Adrenoleukodystrophy
- 1 October 1977
- journal article
- research article
- Published by Wolters Kluwer Health in Neurology
- Vol. 27 (10) , 928
- https://doi.org/10.1212/wnl.27.10.928
Abstract
An accumulation of fatty acids having chain lengths of C22 to C26 was demonstrated in the cholesterol ester fraction of white matter from two boys with adrenoleukodystrophy. A similar accumulation of very long chain fatty acids was also shown in gangliosides and cerebrosides. A disorder of the one-carbon degradation system responsible for the metabolism of some of the very long chain fatty acids may be the basic enzymatic defect in this condition.This publication has 3 references indexed in Scilit:
- Qualitative alteration in substrate specificity of mitochondrial monoamine oxidase in brainJournal of Neurochemistry, 1976
- Maple Syrup DiseaseArchives of Neurology, 1967
- A REVISION OF THE SCHOENHEIMER-SPERRY METHOD FOR CHOLESTEROL DETERMINATIONJournal of Biological Chemistry, 1950