Document Detail


Adrenoleukodystrophy: a correlation between saturated very long-chain fatty acids in mononuclear cells and phenotype.
MedLine Citation:
PMID:  1929220     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Saturated very long-chain fatty acids in erythrocyte membranes, blood plasma, and mononuclear cells were studied in 4 patients with childhood-adolescent adrenoleukodystrophy and 4 patients with adult adrenoleukodystrophy and 19 normal control subjects by using high-performance liquid chromatography. Ratios of C26:0 to C22:0 in mononuclear cells, erythrocyte membranes, and blood plasma in patients with childhood-adolescent and adult adrenoleukodystrophy were significantly higher than in normal control subjects. Furthermore, ratios of C26:0 to C22:0 in mononuclear cells were significantly higher in patients with childhood-adolescent adrenoleukodystrophy than in patients with adult adrenoleukodystrophy, whereas there was no significant difference in the ratios in erythrocyte membranes and blood plasma between the two groups of patients with adrenoleukodystrophy. These results suggest that there is a correlation between phenotype and ratio of C26:0 to C22:0 within mononuclear cells in patients with adrenoleukodystrophy.
Authors:
Y Antoku; F Koike; Y Ohtsuka; T Sakai; K Tsukamoto; H Nagara; H Iwashita; I Goto
Publication Detail:
Type:  Comparative Study; Journal Article    
Journal Detail:
Title:  Annals of neurology     Volume:  30     ISSN:  0364-5134     ISO Abbreviation:  Ann. Neurol.     Publication Date:  1991 Jul 
Date Detail:
Created Date:  1991-11-07     Completed Date:  1991-11-07     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  7707449     Medline TA:  Ann Neurol     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  101-3     Citation Subset:  IM    
Affiliation:
Department of Neurology, National Chikugo Hospital, Fukuoka, Japan.
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MeSH Terms
Descriptor/Qualifier:
Adolescent
Adrenoleukodystrophy / blood*,  classification,  pathology
Adult
Child
Demyelinating Diseases
Erythrocyte Membrane / chemistry*
Fatty Acids / blood*
Female
Fibroblasts / chemistry
Humans
Leukocytes, Mononuclear / chemistry*
Male
Membrane Lipids / analysis*
Middle Aged
Phenotype
Chemical
Reg. No./Substance:
0/Fatty Acids; 0/Membrane Lipids

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