ArticleMetabolic brain disease2015
Investigation of inflammatory profile in MSUD patients: benefit of L-carnitine supplementation.
Article in Metabolic brain disease, 2015. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 21 papers.
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Who cites it
21 citing papers in PubMed, 41 citations in OpenAlex.
- Full-spectrum cannabidiol-rich oil modulates behavior and neurochemical alterations in a rodent model of maple syrup urine disease.Metabolic brain disease · 2026Article
- 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
- Association of the gut microbiome with diabetic nephropathy and the mediated effect of metabolites: friend or enemy?International urology and nephrology · 2025Article
- Neurodegenerative biomarkers and inflammation in patients with propionic and methylmalonic acidemias: effect of L-carnitine treatment.Metabolic brain disease · 2024Article
- Leucine-Enriched Diet Reduces Fecal MPO but Does Not Protect Against DSS Colitis in a Mouse Model of Crohn's Disease-like Ileitis.International journal of molecular sciences · 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
- The association between dietary intake of branched-chain amino acids and odds and severity of rheumatoid arthritis.Scientific reports · 2024Article
- Memantine Improves Memory and Neurochemical Damage in a Model of Maple Syrup Urine Disease.Neurochemical research · 2024Article
- Melatonin improves behavioral parameters and oxidative stress in zebrafish submitted to a leucine-induced MSUD protocol.Metabolic brain disease · 2023Article
- Acute effects of intracerebroventricular administration of α-ketoisocaproic acid in young rats on inflammatory parameters.Metabolic brain disease · 2023Article
- Interplay Between Reactive Oxygen/Reactive Nitrogen Species and Metabolism in Vascular Biology and Disease.Antioxidants & redox signaling · 2021Review
- The effects of L-carnitine supplementation on indicators of inflammation and oxidative stress: a systematic review and meta-analysis of randomized controlled trials.Journal of diabetes and metabolic disorders · 2020Review
- Investigation of L - Carnitine Concentrations in Treated Patients with Maple Syrup Urine Disease.Journal of pediatric genetics · 2019Article
- Paroxysmal spasticity of lower extremities as the initial symptom in two siblings with maple syrup urine disease.Molecular medicine reports · 2019Article
- Branched-chain amino acids promote endothelial dysfunction through increased reactive oxygen species generation and inflammation.Journal of cellular and molecular medicine · 2018Article
- Evaluation of plasma biomarkers of inflammation in patients with maple syrup urine disease.Journal of inherited metabolic disease · 2018Article
- Altered Redox Homeostasis in Branched-Chain Amino Acid Disorders, Organic Acidurias, and Homocystinuria.Oxidative medicine and cellular longevity · 2018Review
- Serum Markers of Neurodegeneration in Maple Syrup Urine Disease.Molecular neurobiology · 2017Article
- "Classical organic acidurias": diagnosis and pathogenesis.Clinical and experimental medicine · 2017Review
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Corrections and comments
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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) is a metabolic disorder caused by a severe deficiency of the branched-chain α-keto acid dehydrogenase complex activity which leads to the accumulation of branched-chain amino acids (BCAA) leucine (Leu), isoleucine and valine and their respective α-keto-acids in body fluids. The main symptomatology presented by MSUD patients includes ketoacidosis, failure to thrive, poor feeding, apnea, ataxia, seizures, coma, psychomotor delay and mental retardation, but, the neurological pathophysiologic mechanisms are poorly understood. The treatment consists of a low protein diet and a semi-synthetic formula restricted in BCAA and supplemented with essential amino acids. It was verified that MSUD patients present L-carnitine (L-car) deficiency and this compound has demonstrated an antioxidant and anti-inflammatory role in metabolic diseases. Since there are no studies in the literature reporting the inflammatory profile of MSUD patients and the L-car role on the inflammatory response in this disorder, the present study evaluates the effect of L-car supplementation on plasma inflammatory cytokines interleukin-1β (IL-1β), interleukin-6 (IL-6), interferon-gamma (INF-ɣ), and a correlation with malondialdehyde (MDA), as a marker of oxidative damage, and with free L-car plasma levels in treated MSUD patients. Significant increases of IL-1β, IL-6, and INF-ɣ were observed before the treatment with L-car. Moreover, there is a negative correlation between all cytokines tested and L-car concentrations and a positive correlation among the MDA content and IL-1β and IL-6 values. Our data show that L-car supplementation can improve cellular defense against inflammation and oxidative stress in MSUD patients and may represent an additional therapeutic approach to the patients affected by this disease.
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