Very long chain fatty acid
A very-long-chain fatty acid (VLCFA) is a fatty acid with 22 or more carbons. Their biosynthesis occurs in the endoplasmic reticulum.[1] VLCFA's can represent up to a few percent of the total fatty acid content of a cell.[2]
Unlike most fatty acids, VLCFAs are too long to be metabolized in the
mitochondria, in the endoplasmic reticulum (ER) in plants and must be metabolized in peroxisomes
.
Certain peroxisomal disorders, such as adrenoleukodystrophy and Zellweger syndrome, can be associated with an accumulation of VLCFAs.[3][4] Enzymes that produce VLCFAs are the targets of herbicides including pyroxasulfone.[5][6]
Major VLCFAs
Some of the more common saturated VLCFAs:
ghedoic acid (C34), and the odd-chain fatty acid ceroplastic acid (C35). Several monounsaturated VLCFAs are also known: nervonic acid (Δ15-24:1), ximenic acid (Δ17-26:1), and lumequeic acid (Δ21-30:1).[7]
See also
- ACADVL
- SLC27A2
- SLC27A5
- Cerotic acid, the fatty acid associated with adrenoleukodystrophy
References
- ^ Kemp, Stephan and Watkins, Paul. "Very long-chain fatty acids". X-ald Database. Retrieved 5 January 2013.
{{cite web}}: CS1 maint: multiple names: authors list (link) - ^ "Very long-chain acyl-CoA dehydrogenase deficiency". Genetics Home Reference, National Institutes of Health. Retrieved 5 January 2013.
- PMID 30363083.
- PMID 15277688.
- PMID 19336244.
Bibliography
- Moser, H. W.; Moser, A. B.; Frayer, K. K.; Chen, W.; Schulman, J. D.; O'Neill, B. P.; Kishimoto, Y. (1981). "Adrenoleukodystrophy: Increased plasma content of saturated very long chain fatty acids". PMID 7202134.
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