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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2014/08/19 11:46:28」(JST)

A very long chain fatty acid (VLCFA) is a fatty acid with aliphatic tails longer than 22 carbons.

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, can be associated with an accumulation of VLCFAs.^[1]^[2]

References

^ Kemp, Stephan and Watkins, Paul. "Very long-chain fatty acids". X-ald Database. Retrieved 5 January 2013.
^ "Very long-chain acyl-CoA dehydrogenase deficiency". Genetics Home Reference, National Institutes of Health. Retrieved 5 January 2013.

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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1. 副腎白質ジストロフィー adrenoleukodystrophy
2. ペルオキシソーム病 peroxisomal disorders
3. 先天性代謝異常：個々の障害の発見 inborn errors of metabolism identifying the specific disorder
4. 小児における原発性副腎不全の原因および臨床症状 causes and clinical manifestations of primary adrenal insufficiency in children
5. 副腎不全の稀な原因 unusual causes of adrenal insufficiency

Wang Y, Busin R, Reeves C, Bezman L, Raymond G, Toomer CJ, Watkins PA, Snowden A, Moser A, Naidu S, Bibat G, Hewson S, Tam K, Clarke JT, Charnas L, Stetten G, Karczeski B, Cutting G, Steinberg S.SourceDNA Diagnostic Laboratory, Johns Hopkins University School of Medicine, CMSC1004, 600 N. Wolfe Street, Baltimore, MD, 21287, USA.
Molecular genetics and metabolism.Mol Genet Metab.2011 Jun 21. [Epub ahead of print]
X-linked adrenoleukodystrophy (X-ALD) is a progressive peroxisomal disorder affecting adrenal glands, testes and myelin stability that is caused by mutations in the ABCD1 (NM_000033) gene. Males with X-ALD may be diagnosed by the demonstration of elevated very long chain fatty acid (VLCFA) levels in
PMID 21700483

Changes in carnitine octanoyltransferase activity induce alteration in fatty acid metabolism.

Le Borgne F, Ben Mohamed A, Logerot M, Garnier E, Demarquoy J.SourceInserm U866, Universite de Bourgogne, Laboratoire de Biochimie Metabolique et Nutritionnelle, 6 blvd Gabriel, F-21000 Dijon, France.
Biochemical and biophysical research communications.Biochem Biophys Res Commun.2011 Jun 17;409(4):699-704. Epub 2011 May 17.
The peroxisomal beta oxidation of very long chain fatty acids (VLCFA) leads to the formation of medium chain acyl-CoAs such as octanoyl-CoA. Today, it seems clear that the exit of shortened fatty acids produced by the peroxisomal beta oxidation requires their conversion into acyl-carnitine and the p
PMID 21619872