Prion diseases

被引:19
作者
Collinge, John [1 ]
Palmer, Mark S. [1 ]
机构
[1] St Marys Hosp, Med Sch, Dept Biochem & Mol Genet, London W2 1PG, England
关键词
D O I
10.1016/S0959-437X(05)80156-X
中图分类号
Q2 [细胞生物学];
学科分类号
071009 ; 090102 ;
摘要
There have been remarkably rapid advances in the understanding of prion diseases over the past year. The controversial notion that the transmissible agent may be an abnormal isoform of a host-encoded protein, the prion protein, is now gaining wide acceptance. The conundrum of how a disease can both be inherited as an autosomal dominant condition and also be experimentally transmissible by inoculation is beginning to make sense.
引用
收藏
页码:448 / 454
页数:7
相关论文
共 36 条
[1]   AMINO-ACID POLYMORPHISM IN HUMAN PRION PROTEIN AND AGE AT DEATH IN INHERITED PRION DISEASE [J].
BAKER, HF ;
POULTER, M ;
CROW, TJ ;
FRITH, CD ;
LOFTHOUSE, R ;
RIDLEY, RM ;
COLLINGE, J .
LANCET, 1991, 337 (8752) :1286-1286
[2]   SCRAPIE AND CELLULAR PRION PROTEINS DIFFER IN THEIR KINETICS OF SYNTHESIS AND TOPOLOGY IN CULTURED-CELLS [J].
BORCHELT, DR ;
SCOTT, M ;
TARABOULOS, A ;
STAHL, N ;
PRUSINER, SB .
JOURNAL OF CELL BIOLOGY, 1990, 110 (03) :743-752
[3]   THE EPIDEMIOLOGY OF CREUTZFELDT-JAKOB DISEASE - CONCLUSION OF A 15-YEAR INVESTIGATION IN FRANCE AND REVIEW OF THE WORLD LITERATURE [J].
BROWN, P ;
CATHALA, F ;
RAUBERTAS, RF ;
GAJDUSEK, DC ;
CASTAIGNE, P .
NEUROLOGY, 1987, 37 (06) :895-904
[4]   MORTALITY, NEOPLASIA, AND CREUTZFELDT-JAKOB DISEASE IN PATIENTS TREATED WITH HUMAN PITUITARY GROWTH-HORMONE IN THE UNITED-KINGDOM [J].
BUCHANAN, CR ;
PREECE, MA ;
MILNER, RDG .
BRITISH MEDICAL JOURNAL, 1991, 302 (6780) :824-828
[5]  
CAUGHEY B, 1991, J BIOL CHEM, V266, P18217
[6]   NORMAL AND SCRAPIE-ASSOCIATED FORMS OF PRION PROTEIN DIFFER IN THEIR SENSITIVITIES TO PHOSPHOLIPASE AND PROTEASES IN INTACT NEUROBLASTOMA-CELLS [J].
CAUGHEY, B ;
NEARY, K ;
BULLER, R ;
ERNST, D ;
PERRY, LL ;
CHESEBRO, B ;
RACE, RE .
JOURNAL OF VIROLOGY, 1990, 64 (03) :1093-1101
[7]  
COLLINGE J, 1991, AM J HUM GENET, V49, P1351
[8]   GENETIC PREDISPOSITION TO IATROGENIC CREUTZFELDT-JAKOB DISEASE [J].
COLLINGE, J ;
PALMER, MS ;
DRYDEN, AJ .
LANCET, 1991, 337 (8755) :1441-1442
[9]   PRION DEMENTIA WITHOUT CHARACTERISTIC PATHOLOGY [J].
COLLINGE, J ;
OWEN, F ;
POULTER, M ;
LEACH, M ;
CROW, TJ ;
ROSSOR, MN ;
HARDY, J ;
MULLAN, MJ ;
JANOTA, I ;
LANTOS, PL .
LANCET, 1990, 336 (8706) :7-9
[10]  
COLLINGE J, 1992, BRAIN