Prion Topology and Toxicity

被引：13

作者：

Aguzzi, Adriano ^{[1
]}

Steele, Andrew D. ^{[2
]}

机构：

[1] Univ Zurich Hosp, Inst Neuropathol, CH-8091 Zurich, Switzerland

[2] CALTECH, Div Biol, Pasadena, CA 91125 USA

来源：

CELL | 2009年 / 137卷 / 06期

关键词：

PROTEIN; NEUROTOXICITY; NEURODEGENERATION;

D O I：

10.1016/j.cell.2009.05.041

中图分类号：

Q5 [生物化学]; Q7 [分子生物学];

学科分类号：

070307 [化学生物学]; 071010 [生物化学与分子生物学];

摘要：

Inactivation of mahogunin, an E3 ubiquitin ligase, causes a spongiform encephalopathy resembling prion disease. Chakrabarti and Hegde (2009) now report that prion proteins with aberrant topologies inactivate mahogunin, providing a plausible explanation for certain aspects of prion pathology.

引用

收藏

页码：994 / 996

页数：4

相关论文

共 9 条

[1]

Molecular mechanisms of prion pathogenesis [J].

Aguzzi, Adriano ;

Sigurdson, Christina ;

Heikenwaelder, Mathias .

ANNUAL REVIEW OF PATHOLOGY-MECHANISMS OF DISEASE, 2008, 3 :11-40

[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]

Spongiform degeneration in mahoganoid mutant mice [J].

He, L ;

Lu, XY ;

Jolly, AF ;

Eldridge, AG ;

Watson, SJ ;

Jackson, PK ;

Barsh, GS ;

Gunn, TM .

SCIENCE, 2003, 299 (5607) :710-712

[4]

Disease-associated prion protein oligomers inhibit the 26S proteasome [J].

Kristiansen, Mark ;

Deriziotis, Pelagia ;

Dimcheff, Derek E. ;

Jackson, Graham S. ;

Ovaa, Huib ;

Naumann, Heike ;

Clarke, Anthony R. ;

van Leeuwen, Fijs W. B. ;

Menendez-Benito, Victoria ;

Dantuma, Nico P. ;

Portis, John L. ;

Collinge, John ;

Tabrizi, Sarah J. .

MOLECULAR CELL, 2007, 26 (02) :175-188

[5]

Neurotoxicity and neurodegeneration when PrP accumulates in the cytosol [J].

Ma, JY ;

Wollmann, R ;

Lindquist, S .

SCIENCE, 2002, 298 (5599) :1781-1785

[6]

Reduced translocation of nascent prion protein during ER stress contributes to neurodegeneration [J].

Rane, Neena S. ;

Kang, Sang-Wook ;

Chakrabarti, Oishee ;

Feigenbaum, Lionel ;

Hegde, Ramanujan S. .

DEVELOPMENTAL CELL, 2008, 15 (03) :359-370

[7]

A transmembrane form of the prion protein is localized in the Golgi apparatus of neurons [J].

Stewart, RS ;

Harris, DA .

JOURNAL OF BIOLOGICAL CHEMISTRY, 2005, 280 (16) :15855-15864

[8]

The interaction between cytoplasmic prion protein and the hydrophobic lipid core of membrane correlates with neurotoxicity [J].

Wang, XH ;

Wang, F ;

Arterburn, L ;

Wollmann, R ;

Ma, JY .

JOURNAL OF BIOLOGICAL CHEMISTRY, 2006, 281 (19) :13559-13565

[9]

Cytoplasmic prion protein induces forebrain neurotoxicity [J].

Wang, Xinhe ;

Bowers, Stephanie L. ;

Wang, Fei ;

Pu, Xin-an ;

Nelson, Randy J. ;

Ma, Jiyan .

BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE, 2009, 1792 (06) :555-583