Of mice and (mad) cows: Transgenic mice help to understand prions

被引:12
作者
Gabizon, R [1 ]
Taraboulos, A [1 ]
机构
[1] HEBREW UNIV JERUSALEM, HADASSAH MED SCH, DEPT MOL BIOL, IL-91010 JERUSALEM, ISRAEL
关键词
D O I
10.1016/S0168-9525(97)01116-5
中图分类号
Q3 [遗传学];
学科分类号
071007 ; 090102 ;
摘要
Prions present a most fascinating biological conundrum. These proteinaceous particles seem to propagate through a chain reaction in which a host protein, PrPC, is post-translationally misfolded to form new prions. By this mechanism they 'replicate' without involvement of specific nucleic acids. Due to their unique modus operandi, prions cause disorders tat can be infectious, inherited and sporadic. Transgenetics has been invaluable in helping to understand this unique phenomenon. Here we describe some of the most salient contributions of transgenic mice to this field.
引用
收藏
页码:264 / 269
页数:6
相关论文
共 54 条
[1]   BIOCHEMICAL AND PHYSICAL-PROPERTIES OF THE PRION PROTEIN FROM 2 STRAINS OF THE TRANSMISSIBLE MINK ENCEPHALOPATHY AGENT [J].
BESSEN, RA ;
MARSH, RF .
JOURNAL OF VIROLOGY, 1992, 66 (04) :2096-2101
[2]   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
[3]   TRANSMISSION OF BOVINE SPONGIFORM ENCEPHALOPATHY AND SCRAPIE TO MICE - STRAIN VARIATION AND THE SPECIES BARRIER [J].
BRUCE, M ;
CHREE, A ;
MCCONNELL, I ;
FOSTER, J ;
PEARSON, G ;
FRASER, H .
PHILOSOPHICAL TRANSACTIONS OF THE ROYAL SOCIETY B-BIOLOGICAL SCIENCES, 1994, 343 (1306) :405-411
[4]   BIOLOGICAL EVIDENCE THAT SCRAPIE AGENT HAS AN INDEPENDENT GENOME [J].
BRUCE, ME ;
DICKINSON, AG .
JOURNAL OF GENERAL VIROLOGY, 1987, 68 :79-89
[5]   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
[6]   NORMAL DEVELOPMENT AND BEHAVIOR OF MICE LACKING THE NEURONAL CELL-SURFACE PRP PROTEIN [J].
BUELER, H ;
FISCHER, M ;
LANG, Y ;
BLUETHMANN, H ;
LIPP, HP ;
DEARMOND, SJ ;
PRUSINER, SB ;
AGUET, M ;
WEISSMANN, C .
NATURE, 1992, 356 (6370) :577-582
[7]  
Carlson GA, 1996, CURR TOP MICROBIOL, V207, P35
[8]   POTENT INHIBITION OF SCRAPIE-ASSOCIATED PRP ACCUMULATION BY CONGO RED [J].
CAUGHEY, B ;
RACE, RE .
JOURNAL OF NEUROCHEMISTRY, 1992, 59 (02) :768-771
[9]   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
[10]   UNALTERED SUSCEPTIBILITY TO BSE IN TRANSGENIC MICE EXPRESSING HUMAN PRION PROTEIN [J].
COLLINGE, J ;
PALMER, MS ;
SIDLE, KCL ;
HILL, AF ;
GOWLAND, I ;
MEADS, J ;
ASANTE, E ;
BRADLEY, R ;
DOEY, LJ ;
LANTOS, PL .
NATURE, 1995, 378 (6559) :779-783