The many shades of prion strain adaptation

被引:27
作者
Baskakov, Ilia V. [1 ]
机构
[1] Univ Maryland, Sch Med, Dept Anat & Neurobiol, Ctr Biomed Engn & Technol, Baltimore, MD 21201 USA
关键词
prion protein; prion diseases; synthetic prions; strain adaptation; species barrier; deformed templating mechanism; neurodegenerative diseases; MISFOLDING CYCLIC AMPLIFICATION; DRUG-RESISTANCE; BSE AGENT; WILD-TYPE; PROTEIN; REPLICATION; MICE; ENCEPHALOPATHY; TRANSMISSION; SELECTION;
D O I
10.4161/pri.27836
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
In several recent studies transmissible prion disease was induced in animals by inoculation with recombinant prion protein amyloid fibrils produced in vitro. Serial transmission of amyloid fibrils gave rise to a new class of prion strains of synthetic origin. Gradual transformation of disease phenotypes and PrPSc properties was observed during serial transmission of synthetic prions, a process that resembled the phenomenon of prion strain adaptation. The current article discusses the remarkable parallels between phenomena of prion strain adaptation that accompanies cross-species transmission and the evolution of synthetic prions occurring within the same host. Two alternative mechanisms underlying prion strain adaptation and synthetic strain evolution are discussed. The current article highlights the complexity of the prion transmission barrier and strain adaptation and proposes that the phenomenon of prion adaptation is more common than previously thought.
引用
收藏
页码:169 / 172
页数:4
相关论文
共 44 条
[11]   Isolation of phosphatidylethanolamine as a solitary cofactor for prion formation in the absence of nucleic acids [J].
Deleault, Nathan R. ;
Piro, Justin R. ;
Walsh, Daniel J. ;
Wang, Fei ;
Ma, Jiyan ;
Geoghegan, James C. ;
Supattapone, Surachai .
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA, 2012, 109 (22) :8546-8551
[12]   Convergent Replication of Mouse Synthetic Prion Strains [J].
Ghaemmaghami, Sina ;
Colby, David W. ;
Nguyen, Hoang-Oanh B. ;
Hayashi, Shigenari ;
Oehler, Abby ;
DeArmond, Stephen J. ;
Prusiner, Stanley B. .
AMERICAN JOURNAL OF PATHOLOGY, 2013, 182 (03) :866-874
[13]   Conformational Transformation and Selection of Synthetic Prion Strains [J].
Ghaemmaghami, Sina ;
Watts, Joel C. ;
Hoang-Oanh Nguyen ;
Hayashi, Shigenari ;
DeArmond, Stephen J. ;
Prusiner, Stanley B. .
JOURNAL OF MOLECULAR BIOLOGY, 2011, 413 (03) :527-542
[14]   Continuous Quinacrine Treatment Results in the Formation of Drug-Resistant Prions [J].
Ghaemmaghami, Sina ;
Ahn, Misol ;
Lessard, Pierre ;
Giles, Kurt ;
Legname, Giuseppe ;
DeArmond, Stephen J. ;
Prusiner, Stanley B. .
PLOS PATHOGENS, 2009, 5 (11)
[15]   Changes in prion replication environment cause prion strain mutation [J].
Gonzalez-Montalban, Nuria ;
Lee, Young Jin ;
Makarava, Natallia ;
Savtchenko, Regina ;
Baskakov, Ilia V. .
FASEB JOURNAL, 2013, 27 (09) :3702-3710
[16]   Assessment of Strain-Specific PrPSc Elongation Rates Revealed a Transformation of PrPSc Properties during Protein Misfolding Cyclic Amplification [J].
Gonzalez-Montalban, Nuria ;
Baskakov, Ilia V. .
PLOS ONE, 2012, 7 (07)
[17]   The same prion strain causes vCJD and BSE [J].
Hill, AF ;
Desbruslais, M ;
Joiner, S ;
Sidle, KCL ;
Gowland, I ;
Collinge, J ;
Doey, LJ ;
Lantos, P .
NATURE, 1997, 389 (6650) :448-450
[18]   Species-barrier-independent prion replication in apparently resistant species [J].
Hill, AF ;
Joiner, S ;
Linehan, J ;
Desbruslais, M ;
Lantos, PL ;
Collinge, J .
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA, 2000, 97 (18) :10248-10253
[19]   Transmission of the BSE agent to mice in the absence of detectable abnormal prion protein [J].
Lasmezas, CI ;
Deslys, JP ;
Robain, O ;
Jaegly, A ;
Beringue, V ;
Peyrin, JM ;
Fournier, JG ;
Hauw, JJ ;
Rossier, J ;
Dormont, D .
SCIENCE, 1997, 275 (5298) :402-405
[20]   Synthetic mammalian prions [J].
Legname, G ;
Baskakov, IV ;
Nguyen, HOB ;
Riesner, D ;
Cohen, FE ;
DeArmond, SJ ;
Prusiner, SB .
SCIENCE, 2004, 305 (5684) :673-676