Document Detail

Physiological significance of fatty acid elongation system in adrenoleukodystrophy.
MedLine Citation:
PMID:  1880537     Owner:  NLM     Status:  MEDLINE    
We studied the metabolism of radioactive fatty acids in cultured skin fibroblasts from patients with adrenoleukodystrophy (ALD) and Zellweger syndrome (ZS) to clarify the physiological significance of the fatty acid elongation system in the accumulation of very long chain fatty acids (VLFA) in ALD. A substantial amount of radioactive C26:0 was synthesized from [18-14C]stearic acid by ALD and ZS fibroblasts, whereas radioactive C26:0 was not detected in control fibroblasts. Kinetic studies demonstrated that the production of radioactive C24:0 and C26:0 in ALD fibroblasts is higher than that of ZS fibroblasts, which suggests that the fatty acid elongation activity plays an important role for the accumulation of VLFA in ALD, in addition to the decreased VLFA oxidation activities. The addition of monounsaturated fatty acids including oleic acid and erucic acid specifically lowered the formation rate of VLFA without any significant effect on degradation activities of VLFA both in ALD and control fibroblasts. The results suggest that the mechanism of decrease of VLFA by administration of monounsaturated fatty acids is based on the inhibition of fatty acid elongation activity.
R Koike; S Tsuji; T Ohno; Y Suzuki; T Orii; T Miyatake
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Journal of the neurological sciences     Volume:  103     ISSN:  0022-510X     ISO Abbreviation:  J. Neurol. Sci.     Publication Date:  1991 Jun 
Date Detail:
Created Date:  1991-09-30     Completed Date:  1991-09-30     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  0375403     Medline TA:  J Neurol Sci     Country:  NETHERLANDS    
Other Details:
Languages:  eng     Pagination:  188-94     Citation Subset:  IM    
Department of Neurology, Niigata University, Japan.
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MeSH Terms
Adrenoleukodystrophy / metabolism*,  pathology
Fatty Acids / chemistry,  metabolism*
Fatty Acids, Unsaturated / pharmacology
Fibroblasts / metabolism
Peptide Chain Elongation, Translational*
Stearic Acids / metabolism
Zellweger Syndrome / metabolism,  pathology
Reg. No./Substance:
0/Fatty Acids; 0/Fatty Acids, Unsaturated; 0/Stearic Acids; 57-11-4/stearic acid

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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