STRUCTURE OF BRAIN DIHEXOSYLCERAMIDE IN GLOBOID CELL LEUKODYSTROPHY

被引：23
作者：
EVANS, JE
MCCLUER, RH
机构：
[1] Departments of Psychiatry and Physiological Chemistry, The Ohio State University, Columbus, Ohio
来源：
JOURNAL OF NEUROCHEMISTRY | 1969年 / 16卷 / 09期
关键词：
D O I：
10.1111/j.1471-4159.1969.tb05991.x
中图分类号：
Q5 [生物化学]; Q7 [分子生物学];
学科分类号：
071010 ; 081704 ;
摘要：
Abstract— 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; C18, and C21 fatty acids composed one‐half of the mixture. All analyses were performed by GLC and TLC utilizing micro‐techniques. Copyright © 1969, Wiley Blackwell. All rights reserved
引用
收藏
页码：1393 / &
相关论文
共 19 条
[1]   CARBOHYDRATE STRUCTURES OF NEUTRAL CERAMIDE GLYCOLIPIDS IN KIDNEYS OF DIFFERENT MOUSE STRAINS WITH SPECIAL REFERENCE TO CERAMIDE DIHEXOSIDES [J].
ADAMS, EP ;
GRAY, GM .
CHEMISTRY AND PHYSICS OF LIPIDS, 1968, 2 (02) :147-&
[2]   STUDIES IN GLOBOID (KRABBE) LEUKODYSTROPHY .1. SIGNIFICANCE OF LIPID ABNORMALITIES IN WHITE MATTER IN 8 GLOBOID AND 13 CONTROL PATIENTS [J].
AUSTIN, J .
ARCHIVES OF NEUROLOGY, 1963, 9 (03) :207-+
[3]  
BACHHAWAT BK, 1967, BIOCHEM J, V104, pC15
[4]   RAPID DETERMINATION OF OLEFIN POSITION IN ORGANIC COMPOUNDS IN MICROGRAM RANGE BY OZONOLYSIS AND GAS CHROMATOGRAPHY [J].
BEROZA, M ;
BIERL, BA .
ANALYTICAL CHEMISTRY, 1967, 39 (10) :1131-&
[5]  
CARTER HE, 1967, J LIPID RES, V8, P391
[6]  
KEAN EL, 1966, J LIPID RES, V7, P449
[7]  
KENNAWAY NG, 1968, J LIPID RES, V9, P755
[8]   MOLECULAR COMPOSITION OF MAJOR GLYCOLIPIDS IN GLOBOID CELL LEUKODYSTROPHY [J].
MENKES, JH ;
DUNCAN, C ;
MOOSSY, J .
NEUROLOGY, 1966, 16 (06) :581-&
[9]   QUANTITATIVE DETERMINATION OF GLUCOSE AND GALACTOSE IN GANGLIOSIDES BY GAS-LIQUID CHROMATOGRAPHY [J].
PENICK, RJ ;
MCCLUER, RH .
BIOCHIMICA ET BIOPHYSICA ACTA, 1966, 116 (02) :288-&
[10]  
ROSENBERG A, 1966, J LIPID RES, V7, P122
12