Genesis of tramsmissible protein states via deformed templating.

Prion
Natallia Makarava, Ilia V Baskakov

Abstract

Prion replication occurs via a template-assisted mechanism, which postulates that the folding pattern of a newly recruited polypeptide chain accurately reproduces that of a template. The concept of prion-like template-assisted propagation of an abnormal protein conformation has been expanded to amyloidogenic proteins associated with Alzheimer, Parkinson, Huntington diseases, amyotrophic lateral sclerosis and others. Recent studies demonstrated that authentic PrP (Sc)  and transmissible prion disease could be generated in wild type animals by inoculation of recombinant prion protein amyloid fibrils, which are structurally different from PrP (Sc) and lack any detectable PrP (Sc)  particles. Here we discuss a new replication mechanism designated as "deformed templating," according to which fibrils with one cross-β folding pattern can seed formation of fibrils or particles with a fundamentally different cross-β folding pattern. Transformation of cross-β folding pattern via deformed templating provides a mechanistic explanation behind genesis of transmissible protein states induced by amyloid fibrils that are considered to be non-infectious. We postulate that deformed templating is responsible for generating conformationally divers...Continue Reading

References

Oct 6, 1998·Annual Review of Biochemistry·F E Cohen, S B Prusiner
Nov 13, 1998·Proceedings of the National Academy of Sciences of the United States of America·S B Prusiner
Oct 17, 2003·Nature·Nathan R DeleaultSurachai Supattapone
Dec 9, 2003·Trends in Microbiology·Andrew F Hill, John Collinge
May 5, 2004·Proceedings of the National Academy of Sciences of the United States of America·Salvador VenturaLuis Serrano
Jul 14, 2004·Proceedings of the National Academy of Sciences of the United States of America·Magdalena I IvanovaDavid Eisenberg
Aug 3, 2004·Science·Giuseppe LegnameStanley B Prusiner
Aug 25, 2004·Proceedings of the National Academy of Sciences of the United States of America·Irina L DerkatchSusan W Liebman
Apr 15, 2005·Proceedings of the National Academy of Sciences of the United States of America·Katarzyna LundmarkPer Westermark
Apr 27, 2005·Cell·Joaquín CastillaClaudio Soto
Mar 31, 2009·The Journal of Biological Chemistry·Natallia MakaravaIlia V Baskakov
Oct 7, 2009·Proceedings of the National Academy of Sciences of the United States of America·Holger WilleGerald Stubbs
Nov 17, 2009·Proceedings of the National Academy of Sciences of the United States of America·David W ColbyStanley B Prusiner
Jan 7, 2010·Acta Neuropathologica·Natallia MakaravaIlia V Baskakov
Apr 2, 2010·Journal of Cell Science·Mimi CushmanJames Shorter
Jun 18, 2010·Journal of Molecular Biology·Valeriy G OstapchenkoIlia V Baskakov
Feb 25, 2011·PLoS Pathogens·Nuria Gonzalez-MontalbanIlia V Baskakov
Oct 27, 2011·Annals of Neurology·Mathias Jucker, Lary C Walker

❮ Previous
Next ❯

Citations

Jan 25, 2013·Archives of Toxicology·Jan K Schluesener, Hermann J Schluesener
Dec 18, 2013·PLoS Pathogens·Natallia Makarava, Ilia V Baskakov
Feb 14, 2016·The American Journal of Pathology·Natallia MakaravaIlia V Baskakov
Feb 13, 2014·Prion·Ilia V Baskakov
Aug 4, 2018·Molecular Neurobiology·Edoardo BistaffaGiuseppe Legname
Mar 26, 2019·PLoS Pathogens·Cassandra M BurkeSurachai Supattapone
Jun 5, 2013·FASEB Journal : Official Publication of the Federation of American Societies for Experimental Biology·Nuria Gonzalez-MontalbanIlia V Baskakov
Nov 12, 2015·Acta Neuropathologica Communications·Natallia MakaravaIlia V Baskakov
May 19, 2020·PLoS Pathogens·Cassandra M BurkeSurachai Supattapone
Jun 26, 2020·PLoS Biology·Jesús R Requena
Jan 1, 2013·Intrinsically Disordered Proteins·Leonid Breydo
Oct 31, 2020·Frontiers in Bioengineering and Biotechnology·Giovanni SpagnolliJesús R Requena
Aug 21, 2019·Ageing Research Reviews·Paramita ChaudhuriKarunakar Kar
Jan 19, 2021·The FEBS Journal·Katerina KonstantouleaJoost Schymkowitz
Oct 4, 2018·Chemical Reviews·Corey J WilsonJennifer M Heemstra

❮ Previous
Next ❯

Methods Mentioned

BETA
X-ray

Related Concepts

Related Feeds

Alzheimer's Disease: APP

Amyloid precursor protein (APP) proteolysis is critical for the development of Alzheimer's disease, a neurodegenerative disease associated with accumulation of amyloid plaques in the brain. Here is the latest research on APP and Alzheimer's disease.

Amyloid Lateral Sclerosis

Amyotrophic Lateral Sclerosis (ALS) is a progressive nervous system disease associated with the death of neurons that control voluntary muscles. Discover the latest research on ALS here.

Alzheimer's Disease: Amyloid Beta

Alzheimer's disease is a neurodegenerative disease associated with the accumulation of amyloid plaques in the brain; these plaques are comprised of amyloid beta deposits. Here is the latest research in this field.

ALS: Prions

Prions are misfolded proteins which characterize several fatal neurodegenerative diseases. Prion-like mechanisms are associated with the pathogenesis of Amyotrophic Lateral Sclerosis (ALS). Here is the latest research on ALS and prions.

Related Papers

The American Journal of Pathology
Sina GhaemmaghamiS B Prusiner
The Journal of Neuroscience : the Official Journal of the Society for Neuroscience
Natallia MakaravaIlia V Baskakov
© 2021 Meta ULC. All rights reserved