INTRACEREBRAL DISTRIBUTION OF INFECTIOUS AMYLOID PROTEIN IN SPONGIFORM ENCEPHALOPATHY

被引:34
作者
BROWN, P
KENNEY, K
LITTLE, B
IRONSIDE, J
WILL, R
CERVENAKOVA, L
BJORK, RJ
SANMARTIN, RA
SAFAR, J
ROOS, R
HALTIA, M
GIBBS, CJ
GAJDUSEK, DC
机构
[1] LEHIGH VALLEY MED CTR,DEPT PATHOL,ALLENTOWN,PA
[2] WESTERN GEN HOSP,CJD SURVEILLANCE UNIT,EDINBURGH EH4 2XU,MIDLOTHIAN,SCOTLAND
[3] COLORADO SPRINGS NEUROL ASSOCIATES,COLORADO SPRINGS,CO
[4] UNIV CHICAGO,MED CTR,DEPT NEUROL,CHICAGO,IL 60637
[5] HELSINKI UNIV,DEPT PATHOL,HELSINKI,FINLAND
关键词
D O I
10.1002/ana.410380218
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
We studied the regional distribution of infectious amyloid protein by western immunoblots of brain tissue extracts from 37 patients with different forms of spongiform encephalopathy, i.e., 16 sporadic cases, 18 familial cases with a variety of mutations, and 3 iatrogenic cases. In sporadic and familial Creutzfeldt-Jakob disease, amyloid protein concentrations were usually highest in the frontotemporal regions of the cerebral cortex, whereas iatrogenic Creutzfeldt-Jakob disease and Gerstmann-Straussler-Scheinker syndrome had as high or higher concentrations in the deep cerebral nuclei and cerebellum. As a group, familial cases had lower amyloid protein concentrations than either sporadic or iatrogenic cases, and fatal familial insomnia patients had the lowest concentrations found in any form of disease. This hierarchy of amyloid protein concentrations corresponds to the experimental transmission rates observed for each form of disease and is consistent with the concept that the protein molecule is an integral component of the infectious agent. Regional amyloid protein pattern analysis of brain and spinal cord may help to distinguish sporadic from environmentally acquired infections, as for example, cases of human disease suspected to have arisen from exposure to sheep or cows infected with scrapie or bovine spongiform encephalopathy.
引用
收藏
页码:245 / 253
页数:9
相关论文
共 23 条
[11]   FATAL FAMILIAL INSOMNIA AND FAMILIAL CREUTZFELDT-JAKOB DISEASE - DISEASE PHENOTYPE DETERMINED BY A DNA POLYMORPHISM [J].
GOLDFARB, LG ;
PETERSEN, RB ;
TABATON, M ;
BROWN, P ;
LEBLANC, AC ;
MONTAGNA, P ;
CORTELLI, P ;
JULIEN, J ;
VITAL, C ;
PENDELBURY, WW ;
HALTIA, M ;
WILLS, PR ;
HAUW, JJ ;
MCKEEVER, PE ;
MONARI, L ;
SCHRANK, B ;
SWERGOLD, GD ;
AUTILIOGAMBETTI, L ;
GAJDUSEK, DC ;
LUGARESI, E ;
GAMBETTI, P .
SCIENCE, 1992, 258 (5083) :806-808
[12]  
GOLDGABER D, 1989, EXP NEUROL, V106, P104
[13]  
GOODBRAND IA, 1995, NEUROSCI LETT, V183, P1
[14]   A RAPID AND EFFICIENT METHOD TO ENRICH SAF-PROTEIN FROM SCRAPIE BRAINS OF HAMSTERS [J].
HILMERT, H ;
DIRINGER, H .
BIOSCIENCE REPORTS, 1984, 4 (02) :165-170
[15]   PROTEINASE-RESISTANT PRION PROTEIN ACCUMULATION IN SYRIAN-HAMSTER BRAIN CORRELATES WITH REGIONAL PATHOLOGY AND SCRAPIE INFECTIVITY [J].
JENDROSKA, K ;
HEINZEL, FP ;
TORCHIA, M ;
STOWRING, L ;
KRETZSCHMAR, HA ;
KON, A ;
STERN, A ;
PRUSINER, SB ;
DEARMOND, SJ .
NEUROLOGY, 1991, 41 (09) :1482-1490
[16]   DEVELOPMENTAL EXPRESSION AND REGIONAL DISTRIBUTION OF THE SCRAPIE-ASSOCIATED PROTEIN MESSENGER-RNA IN THE RAT CENTRAL-NERVOUS-SYSTEM [J].
LIEBERBURG, I .
BRAIN RESEARCH, 1987, 417 (02) :363-366
[17]  
Manson J., 1992, Neurodegeneration, V1, P45
[18]   FATAL FAMILIAL INSOMNIA, A PRION DISEASE WITH A MUTATION AT CODON-178 OF THE PRION PROTEIN GENE [J].
MEDORI, R ;
TRITSCHLER, HJ ;
LEBLANC, A ;
VILLARE, F ;
MANETTO, V ;
CHEN, HY ;
XUE, R ;
LEAL, S ;
MONTAGNA, P ;
CORTELLI, P ;
TINUPER, P ;
AVONI, P ;
MOCHI, M ;
BARUZZI, A ;
HAUW, JJ ;
OTT, J ;
LUGARESI, E ;
AUTILIOGAMBETTI, L ;
GAMBETTI, P .
NEW ENGLAND JOURNAL OF MEDICINE, 1992, 326 (07) :444-449
[19]   NERVE GROWTH-FACTOR INCREASES MESSENGER-RNA LEVELS FOR THE PRION PROTEIN AND THE BETA-AMYLOID PROTEIN-PRECURSOR IN DEVELOPING HAMSTER BRAIN [J].
MOBLEY, WC ;
NEVE, RL ;
PRUSINER, SB ;
MCKINLEY, MP .
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA, 1988, 85 (24) :9811-9815
[20]  
MURAMOTO T, 1992, AM J PATHOL, V140, P1411