ArticleJournal of lipid research2014
Lorenzo's oil inhibits ELOVL1 and lowers the level of sphingomyelin with a saturated very long-chain fatty acid.
Article in Journal of lipid research, 2014. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 26 papers.
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Who cites it
26 citing papers in PubMed, 65 citations in OpenAlex.
- The complex lipidome as a driver of tissue-specific pathology in adrenoleukodystrophy.Molecular neurodegeneration · 2026Review
- Nervonic acid supplementation mitigates disease severity biomarkers in adrenoleukodystrophy.Neurotherapeutics : the journal of the American Society for Experimental NeuroTherapeutics · 2026Article
- Adrenoleukodystrophy in adults: phenotypic characterisation and natural history in a large cohort.Journal of neurology, neurosurgery, and psychiatry · 2026Observational
- Modeling lipid homeostasis using stable isotope tracing and flux analysis.Cell metabolism · 2026Article
- Plasma Fatty Acid Profiling and Mathematical Estimation of the Omega-3 Index: Toward Diagnostic Tools in Atherosclerosis and Statin Therapy Monitoring.Biomedicines · 2025Article
- Fatty Acid Metabolism in Peroxisomes and Related Disorders.Advances in experimental medicine and biology · 2024Review
- Erucic Acid: A Possible Therapeutic Agent for Neurodegenerative Diseases.Current molecular medicine · 2024Review
- A comprehensive review of the family of very-long-chain fatty acid elongases: structure, function, and implications in physiology and pathology.European journal of medical research · 2023Review
- Therapeutic Role of ELOVL in Neurological Diseases.ACS omega · 2023Review
- Intracerebral lentiviral ABCD1 gene therapy in an early disease onset ALD mouse model.Gene therapy · 2023Article
- Nervonic Acid Attenuates Accumulation of Very Long-Chain Fatty Acids and is a Potential Therapy for Adrenoleukodystrophy.Neurotherapeutics : the journal of the American Society for Experimental NeuroTherapeutics · 2022Article
- X-linked Adrenoleukodystrophy in a 20-Year-Old Male With an ABCD1 Gene Mutation: First Case From Pakistan.Cureus · 2022Article
- Emerging cellular themes in leukodystrophies.Frontiers in cell and developmental biology · 2022Review
- AnFrontiers in molecular neuroscience · 2022Article
- The structural basis of fatty acid elongation by the ELOVL elongases.Nature structural & molecular biology · 2021Article
- Dietary Lipid Modulation of Intestinal Serotonin in Ballan Wrasse (Frontiers in endocrinology · 2021Article
- X-linked adrenoleukodystrophy: Pathology, pathophysiology, diagnostic testing, newborn screening and therapies.International journal of developmental neuroscience : the official journal of the International Society for Developmental Neuroscience · 2020Review
- Dietary rescue of lipotoxicity-induced mitochondrial damage in Peroxin19 mutants.PLoS biology · 2018Article
- Effect of Lorenzo's Oil on Hepatic Gene Expression and the Serum Fatty Acid Level in abcd1-Deficient Mice.JIMD reports · 2018Article
- Rapamycin Inhibits Expression of Elongation of Very-long-chain Fatty Acids 1 and Synthesis of Docosahexaenoic Acid in Bovine Mammary Epithelial Cells.Asian-Australasian journal of animal sciences · 2016Article
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
X-linked adrenoleukodystrophy (X-ALD) is a peroxisomal disorder caused by impaired degradation of very long-chain fatty acids (VLCFAs) due to mutations in the ABCD1 gene responsible for VLCFA transport into peroxisomes. Lorenzo's oil, a 4:1 mixture of glyceryl trioleate and glyceryl trierucate, has been used to reduce the saturated VLCFA level in the plasma of X-ALD patients; however, the mechanism by which this occurs remains elusive. We report the biochemical characterization of Lorenzo's oil activity toward elongation of very long-chain fatty acid (ELOVL) 1, the primary enzyme responsible for the synthesis of saturated and monounsaturated VLCFAs. Oleic and erucic acids inhibited ELOVL1, and, moreover, their 4:1 mixture (the FA composition of Lorenzo's oil) exhibited the most potent inhibitory activity. The kinetics analysis revealed that this was a mixed (not a competitive) inhibition. At the cellular level, treatment with the 4:1 mixture reduced the level of SM with a saturated VLCFA accompanied by an increased level of SM with a monounsaturated VLCFA, probably due to the incorporation of erucic acid into the FA elongation cycle. These results suggest that inhibition of ELOVL1 may be an underlying mechanism by which Lorenzo's oil exerts its action.
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