The DOI matches its Crossref record; 59 of the 59 references it cites were found in a registry or an index.
Very long-chain fatty acids: elongation, physiology and related disorders
Abstract
Very long-chain fatty acids (VLCFAs) are fatty acids (FAs) with a chain-length of ≥22 carbons. Mammals have a variety of VLCFAs differing in chain-length and the number of double bonds. Each VLCFA exhibits certain functions, for example in skin barrier formation, liver homeostasis, myelin maintenance, spermatogenesis, retinal function and anti-inflammation. These functions are elicited not by free VLCFAs themselves, but through their influences as components of membrane lipids (sphingolipids and glycerophospholipids) or precursors of inflammation-resolving lipid mediators. VLCFAs are synthesized by endoplasmic reticulum membrane-embedded enzymes through a four-step cycle. The most important enzymes determining the tissue distribution of VLCFAs are FA elongases, which catalyze the first, rate-limiting step of the FA elongation cycle. Mammals have seven elongases (ELOVL1-7), each exhibiting a characteristic substrate specificity. Several inherited disorders are caused by mutations in genes involved in VLCFA synthesis or degradation. In this review, I describe the molecular mechanism of FA elongation and the responsible enzymes in mammals and yeast, as well as VLCFA-related disorders in human.
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Cites
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The important role of stratum corneum lipids for the cutaneous barrier function
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Identification of phagocytosis regulators using magnetic genome-wide CRISPR screens
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Loss-or gain-of-function mutations in ACOX1 cause axonal loss via different mechanisms
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Organelle interplay-peroxisome interactions in health and disease.
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Role of natural products for the treatment of Alzheimer’s disease
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Metadata
- Authors
- Kihara, A.
- Venue
- Journal of Biochemistry
- Publisher
- Oxford University Press (OUP)
- Published
- 2012-09-14
- Volume
- 152
- Issue
- 5
- Pages
- 387-395
- ISSN
- 0021-924X (ISSN-L)
- OpenAlex
- W2067253037
- Cited by
- 441
- References
- 59
- FWCI
- 5.98
- Type
- journal article
- Language
- en
- Open access
- closed
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