ReviewJournal of inherited metabolic disease2004
The role of oxidative damage in the neuropathology of organic acidurias: insights from animal studies.
Review in Journal of inherited metabolic disease, 2004. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 84 papers.
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Who cites it
84 citing papers in PubMed, 195 citations in OpenAlex.
- The Gut-Brain Axis in Autism: Inflammatory Mechanisms, Molecular Insights, and Emerging Microbiome-Based Therapies.Molecular neurobiology · 2025Review
- Minocycline Protects Against Oxidative Stress in a Model of Maple Syrup Urine Disease.Neurochemical research · 2025Article
- Oxidative damage and mitochondrial dysfunction in cystathionine beta-synthase deficiency.Scientific reports · 2024Article
- Metabolic Determinants of Cerebellar Circuit Formation and Maintenance.Cerebellum (London, England) · 2024Review
- IDH1 mutation produces R-2-hydroxyglutarate (R-2HG) and induces mir-182-5p expression to regulate cell cycle and tumor formation in glioma.Biological research · 2024Article
- Microbiota-derived short chain fatty acids in pediatric health and diseases: from gut development to neuroprotection.Frontiers in microbiology · 2024Review
- MicroRNA miR-223 modulates NLRP3 and Keap1, mitigating lipopolysaccharide-induced inflammation and oxidative stress in bovine mammary epithelial cells and murine mammary glands.Veterinary research · 2023Article
- Melatonin improves behavioral parameters and oxidative stress in zebrafish submitted to a leucine-induced MSUD protocol.Metabolic brain disease · 2023Article
- L-theanine attenuates LPS-induced motor deficit in experimental rat model of Parkinson's disease: emphasis on mitochondrial activity, neuroinflammation, and neurotransmitters.Psychopharmacology · 2023Article
- Tetrahydrobiopterin: Beyond Its Traditional Role as a Cofactor.Antioxidants (Basel, Switzerland) · 2023Review
- Biological Health Markers Associated with Oxidative Stress in Dairy Cows during Lactation Period.Metabolites · 2023Review
- Gut microbiota and Autism Spectrum Disorder: From pathogenesis to potential therapeutic perspectives.Journal of traditional and complementary medicine · 2023Review
- A review of probiotics in the treatment of autism spectrum disorders: Perspectives from the gut-brain axis.Frontiers in microbiology · 2023Review
- Disturbance of Mitochondrial Dynamics, Endoplasmic Reticulum-Mitochondria Crosstalk, Redox Homeostasis, and Inflammatory Response in the Brain of Glutaryl-CoA Dehydrogenase-Deficient Mice: Neuroprotective Effects of Bezafibrate.Molecular neurobiology · 2022Article
- Tissue Engineering Approaches to Uncover Therapeutic Targets for Endothelial Dysfunction in Pathological Microenvironments.International journal of molecular sciences · 2022Review
- Lactobacillus plantarum PS128 prevents cognitive dysfunction in Alzheimer's disease mice by modulating propionic acid levels, glycogen synthase kinase 3 beta activity, and gliosis.BMC complementary medicine and therapies · 2021Article
- The anti-inflammatory and antiapoptotic effects of probiotic on induced neurotoxicity in juvenile hamsters.Food science & nutrition · 2021Article
- Nuclear Factor Erythroid-2-Related Factor 2 Signaling in the Neuropathophysiology of Inherited Metabolic Disorders.Frontiers in cellular neuroscience · 2021Review
- Association of microbiota-derived propionic acid and Alzheimer's disease; bioinformatics analysis.Journal of diabetes and metabolic disorders · 2020Article
- Novel canine isocitrate dehydrogenase 1 mutation Y208C attenuates dimerization ability.Oncology letters · 2020Article
24 more citing papers are in PubMed but not listed here.
Corrections and comments
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Authors and funding
4 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Organic acidurias represent a group of inherited disorders resulting from deficient activity of specific enzymes of the catabolism of amino acids, carbohydrates or lipids, leading to tissue accumulation of one or more carboxylic (organic) acids. Patients affected by organic acidurias predominantly present neurological symptoms and structural brain abnormalities, of which the aetiopathogenesis is poorly understood. However, in recent years increasing evidence has emerged suggesting that oxidative stress is possibly involved in the pathology of some organic acidurias and other inborn errors of metabolism. This review addresses some of the recent developments obtained mainly from animal studies indicating oxidative damage as an important determinant of the neuropathophysiology of some organic acidurias. Recent data showing that various organic acids are capable of inducing free radical generation and decreasing brain antioxidant defences is presented. The discussion focuses on the relatively low antioxidant defences of the brain and the vulnerability of this tissue to reactive species. This offers new perspectives for potential therapeutic strategies for these disorders, which may include the early use of appropriate antioxidants as a novel adjuvant therapy, besides the usual treatment based on removing toxic compounds and using special diets and pharmacological agents, such as cofactors and L-carnitine.
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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.