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Metabolism of Very Long-Chain Fatty Acids: Genes and Pathophysiology
Open accessAbstract
Fatty acids (FAs) are highly diverse in terms of carbon (C) chain-length and number of double bonds. FAs with C>20 are called very long-chain fatty acids (VLCFAs). VLCFAs are found not only as constituents of cellular lipids such as sphingolipids and glycerophospholipids but also as precursors of lipid mediators. Our understanding on the function of VLCFAs is growing in parallel with the identification of enzymes involved in VLCFA synthesis or degradation. A variety of inherited diseases, such as ichthyosis, macular degeneration, myopathy, mental retardation, and demyelination, are caused by mutations in the genes encoding VLCFA metabolizing enzymes. In this review, we describe mammalian VLCFAs by highlighting their tissue distribution and metabolic pathways, and we discuss responsible genes and enzymes with reference to their roles in pathophysiology.
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“Half of myelin sphingolipids contain VLCFAs (C > 22) [213,214,215].”
From Revisiting the Pathogenesis of X-Linked Adrenoleukodystrophy (2025) · cites this paper as [213]
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“VLCFAs increase membrane stiffness, thereby decreasing fluidity, and they are likely critical for proper myelin function [191,213,214,217], myelination [218], myelin maintenance [207,213,215], and neuron polarity during CNS development [219].”
From Revisiting the Pathogenesis of X-Linked Adrenoleukodystrophy (2025) · cites this paper as [213]
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Acylcarnitines: Nomenclature, Biomarkers, Therapeutic Potential, Drug Targets, and Clinical Trials
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Metadata
- Authors
- Sassa, T., & Kihara, A.
- Affiliations
- Hokkaido University (JP)
- Corresponding author
- Kihara, Akio
- Venue
- Biomolecules & Therapeutics
- Published
- 2014-03-31
- Volume
- 22
- Issue
- 2
- Pages
- 83-92
- ISSN
- 1976-9148 (ISSN-L)
- 2005-4483
- PMC
- PMC3975470
- OpenAlex
- W2009332633
- Cited by
- 302
- References
- 95
- Citations by year
- 2026: 18 · 2025: 27 · 2024: 34 · 2023: 35 · 2022: 22 · 2021: 46 · 2020: 32 · 2019: 23 · 2018: 28 · 2017: 17 · 2016: 11 · 2015: 4 · 2014: 4
- FWCI
- 1.03
- Type
- journal article
- Language
- en
- Open access
- green
- europepmc.org
- Licence
- Locations
- Biomolecules & Therapeutics (publishedVersion)
- Europe PMC (PubMed Central) (publishedVersion, other-oa)
- PubMed Central (publishedVersion, cc-by-nc)
- PubMed (publishedVersion)
- Funders
- Japan Society for the Promotion of Science (23370057, 24590073)
- Topics
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Biochemistry › Lipid metabolism and biosynthesis
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Clinical Biochemistry › Metabolism and Genetic Disorders
- Health Sciences › Nursing › Nutrition and Dietetics › Fatty Acid Research and Health
- Keywords
- very long chain fatty acids, fatty acid synthesis, fatty acid degradation, ichthyosis, macular degeneration, myopathy, demyelination, lipid mediators, sphingolipids, tissue distribution
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