Mammalian prion proteins

被引:72
作者
Jackson, GS
Clarke, AR
机构
[1] Univ London Imperial Coll Sci Technol & Med, Sch Med, Med Res Council Prion Unit, London W2 1PG, England
[2] Univ London Imperial Coll Sci Technol & Med, Sch Med, Dept Neurogenet, London W2 1PG, England
[3] Univ Bristol, Sch Med Sci, Dept Biochem, Bristol BS8 1TD, Avon, England
关键词
D O I
10.1016/S0959-440X(99)00051-2
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
The past two years have seen the extension of our knowledge on the cellular prion protein structure with new NMR data on both the hamster and human proteins, In addition, the folding dynamics of two cellular prion proteins have been elucidated. There are now several examples of recombinant prion proteins that are able to adopt different conformations in solution and recent work on the molecular basis of prion strains has done much to consolidate the protein-only hypothesis. Important advances in relating disease to structure have also been made through the identification of the minimal prion protein fragment that is capable of conferring susceptibility to and propagation of the scrapie agent.
引用
收藏
页码:69 / 74
页数:6
相关论文
共 42 条
[1]   IDENTIFICATION OF A PROTEIN THAT PURIFIES WITH THE SCRAPIE PRION [J].
BOLTON, DC ;
MCKINLEY, MP ;
PRUSINER, SB .
SCIENCE, 1982, 218 (4579) :1309-1311
[2]   Normal host prion protein (PrPc) is required for scrapie spread within the central nervous system [J].
Brandner, S ;
Raeber, A ;
Sailer, A ;
Blattler, T ;
Fischer, M ;
Weissmann, C ;
Aguzzi, A .
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA, 1996, 93 (23) :13148-13151
[3]   Normal host prion protein necessary for scrapie-induced neurotoxicity [J].
Brandner, S ;
Isenmann, S ;
Raeber, A ;
Fischer, M ;
Sailer, A ;
Kobayashi, Y ;
Marino, S ;
Weissmann, C ;
Aguzzi, A .
NATURE, 1996, 379 (6563) :339-343
[4]   Prion protein-deficient cells show altered response to oxidative stress due to decreased SOD-1 activity [J].
Brown, DR ;
SchulzSchaeffer, WJ ;
Schmidt, B ;
Kretzschmar, HA .
EXPERIMENTAL NEUROLOGY, 1997, 146 (01) :104-112
[5]   Transmissions to mice indicate that 'new variant' CJD is caused by the BSE agent [J].
Bruce, ME ;
Will, RG ;
Ironside, JW ;
McConnell, I ;
Drummond, D ;
Suttie, A ;
McCardle, L ;
Chree, A ;
Hope, J ;
Birkett, C ;
Cousens, S ;
Fraser, H ;
Bostock, CJ .
NATURE, 1997, 389 (6650) :498-501
[6]   MICE DEVOID OF PRP ARE RESISTANT TO SCRAPIE [J].
BUELER, H ;
AGUZZI, A ;
SAILER, A ;
GREINER, RA ;
AUTENRIED, P ;
AGUET, M ;
WEISSMANN, C .
CELL, 1993, 73 (07) :1339-1347
[7]   Inhibition of protease-resistant prion protein formation by porphyrins and phthalocyanines [J].
Caughey, WS ;
Raymond, LD ;
Horiuchi, M ;
Caughey, B .
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA, 1998, 95 (21) :12117-12122
[8]   Molecular analysis of prion strain variation and the aetiology of 'new variant' CJD [J].
Collinge, J ;
Sidle, KCL ;
Meads, J ;
Ironside, J ;
Hill, AF .
NATURE, 1996, 383 (6602) :685-690
[9]   PRION PROTEIN IS NECESSARY FOR NORMAL SYNAPTIC FUNCTION [J].
COLLINGE, J ;
WHITTINGTON, MA ;
SIDLE, KCL ;
SMITH, CJ ;
PALMER, MS ;
CLARKE, AR ;
JEFFERYS, JGR .
NATURE, 1994, 370 (6487) :295-297
[10]   Surgical treatment and risk of sporadic Creutzfeldt-Jakob disease: a case-control study [J].
Collins, S ;
Law, MG ;
Fletcher, A ;
Boyd, A ;
Kaldor, J ;
Masters, CL .
LANCET, 1999, 353 (9154) :693-697