Loss of functional prion protein: A role in prion disorders?

被引：11

作者：

Borchelt, DR

Sisodia, SS

机构：

[1] JOHNS HOPKINS UNIV,SCH MED,DEPT NEUROSCI,BALTIMORE,MD 21205

[2] JOHNS HOPKINS UNIV,SCH MED,NEUROPATHOL LAB,BALTIMORE,MD 21205

来源：

CHEMISTRY & BIOLOGY | 1996年 / 3卷 / 08期

关键词：

D O I：

10.1016/S1074-5521(96)90128-3

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：

To understand the normal function of the prion protein (PrP) and its role in prion disorders, several groups have generated mice lacking PrP. Some of these mice develop symptoms associated with prion diseases, but other experimental evidence suggests that the loss of functional PrP is not the instigating factor in these disorders.

引用

页码：619 / 621

页数：3

共 20 条

[1] SCRAPIE AND CELLULAR PRION PROTEINS DIFFER IN THEIR KINETICS OF SYNTHESIS AND TOPOLOGY IN CULTURED-CELLS
BORCHELT, DR
SCOTT, M
TARABOULOS, A
STAHL, N
PRUSINER, SB
[J]. JOURNAL OF CELL BIOLOGY, 1990, 110 (03) : 743 - 752
[2] Normal host prion protein necessary for scrapie-induced neurotoxicity
Brandner, S
Isenmann, S
Raeber, A
Fischer, M
Sailer, A
Kobayashi, Y
Marino, S
Weissmann, C
Aguzzi, A
[J]. NATURE, 1996, 379 (6563) : 339 - 343
[3] MICE DEVOID OF PRP ARE RESISTANT TO SCRAPIE
BUELER, H
AGUZZI, A
SAILER, A
GREINER, RA
AUTENRIED, P
AGUET, M
WEISSMANN, C
[J]. CELL, 1993, 73 (07) : 1339 - 1347
[4] PRION PROTEIN IS NECESSARY FOR NORMAL SYNAPTIC FUNCTION
COLLINGE, J
WHITTINGTON, MA
SIDLE, KCL
SMITH, CJ
PALMER, MS
CLARKE, AR
JEFFERYS, JGR
[J]. NATURE, 1994, 370 (6487) : 295 - 297
[5] CYSTIC-FIBROSIS IN THE MOUSE BY TARGETED INSERTIONAL MUTAGENESIS
DORIN, JR
DICKINSON, P
ALTON, EWFW
SMITH, SN
GEDDES, DM
STEVENSON, BJ
KIMBER, WL
FLEMING, S
CLARKE, AR
HOOPER, ML
ANDERSON, L
BEDDINGTON, RSP
PORTEOUS, DJ
[J]. NATURE, 1992, 359 (6392) : 211 - 215
[6] Mice deficient for prion protein exhibit normal neuronal excitability and synaptic transmission in the hippocampus
Lledo, PM
Tremblay, P
Dearmond, SJ
Prusiner, SB
Nicoll, RA
[J]. PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA, 1996, 93 (06) : 2403 - 2407
[7] 129/OLA MICE CARRYING A NULL MUTATION IN PRP THAT ABOLISHES MESSENGER-RNA PRODUCTION ARE DEVELOPMENTALLY NORMAL
MANSON, JC
CLARKE, AR
HOOPER, ML
AITCHISON, L
MCCONNELL, I
HOPE, J
[J]. MOLECULAR NEUROBIOLOGY, 1994, 8 (2-3) : 121 - 127
[8] CONVERSION OF ALPHA-HELICES INTO BETA-SHEETS FEATURES IN THE FORMATION OF THE SCRAPIE PRION PROTEINS
PAN, KM
BALDWIN, M
NGUYEN, J
GASSET, M
SERBAN, A
GROTH, D
MEHLHORN, I
HUANG, ZW
FLETTERICK, RJ
COHEN, FE
PRUSINER, SB
[J]. PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA, 1993, 90 (23) : 10962 - 10966
[9] PURIFICATION AND STRUCTURAL STUDIES OF A MAJOR SCRAPIE PRION PROTEIN
PRUSINER, SB
GROTH, DF
BOLTON, DC
KENT, SB
HOOD, LE
[J]. CELL, 1984, 38 (01) : 127 - 134
[10] SCRAPIE AGENT CONTAINS A HYDROPHOBIC PROTEIN
PRUSINER, SB
MCKINLEY, MP
GROTH, DF
BOWMAN, KA
MOCK, NI
COCHRAN, SP
MASIARZ, FR
[J]. PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA-BIOLOGICAL SCIENCES, 1981, 78 (11): : 6675 - 6679

← 1 2 →