leucine, isoleucine, and valine, and their respective ketoacids in tissues. The main clinical symptoms presented by MSUD patients include ketoacidosis, hypoglycemia, opisthotonos, poor feeding, apnea, ataxia, convulsions, coma, psychomotor delay, and mental retardation. Although increasing evidence indicates that oxidative stress is involved in the pathophysiology of this disease, the mechanisms of the brain damage caused by this disorder remain poorly understood. In the present study, we investigated the effect of BCAAs on some oxidative stress parameters and evaluated the efficacy of L-carnitine (L-car), an efficient antioxidant that may be involved in the reduction of oxidative damage observed in some inherited neurometabolic diseases, against these possible pro-oxidant effects of a chronic MSUD model in the cerebral cortex and cerebellum of rats. Our results showed that chronic BCAA administration was able to promote both lipid and protein oxidation, impair brain antioxidant defenses, and increase reactive species production, particularly in the cerebral cortex, and that L-car was able to prevent these effects. Taken together, the present data indicate that chronic BCAA administration significantly increased oxidative damage in the brains of rats subjected to a chronic model of MSUD and that L-car may be an efficient antioxidant in this disorder.
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