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THE STRUCTURE OF BRAIN DIHEXOSYLCERAMIDE IN GLOBOID CELL LEUKODYSTROPHY 1
Author(s) -
Evans J. E.,
McCluer R. H.
Publication year - 1969
Publication title -
journal of neurochemistry
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.75
H-Index - 229
eISSN - 1471-4159
pISSN - 0022-3042
DOI - 10.1111/j.1471-4159.1969.tb05991.x
Subject(s) - hydrolysis , chemistry , carbohydrate , ozonolysis , biochemistry , lactose , moiety , leukodystrophy , acid hydrolysis , methylation , fatty acid , chromatography , stereochemistry , organic chemistry , gene , medicine , disease , pathology
— Dihexosylceramide from the brain of a case of globoid cell leukodystrophy was isolated and characterized. Studies utilizing partial acid hydrolysis and methylation indicated the carbohydrate sequence to be galactosylglucosylceramide. The carbohydrate moiety was liberated by ozonolysis and identified to be lactose by GLC. 4‐Sphingenine and sphinganine were the only long chain bases detected. The chain length of the fatty acids present ranged from 14 to 25 carbons; C 18 , and C 21 fatty acids composed one‐half of the mixture. All analyses were performed by GLC and TLC utilizing micro‐techniques.