The DOI matches its Crossref record; 95 of the 95 references it cites were found in a registry or an index.
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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Functional rafts in cell membranes
Simons, K., & Ikonen, E. (1997) · Nature · doi:10.1038/42408 · Cited by 9,757 · cited 1×
“…levels, C24 sphingolipids have unique biophysical properties not possessed by C16 sphingolipids such as effects on membrane fluidity, lipid microdomain formation, and signaling across the membrane (Simons and Ikonen, 1997; Kasahara and Sanai, 2000; Sonnino et al., 2009; Silva et al., 2012).”
The skin: an indispensable barrier
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Putative X-linked adrenoleukodystrophy gene shares unexpected homology with ABC transporters
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Fatty acid elongases in mammals: their regulation and roles in metabolism
JAKOBSSON, A., WESTERBERG, R., & JACOBSSON, A. (2006) · Progress in Lipid Research · doi:10.1016/j.plipres.2006.01.004 · Cited by 936
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The role of omega-3 long-chain polyunsaturated fatty acids in health and disease of the retina
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Lipids of nervous tissue: Composition and metabolism
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Crucial role of a long-chain fatty acid elongase, Elovl6, in obesity-induced insulin resistance
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Baz, L., & Al-thepyani, M. (2026) · Frontiers in Molecular Biosciences · doi:10.3389/fmolb.2026.1906904
Ceramide Metabolic Pathways and Their Regulation in Adolescent Acne Vulgaris
Gou, Y., Wu, H., Wu, X., Dong, G., Li, H., Yuan, H., & Li, L. (2026) · International Journal of Molecular Sciences · doi:10.3390/ijms27198737
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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