CHARACTERIZATION OF A NEW MODEL OF GM2-GANGLIOSIDOSIS (SANDHOFFS DISEASE) IN KORAT CATS

被引:56
作者
NEUWELT, EA
JOHNSON, WG
BLANK, NK
PAGEL, MA
MASLENMCCLURE, C
MCCLURE, MJ
WU, PM
机构
[1] OREGON HLTH SCI UNIV,DEPT SURG,DIV NEUROSURG,PORTLAND,OR 97201
[2] OREGON HLTH SCI UNIV,DEPT NEUROL,PORTLAND,OR 97201
[3] OREGON HLTH SCI UNIV,DEPT PATHOL,PORTLAND,OR 97201
[4] OREGON HLTH SCI UNIV,DEPT MED GENET,PORTLAND,OR 97201
[5] OREGON HLTH SCI UNIV,PORTLAND,OR 97201
[6] COLUMBIA UNIV COLL PHYS & SURG,DEPT NEUROL,NEW YORK,NY 10032
关键词
D O I
10.1172/JCI111997
中图分类号
R-3 [医学研究方法]; R3 [基础医学];
学科分类号
1001 ;
摘要
引用
收藏
页码:482 / 490
页数:9
相关论文
共 35 条
[2]  
BAKER HJ, 1976, FED PROC, V35, P1193
[3]   GANGLIOSIDOSES - COMPARATIVE FEATURES AND RESEARCH APPLICATIONS [J].
BAKER, HJ ;
REYNOLDS, GD ;
WALKLEY, SU ;
COX, NR ;
BAKER, GH .
VETERINARY PATHOLOGY, 1979, 16 (06) :635-649
[4]   HUMAN BETA-GLUCURONIDASE DEFICIENCY MUCOPOLYSACCHARIDOSIS - IDENTIFICATION OF CROSS-REACTIVE ANTIGEN IN CULTURED FIBROBLASTS OF DEFICIENT PATIENTS BY ENZYME IMMUNOASSAY [J].
BELL, CE ;
SLY, WS ;
BROT, FE .
JOURNAL OF CLINICAL INVESTIGATION, 1977, 59 (01) :97-105
[5]   ENZYME REPLACEMENT TREATMENT FOR TAY-SACHS DISEASE BRAIN-CELLS IN CULTURE UTILIZING CONCANAVALIN A-MEDIATED HEXOSAMINIDASE-A UPTAKE - BIOCHEMICAL AND MORPHOLOGICAL EVIDENCE OF GM2 MOBILIZATION [J].
BROOKS, SE ;
HOFFMAN, LM ;
ADACHI, M ;
AMSTERDAM, D ;
SCHNECK, L .
ACTA NEUROPATHOLOGICA, 1980, 50 (01) :9-17
[6]   GM2 GANGLIOSIDE LYSOSOMAL STORAGE DISEASE IN CATS WITH BETA-HEXOSAMINIDASE DEFICIENCY [J].
CORK, LC ;
MUNNELL, JF ;
LORENZ, MD ;
MURPHY, JV ;
BAKER, HJ ;
RATTAZZI, MC .
SCIENCE, 1977, 196 (4293) :1014-1017
[7]  
FOLCH J, 1957, J BIOL CHEM, V226, P497
[8]  
Galjaard H., 1980, GENETIC METABOLIC DI, P825
[9]  
Hakomori S I, 1974, Methods Enzymol, V32, P345
[10]   RECOGNITION MARKER REQUIRED FOR UPTAKE OF A LYSOSOMAL ENZYME BY CULTURED FIBROBLASTS [J].
HICKMAN, S ;
SHAPIRO, LJ ;
NEUFELD, EF .
BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS, 1974, 57 (01) :55-61