Cellular Prion Protein Mediates Toxic Signaling of Amyloid Beta

被引：22

作者：

Resenberger, Ulrike K. ^{[1
]}

Winklhofer, Konstanze F. ^{[1
,2
]}

Tatzelt, Joerg ^{[1
,2
]}

机构：

[1] Univ Munich, Adolf Butenandt Inst, DE-80336 Munich, Germany

[2] German Ctr Neurodegenerat Dis DZNE, Munich, Germany

来源：

NEURODEGENERATIVE DISEASES | 2012年 / 10卷 / 1-4期

关键词：

Prion; Alzheimer's disease; N-methyl-D-aspartate; Intrinsically disordered N-terminal domain of PrPC; Neurodegeneration; beta-Sheet; DISEASE; IMPAIRMENT; PRP;

D O I：

10.1159/000332596

中图分类号：

R74 [神经病学与精神病学];

学科分类号：

摘要：

Prion diseases in humans and animals comprise a group of invariably fatal neurodegenerative diseases characterized by the formation of a pathogenic protein conformer designated PrPSc and infectious particles denoted prions. The cellular prion protein (PrPC) has a central role in the pathogenesis of prion disease. First, it is the precursor of PrPSc and infectious prions and second, its expression on neuronal cells is required to mediate toxic effects of prions. To specifically study the role of PrPC as a mediator of toxic signaling, we have developed novel cell culture models, including primary neurons prepared from PrP-deficient mice. Using these approaches we have been able to show that PrPC can interact with and mediate toxic signaling of various beta-sheet-rich conformers of different origins, including amyloid beta, suggesting a pathophysiological role of the prion protein beyond prion diseases. Copyright (C) 2011 S. Karger AG, Basel

引用

页码：298 / 300

页数：3

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