ReviewCellular and molecular neurobiology2022
Evidence that Oxidative Disbalance and Mitochondrial Dysfunction are Involved in the Pathophysiology of Fatty Acid Oxidation Disorders.
Review in Cellular and molecular neurobiology, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 37 papers.
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Who cites it
37 citing papers in PubMed, 72 citations in OpenAlex.
- L-Carnitine Prevents Lipopolysaccharide-Induced Inflammatory Response, Microglia Activation and Neuronal Damage in Cerebral Cortex, as Well as Neuromotor Development Delay in Neonatal Glutaryl-CoA Dehydrogenase Deficient (GcdhJournal of inherited metabolic disease · 2026Article
- Rewiring Lipid Metabolism: PINK1 as a Central Regulator of Mitochondrial Homeostasis in Parkinson's Disease.International journal of molecular sciences · 2026Review
- SR-B1-Mediated Transplacental Transfer of Hydrophobic Toxicants Disrupts Fetal Development During Barriergenesis.Advanced science (Weinheim, Baden-Wurttemberg, Germany) · 2026Article
- Metabolic profiles associated with fat loss in Parkinson's disease.Journal of neurology, neurosurgery, and psychiatry · 2026Article
- Preliminary metabolic characterization of hepatic lipidosis in cats using liquid chromatography-mass spectrometry and gas chromatography-mass spectrometry: pathway insights and candidate biomarkers.Journal of veterinary internal medicine · 2026Article
- Molecular pathways, tissue-specific roles, and therapeutic opportunities in mitochondrial resilience-based strategies for insulin resistance.Frontiers in clinical diabetes and healthcare · 2026Review
- Mitochondrial dysfunction mediates the association between mercury exposure and lipid metabolism in children and adolescents.Lipids in health and disease · 2025Article
- A Longitudinal Study of Baseline Fatty Acid Concentrations and Executive Function Over 12 Months Among Adolescents With and Without Perinatal HIV Exposure or Infection From Kampala, Uganda.The Journal of nutrition · 2025Article
- Effects of Exercise Snack Program on Quality of Life, Cardiorespiratory Fitness, and Metabolic Flexibility in Elderly Cancer Survivors: A Preliminary Study.Life (Basel, Switzerland) · 2025Article
- Non-Alcoholic Fatty Liver Disease in Poultry: Risk Factors, Mechanism of Development, and Emerging Strategies.International journal of molecular sciences · 2025Review
- Remodeling adipocytes' lipid metabolism with a polycation loaded enzyme-active framework reverses osteoporotic bone marrow.Nature communications · 2025Article
- Toxins to treatments: Impact of environmental pollutants, gut microbiota, and natural compounds on non-alcoholic fatty liver disease progression.World journal of hepatology · 2025Review
- The Pathogenesis of Very Long-Chain Acyl-CoA Dehydrogenase Deficiency.Biomolecules · 2025Review
- Circulatory response to exercise relative to oxygen uptake assessed in the follow-up of patients with fatty acid beta-oxidation disorders.Journal of inherited metabolic disease · 2025Article
- Unveiling therapeutic targets and preventive components for kidney insufficiency and blood stasis-type BPH: bridging metabolomics, network pharmacology and reverse screening.Frontiers in pharmacology · 2025Article
- Pea Albumin Alleviates Oleic Acid-Induced Lipid Accumulation in LO2 Cells Through Modulating Lipid Metabolism and Fatty Acid Oxidation Pathways.Foods (Basel, Switzerland) · 2024Article
- A promising anti-tumor targeting on ERMMDs mediated abnormal lipid metabolism in tumor cells.Cell death & disease · 2024Review
- Mitochondrial Dysfunction: A Roadmap for Understanding and Tackling Cardiovascular Aging.Aging and disease · 2024Review
- Neurological outcome in long-chain hydroxy fatty acid oxidation disorders.Annals of clinical and translational neurology · 2024Article
- Korean Nationwide Exploration of Sarcopenia Prevalence and Risk Factors in Late Middle-Aged Women.Healthcare (Basel, Switzerland) · 2024Article
Corrections and comments
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Authors and funding
2 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Mitochondrial fatty acid β-oxidation disorders (FAODs) are a group of about 20 diseases which are caused by specific mutations in genes that codify proteins or enzymes involved in the fatty acid transport and mitochondrial β-oxidation. As a consequence of these inherited metabolic defects, fatty acids can not be used as an appropriate energetic source during special conditions, such as prolonged fasting, exercise or other catabolic states. Therefore, patients usually present hepatopathy, cardiomyopathy, severe skeletal myopathy and neuropathy, besides biochemical features like hypoketotic hypoglycemia, metabolic acidosis, hypotony and hyperammonemia. This set of symptoms seems to be related not only with the energy deficiency, but also with toxic effects provoked by fatty acids and carnitine derivatives accumulated in the tissues of the patients. The understanding of the mechanisms by which these metabolites provoke tissue injury in FAODs is crucial for the developmental of novel therapeutic strategies that promote increased life expectancy, as well as improved life quality for patients. In this sense, the objective of this review is to present evidence from the scientific literature on the role of oxidative damage and mitochondrial dysfunction in the pathogenesis of the most prevalent FAODs: medium-chain acyl-CoA dehydrogenase (MCAD), long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) and very long-chain acyl-CoA dehydrogenase (VLCAD) deficiencies. It is expected that the findings presented in this review, obtained from both animal model and patients studies, may contribute to a better comprehension of the pathophysiology of these diseases.
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