LACTOSYL CERAMIDOSIS - CATABOLIC ENZYME DEFECT OF GLYCOSPHINGOLIPID METABOLISM

被引：80
作者：
DAWSON, G
STEIN, AO
机构：
来源：
SCIENCE | 1970年 / 170卷 / 3957期
关键词：
D O I：
10.1126/science.170.3957.556
中图分类号：
O [数理科学和化学]; P [天文学、地球科学]; Q [生物科学]; N [自然科学总论];
学科分类号：
07 ; 0710 ; 09 ;
摘要：
引用
收藏
页码：556 / &
相关论文
共 26 条
[1]   ENZYMATIC DEFECT IN FABRYS DISEASE - CERAMIDETRIHEXOSIDASE DEFICIENCY [J].
BRADY, RO ;
GAL, AE ;
BRADLEY, RM ;
MARTENSS.E ;
WARSHAW, AL ;
LASTER, L .
NEW ENGLAND JOURNAL OF MEDICINE, 1967, 276 (21) :1163-&
[2]   METABOLISM OF GLUCOCEREBROSIDES .2. EVIDENCE OF AN ENZYMATIC DEFICIENCY IN GAUCHERS DISEASE [J].
BRADY, RO ;
KANFER, JN ;
SHAPIRO, D .
BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS, 1965, 18 (02) :221-&
[3]  
DAWSON G, IN PRESS
[4]   STRUCTURE OF BRAIN DIHEXOSYLCERAMIDE IN GLOBOID CELL LEUKODYSTROPHY [J].
EVANS, JE ;
MCCLUER, RH .
JOURNAL OF NEUROCHEMISTRY, 1969, 16 (09) :1393-&
[5]  
FOLCH J, 1957, J BIOL CHEM, V226, P497
[6]  
HULTBERG B, 1969, LANCET, V2, P1195
[7]   DEMONSTRATION OF AN ALTERATION OF GANGLIOSIDE METABOLISM IN TAY-SACHS DISEASE [J].
KOLODNY, EH ;
BRADY, RO ;
VOLK, BW .
BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS, 1969, 37 (03) :526-&
[8]  
Malone M., 1970, T AM SOC NEUROCHE, V1, P56
[9]   GALACTOSYLGALACTOSYLGLUCOSYLCERAMIDE - GALACTOSYL HYDROLASE IN NORMAL HUMAN PLASMA AND ITS ABSENCE IN PATIENTS WITH FABRYS DISEASE [J].
MAPES, CA ;
ANDERSON, RL ;
SWEELEY, CC .
FEBS LETTERS, 1970, 7 (02) :180-&
[10]   GLYCOLIPID AND MUCOPOLYSACCHARIDE ABNORMALITY IN FIBROBLASTS OF FABRYS DISEASE [J].
MATALON, R ;
DORFMAN, A ;
DAWSON, G ;
SWEELEY, CC .
SCIENCE, 1969, 164 (3887) :1522-&
123