Document Detail

Sjögren-Larsson syndrome: delta 5-and delta 6-fatty acid desaturases in skin fibroblasts.
MedLine Citation:
PMID:  3974899     Owner:  NLM     Status:  MEDLINE    
Cultured skin fibroblasts from two patients with Sjögren-Larsson syndrome (SLS) and from a normal control were analyzed for trienoic and tetraenoic fatty acids. In addition, we assayed desaturation of [1-14C]linoleic acid in cells from four patients and four controls. There was no significant effect of the disease on the composition of polyunsaturated fatty acids or on the rate of linoleic acid desaturation in fibroblasts. The results indicate the presence of delta 5- and delta 6-fatty acid desaturases in cells from SLS patients.
J Avigan; B D Campbell; D A Yost; O Hernell; G Holmgren; S F Jagell
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Neurology     Volume:  35     ISSN:  0028-3878     ISO Abbreviation:  Neurology     Publication Date:  1985 Mar 
Date Detail:
Created Date:  1985-04-10     Completed Date:  1985-04-10     Revised Date:  2009-11-19    
Medline Journal Info:
Nlm Unique ID:  0401060     Medline TA:  Neurology     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  401-3     Citation Subset:  AIM; IM    
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MeSH Terms
Chromatography, High Pressure Liquid
Chromatography, Thin Layer
Fatty Acid Desaturases / metabolism*
Fibroblasts / enzymology
Ichthyosis / enzymology*,  pathology
Linoleoyl-CoA Desaturase
Middle Aged
Skin / enzymology*,  pathology
Reg. No./Substance:
EC 1.14.19.-/Fatty Acid Desaturases; EC Desaturase; EC 1.14.99.-/delta-5 fatty acid desaturase

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

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