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, 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]
See also
- ACADVL
- SLC27A2
- SLC27A5
- Cerotic acid, the fatty acid associated with adrenoleukodystrophy
References
- ^ Jakobsson, Andreas; Westerberg, Rolf; Jacobsson, Anders "Fatty acid elongases in mammals: their regulation and roles in metabolism" Progress in Lipid Research 2006, volume 45, pp. 237-249. doi:10.1016/j.plipres.2006.01.004
- ^ "Very-long-chain fatty acids from the animal and plant kingdoms" Rezanka, Tomas Progress in Lipid Research 1989, volume 28, pp. 147-87. doi:10.1016/0163-7827(89)90011-8
- ^ 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.
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". Neurology. 31 (10): 1241–1241. doi:10.1212/WNL.31.10.1241. ISSN 0028-3878.
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