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RNA editing alterations define manifestation of prion diseases

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RNA editing alterations define manifestation of prion diseases. / Kanata, Eirini; Llorens, Franc; Dafou, Dimitra; Dimitriadis, Athanasios; Thüne, Katrin; Xanthopoulos, Konstantinos; Bekas, Nikolaos; Espinosa, Juan Carlos; Schmitz, Matthias; Marín-Moreno, Alba; Capece, Vincenzo; Shormoni, Orr; Andréoletti, Olivier; Bonn, Stefan; Torres, Juan María; Ferrer, Isidre; Zerr, Inga; Sklaviadis, Theodoros.

in: P NATL ACAD SCI USA, Jahrgang 116, Nr. 39, 24.09.2019, S. 19727-19735.

Publikationen: SCORING: Beitrag in Fachzeitschrift/Zeitung › SCORING: Zeitschriftenaufsatz › Forschung › Begutachtung

Harvard

Kanata, E, Llorens, F, Dafou, D, Dimitriadis, A, Thüne, K, Xanthopoulos, K, Bekas, N, Espinosa, JC, Schmitz, M, Marín-Moreno, A, Capece, V, Shormoni, O, Andréoletti, O, Bonn, S, Torres, JM, Ferrer, I, Zerr, I & Sklaviadis, T 2019, 'RNA editing alterations define manifestation of prion diseases', P NATL ACAD SCI USA, Jg. 116, Nr. 39, S. 19727-19735. https://doi.org/10.1073/pnas.1803521116

APA

Kanata, E., Llorens, F., Dafou, D., Dimitriadis, A., Thüne, K., Xanthopoulos, K., Bekas, N., Espinosa, J. C., Schmitz, M., Marín-Moreno, A., Capece, V., Shormoni, O., Andréoletti, O., Bonn, S., Torres, J. M., Ferrer, I., Zerr, I., & Sklaviadis, T. (2019). RNA editing alterations define manifestation of prion diseases. P NATL ACAD SCI USA, 116(39), 19727-19735. https://doi.org/10.1073/pnas.1803521116

Vancouver

Kanata E, Llorens F, Dafou D, Dimitriadis A, Thüne K, Xanthopoulos K et al. RNA editing alterations define manifestation of prion diseases. P NATL ACAD SCI USA. 2019 Sep 24;116(39):19727-19735. https://doi.org/10.1073/pnas.1803521116

Bibtex

@article{e113b8047d8144de821a8877a7301242,
title = "RNA editing alterations define manifestation of prion diseases",
abstract = "Prion diseases are fatal neurodegenerative disorders caused by misfolding of the normal prion protein into an infectious cellular pathogen. Clinically characterized by rapidly progressive dementia and accounting for 85% of human prion disease cases, sporadic Creutzfeldt-Jakob disease (sCJD) is the prevalent human prion disease. Although sCJD neuropathological hallmarks are well-known, associated molecular alterations are elusive due to rapid progression and absence of preclinical stages. To investigate transcriptome alterations during disease progression, we utilized tg340-PRNP129MM mice infected with postmortem material from sCJD patients of the most susceptible genotype (MM1 subtype), a sCJD model that faithfully recapitulates the molecular and pathological alterations of the human disease. Here we report that transcriptomic analyses from brain cortex in the context of disease progression, reveal epitranscriptomic alterations (specifically altered RNA edited pathway profiles, eg., ER stress, lysosome) that are characteristic and possibly protective mainly for preclinical and clinical disease stages. Our results implicate regulatory epitranscriptomic mechanisms in prion disease neuropathogenesis, whereby RNA-editing targets in a humanized sCJD mouse model were confirmed in pathological human autopsy material.",
author = "Eirini Kanata and Franc Llorens and Dimitra Dafou and Athanasios Dimitriadis and Katrin Th{\"u}ne and Konstantinos Xanthopoulos and Nikolaos Bekas and Espinosa, {Juan Carlos} and Matthias Schmitz and Alba Mar{\'i}n-Moreno and Vincenzo Capece and Orr Shormoni and Olivier Andr{\'e}oletti and Stefan Bonn and Torres, {Juan Mar{\'i}a} and Isidre Ferrer and Inga Zerr and Theodoros Sklaviadis",
note = "Copyright {\textcopyright} 2019 the Author(s). Published by PNAS.",
year = "2019",
month = sep,
day = "24",
doi = "10.1073/pnas.1803521116",
language = "English",
volume = "116",
pages = "19727--19735",
journal = "P NATL ACAD SCI USA",
issn = "0027-8424",
publisher = "National Academy of Sciences",
number = "39",

}

RIS

TY - JOUR

T1 - RNA editing alterations define manifestation of prion diseases

AU - Kanata, Eirini

AU - Llorens, Franc

AU - Dafou, Dimitra

AU - Dimitriadis, Athanasios

AU - Thüne, Katrin

AU - Xanthopoulos, Konstantinos

AU - Bekas, Nikolaos

AU - Espinosa, Juan Carlos

AU - Schmitz, Matthias

AU - Marín-Moreno, Alba

AU - Capece, Vincenzo

AU - Shormoni, Orr

AU - Andréoletti, Olivier

AU - Bonn, Stefan

AU - Torres, Juan María

AU - Ferrer, Isidre

AU - Zerr, Inga

AU - Sklaviadis, Theodoros

N1 - Copyright © 2019 the Author(s). Published by PNAS.

PY - 2019/9/24

Y1 - 2019/9/24

N2 - Prion diseases are fatal neurodegenerative disorders caused by misfolding of the normal prion protein into an infectious cellular pathogen. Clinically characterized by rapidly progressive dementia and accounting for 85% of human prion disease cases, sporadic Creutzfeldt-Jakob disease (sCJD) is the prevalent human prion disease. Although sCJD neuropathological hallmarks are well-known, associated molecular alterations are elusive due to rapid progression and absence of preclinical stages. To investigate transcriptome alterations during disease progression, we utilized tg340-PRNP129MM mice infected with postmortem material from sCJD patients of the most susceptible genotype (MM1 subtype), a sCJD model that faithfully recapitulates the molecular and pathological alterations of the human disease. Here we report that transcriptomic analyses from brain cortex in the context of disease progression, reveal epitranscriptomic alterations (specifically altered RNA edited pathway profiles, eg., ER stress, lysosome) that are characteristic and possibly protective mainly for preclinical and clinical disease stages. Our results implicate regulatory epitranscriptomic mechanisms in prion disease neuropathogenesis, whereby RNA-editing targets in a humanized sCJD mouse model were confirmed in pathological human autopsy material.

AB - Prion diseases are fatal neurodegenerative disorders caused by misfolding of the normal prion protein into an infectious cellular pathogen. Clinically characterized by rapidly progressive dementia and accounting for 85% of human prion disease cases, sporadic Creutzfeldt-Jakob disease (sCJD) is the prevalent human prion disease. Although sCJD neuropathological hallmarks are well-known, associated molecular alterations are elusive due to rapid progression and absence of preclinical stages. To investigate transcriptome alterations during disease progression, we utilized tg340-PRNP129MM mice infected with postmortem material from sCJD patients of the most susceptible genotype (MM1 subtype), a sCJD model that faithfully recapitulates the molecular and pathological alterations of the human disease. Here we report that transcriptomic analyses from brain cortex in the context of disease progression, reveal epitranscriptomic alterations (specifically altered RNA edited pathway profiles, eg., ER stress, lysosome) that are characteristic and possibly protective mainly for preclinical and clinical disease stages. Our results implicate regulatory epitranscriptomic mechanisms in prion disease neuropathogenesis, whereby RNA-editing targets in a humanized sCJD mouse model were confirmed in pathological human autopsy material.

U2 - 10.1073/pnas.1803521116

DO - 10.1073/pnas.1803521116

M3 - SCORING: Journal article

C2 - 31492812

VL - 116

SP - 19727

EP - 19735

JO - P NATL ACAD SCI USA

JF - P NATL ACAD SCI USA

SN - 0027-8424

IS - 39

ER -