PARADOXICAL SHORTENING OF SCRAPIE INCUBATION TIMES BY EXPRESSION OF PRION PROTEIN TRANSGENES DERIVED FROM LONG INCUBATION PERIOD MICE

被引:105
作者
WESTAWAY, D
MIRENDA, CA
FOSTER, D
ZEBARJADIAN, Y
SCOTT, M
TORCHIA, M
YANG, SL
SERBAN, H
DEARMOND, SJ
EBELING, C
PRUSINER, SB
CARLSON, GA
机构
[1] UNIV CALIF SAN FRANCISCO,DEPT BIOCHEM & BIOPHYS,SAN FRANCISCO,CA 94143
[2] UNIV CALIF SAN FRANCISCO,DEPT PATHOL,SAN FRANCISCO,CA 94143
[3] MCLAUGHLIN RES INST,GREAT FALLS,MT 59401
关键词
D O I
10.1016/0896-6273(91)90074-A
中图分类号
Q189 [神经科学];
学科分类号
071006 ;
摘要
Prolonged incubation times for experimental scrapie in I/LnJ mice are dictated by a dominant gene linked to the prion protein gene (Prn-p). Transgenic mice were analyzed to discriminate between an effect of the I/LnJ Prn-p(b) allele and a distinct incubation time locus designated Prn-i. Paradoxically, 4 independent Prn-p(b) transgenic mouse lines had scrapie incubation times shorter than nontransgenic controls, instead of the anticipated prolonged incubation periods. Aberrant or overexpression of the Prn-p(b) transgenes may dictate abbreviated incubation times, masking genuine Prn-p/Prn-i congruence; alternatively, a discrete Prn-i gene lies adjacent to Prn-p.
引用
收藏
页码:59 / 68
页数:10
相关论文
共 43 条
[1]   SCRAPIE AND CELLULAR PRP ISOFORMS ARE ENCODED BY THE SAME CHROMOSOMAL GENE [J].
BASLER, K ;
OESCH, B ;
SCOTT, M ;
WESTAWAY, D ;
WALCHLI, M ;
GROTH, DF ;
MCKINLEY, MP ;
PRUSINER, SB ;
WEISSMANN, C .
CELL, 1986, 46 (03) :417-428
[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]   BIOLOGICAL EVIDENCE THAT SCRAPIE AGENT HAS AN INDEPENDENT GENOME [J].
BRUCE, ME ;
DICKINSON, AG .
JOURNAL OF GENERAL VIROLOGY, 1987, 68 :79-89
[4]   SCRAPIE-INFECTED MURINE NEURO-BLASTOMA CELLS PRODUCE PROTEASE-RESISTANT PRION PROTEINS [J].
BUTLER, DA ;
SCOTT, MRD ;
BOCKMAN, JM ;
BORCHELT, DR ;
TARABOULOS, A ;
HSIAO, KK ;
KINGSBURY, DT ;
PRUSINER, SB .
JOURNAL OF VIROLOGY, 1988, 62 (05) :1558-1564
[5]   PRIMARY STRUCTURE OF PRION PROTEIN MAY MODIFY SCRAPIE ISOLATE PROPERTIES [J].
CARLSON, GA ;
WESTAWAY, D ;
DEARMOND, SJ ;
PETERSONTORCHIA, M ;
PRUSINER, SB .
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA, 1989, 86 (19) :7475-7479
[6]   LINKAGE OF PRION PROTEIN AND SCRAPIE INCUBATION-TIME GENES [J].
CARLSON, GA ;
KINGSBURY, DT ;
GOODMAN, PA ;
COLEMAN, S ;
MARSHALL, ST ;
DEARMOND, S ;
WESTAWAY, D ;
PRUSINER, SB .
CELL, 1986, 46 (04) :503-511
[7]   GENETICS AND POLYMORPHISM OF THE MOUSE PRION GENE-COMPLEX - CONTROL OF SCRAPIE INCUBATION-TIME [J].
CARLSON, GA ;
GOODMAN, PA ;
LOVETT, M ;
TAYLOR, BA ;
MARSHALL, ST ;
PETERSONTORCHIA, M ;
WESTAWAY, D ;
PRUSINER, SB .
MOLECULAR AND CELLULAR BIOLOGY, 1988, 8 (12) :5528-5540
[8]  
CHANDLER RL, 1961, LANCET, V1, P1378
[9]  
CHOMCZYNSKI P, 1987, ANAL BIOCHEM, V162, P156, DOI 10.1016/0003-2697(87)90021-2
[10]   ROLE OF CHROMOSOME TRANSLOCATIONS IN HUMAN NEOPLASIA [J].
CROCE, CM .
CELL, 1987, 49 (02) :155-156