Literature DB >> 11286781

Prions: disease propagation and disease therapy by conformational transmission.

C Soto1, G P Saborío.   

Abstract

Transmissible spongiform encephalopathies - also known as prion-related diseases - are a group of fatal neurodegenerative disorders associated with the misfolding of prion protein. Several unprecedented scientific findings, which have directly confronted popular dogmas in biology, have put prion research in the spotlight. The experimental evidence supports an entirely novel disease mechanism, involving disease transmission by replication of protein conformation. Here, we describe exciting scientific findings that make the prion field attractively heretical, and we propose the transmission of protein conformation as a novel approach to producing drugs to combat a variety of diseases.

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Year:  2001        PMID: 11286781     DOI: 10.1016/s1471-4914(01)01931-1

Source DB:  PubMed          Journal:  Trends Mol Med        ISSN: 1471-4914            Impact factor:   11.951


  7 in total

Review 1.  Prion diseases: current understanding of epidemiology and pathogenesis, and therapeutic advances.

Authors:  Maria Caramelli; Giuseppe Ru; Pierluigi Acutis; Gianluigi Forloni
Journal:  CNS Drugs       Date:  2006       Impact factor: 5.749

2.  The extent of protease resistance of misfolded prion protein is highly dependent on the salt concentration.

Authors:  Luis Concha-Marambio; Rodrigo Diaz-Espinoza; Claudio Soto
Journal:  J Biol Chem       Date:  2013-12-12       Impact factor: 5.157

3.  All-atom molecular dynamics study of EAK16 peptide: the effect of pH on single-chain conformation, dimerization and self-assembly behavior.

Authors:  Soheila Emamyari; Hossein Fazli
Journal:  Eur Biophys J       Date:  2014-03-13       Impact factor: 1.733

4.  Cyclic amplification of prion protein misfolding.

Authors:  Marcelo A Barria; Dennisse Gonzalez-Romero; Claudio Soto
Journal:  Methods Mol Biol       Date:  2012

Review 5.  Synaptic pathology and cell death in the cerebellum in Creutzfeldt-Jakob disease.

Authors:  I Ferrer
Journal:  Cerebellum       Date:  2002-07       Impact factor: 3.847

6.  Prion protein misfolding affects calcium homeostasis and sensitizes cells to endoplasmic reticulum stress.

Authors:  Mauricio Torres; Karen Castillo; Ricardo Armisén; Andrés Stutzin; Claudio Soto; Claudio Hetz
Journal:  PLoS One       Date:  2010-12-29       Impact factor: 3.240

7.  The Endoplasmic Reticulum Chaperone GRP78/BiP Modulates Prion Propagation in vitro and in vivo.

Authors:  Kyung-Won Park; Gyoung Eun Kim; Rodrigo Morales; Fabio Moda; Ines Moreno-Gonzalez; Luis Concha-Marambio; Amy S Lee; Claudio Hetz; Claudio Soto
Journal:  Sci Rep       Date:  2017-03-23       Impact factor: 4.379

  7 in total

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