HUMAN PRION DISEASES

被引:72
作者
PARCHI, P
GAMBETTI, P
机构
[1] Division of Neuropathology, Institute of Pathology, Case Western Reserve University, Cleveland, OH 44106
关键词
D O I
10.1097/00019052-199508000-00007
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
Major advances have been made in prion diseases. Recent data indicate that the prion protein is likely to be a synaptic protein with a functional role in synaptic transmission. An impressive body of evidence suggests that (1) the normal prion protein plays a central role in prion replication; (2) the replication process implies an interaction between the normal prion protein and the pathogenic prion protein; and (3) the pathogenic prion protein is the infectious agent, the infectivity of which is dependent on its abnormal conformation, Genetic and protein analyses have expanded the spectrum of prion diseases and have underlined the complexity of genotype-phenotype interactions.
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收藏
页码:286 / 293
页数:8
相关论文
共 55 条
[51]   TRANSMISSION OF CREUTZFELDT-JAKOB-DISEASE FROM HUMANS TO TRANSGENIC MICE EXPRESSING CHIMERIC HUMAN-MOUSE PRION PROTEIN [J].
TELLING, GC ;
SCOTT, M ;
HSIAO, KK ;
FOSTER, D ;
YANG, SL ;
TORCHIA, M ;
SIDLE, KCL ;
COLLINGE, J ;
DEARMOND, SJ ;
PRUSINER, SB .
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA, 1994, 91 (21) :9936-9940
[52]   DEGENERATION OF SKELETAL-MUSCLE, PERIPHERAL-NERVES, AND THE CENTRAL-NERVOUS-SYSTEM IN TRANSGENIC MICE OVEREXPRESSING WILD-TYPE PRION PROTEINS [J].
WESTAWAY, D ;
DEARMOND, SJ ;
CAYETANOCANLAS, J ;
GROTH, D ;
FOSTER, D ;
YANG, SL ;
TORCHIA, M ;
CARLSON, GA ;
PRUSINER, SB .
CELL, 1994, 76 (01) :117-129
[53]   RESCUE OF NEUROPHYSIOLOGICAL PHENOTYPE SEEN IN PRP NULL MICE BY TRANSGENE ENCODING HUMAN PRION PROTEIN [J].
WHITTINGTON, MA ;
SIDLE, KCL ;
GOWLAND, I ;
MEADS, J ;
HILL, AF ;
PALMER, MS ;
JEFFERYS, JGR ;
COLLINGE, J .
NATURE GENETICS, 1995, 9 (02) :197-201
[54]   [URE3] AS AN ALTERED URE2 PROTEIN - EVIDENCE FOR A PRION ANALOG IN SACCHAROMYCES-CEREVISIAE [J].
WICKNER, RB .
SCIENCE, 1994, 264 (5158) :566-569
[55]   CREUTZFELDT-JAKOB-DISEASE TRANSMITTED BY A CADAVERIC DURA-MATER GRAFT [J].
YAMADA, S ;
AIBA, T ;
ENDO, Y ;
HARA, M ;
KITAMOTO, T ;
TATEISHI, J .
NEUROSURGERY, 1994, 34 (04) :740-743