Regulation of mitochondrial expression

Livrables

Recruitment of Project Manager
Summer School

Summer School: “Advanced Mitochondrial Genetics”– The summer school will cover basic and advanced aspects of mitochondrial genetics, role of mtDNA mutations in development of disease and ageing, regulation of mitochondrial transcription and translation. The special focus of this school would be on mitochondrial disease and therefore external experts both from clinical and basic scientific side will be invited to provide expertise on specific aspects. The summer school will be organized by multiple partners and will be open for application outside of Network. (5 days)

Advertising of positions and Recruitment of ESR

The positions offered to ESRs will be publicized through the ITN website, and also through other means such as host universities/institutes websites, renowned national and international vacancy sites, mailing lists and other academic media, including EURAXESS, the Commission’s job portal (http://ec.europa.eu/euraxess/). The announcements will provide comprehensive information about the application procedure and schedule. The Committee will also make additional details available about employment conditions and re-location arrangements.

Creation of Internal and External Web site

Publications

Mitochondrial transplantation—a possible therapeutic for mitochondrial dysfunction?

Auteurs: Robert N Lightowlers, Zofia MA Chrzanowska‐Lightowlers, Oliver M Russell
Publié dans: EMBO reports, Numéro 21/9, 2020, ISSN 1469-221X
Éditeur: Nature Publishing Group
DOI: 10.15252/embr.202050964

Structural basis of mitochondrial translation

Auteurs: Shintaro Aibara, Vivek Singh, Angelika Modelska, Alexey Amunts
Publié dans: eLife, Numéro 9, 2020, ISSN 2050-084X
Éditeur: eLife Sciences Publications
DOI: 10.7554/elife.58362

A Single Intravenous Injection of AAV-PHP.B-hNDUFS4 Ameliorates the Phenotype of Ndufs4 Mice

Auteurs: Pedro Silva-Pinheiro, Raffaele Cerutti, Marta Luna-Sanchez, Massimo Zeviani, Carlo Viscomi
Publié dans: Molecular Therapy - Methods & Clinical Development, Numéro 17, 2020, Page(s) 1071-1078, ISSN 2329-0501
Éditeur: Cell Press
DOI: 10.1016/j.omtm.2020.04.026

DNA polymerase gamma mutations that impair holoenzyme stability cause catalytic subunit depletion

Auteurs: Pedro Silva-Pinheiro, Carlos Pardo-Hernández, Aurelio Reyes, Lisa Tilokani, Anup Mishra, Raffaele Cerutti, Shuaifeng Li, Dieu-Hien Rozsivalova, Sebastian Valenzuela, Sukru A Dogan, Bradley Peter, Patricio Fernández-Silva, Aleksandra Trifunovic, Julien Prudent, Michal Minczuk, Laurence Bindoff, Bertil Macao, Massimo Zeviani, Maria Falkenberg, Carlo Viscomi
Publié dans: Nucleic Acids Research, 2021, ISSN 0305-1048
Éditeur: Oxford University Press
DOI: 10.1093/nar/gkab282

Quantitative density gradient analysis by mass spectrometry (qDGMS) and complexome profiling analysis (ComPrAn) R package for the study of macromolecular complexes

Auteurs: Petra Páleníková, Michael E. Harbour, Shujing Ding, Ian M. Fearnley, Lindsey Van Haute, Joanna Rorbach, Rick Scavetta, Michal Minczuk, Pedro Rebelo-Guiomar
Publié dans: Biochimica et Biophysica Acta (BBA) - Bioenergetics, Numéro 1862/6, 2021, Page(s) 148399, ISSN 0005-2728
Éditeur: Elsevier BV
DOI: 10.1016/j.bbabio.2021.148399

How much does a disrupted mitochondrial network influence neuronal dysfunction?

Auteurs: Zofia MA Chrzanowska‐Lightowlers, Robert N Lightowlers
Publié dans: EMBO Molecular Medicine, Numéro 11/1, 2019, ISSN 1757-4676
Éditeur: John Wiley & Sons Ltd.
DOI: 10.15252/emmm.201809899

Structural basis for adPEO-causing mutations in the mitochondrial TWINKLE helicase

Auteurs: Bradley Peter, Geraldine Farge, Carlos Pardo-Hernandez, Stefan Tångefjord, Maria Falkenberg
Publié dans: Human Molecular Genetics, Numéro 28/7, 2018, Page(s) 1090-1099, ISSN 0964-6906
Éditeur: Oxford University Press
DOI: 10.1093/hmg/ddy415

High-resolution imaging reveals compartmentalization of mitochondrial protein synthesis in cultured human cells

Auteurs: Matthew Zorkau, Christin A. Albus, Rolando Berlinguer-Palmini, Zofia M. A. Chrzanowska-Lightowlers, Robert N. Lightowlers
Publié dans: Proceedings of the National Academy of Sciences, Numéro 118/6, 2021, Page(s) e2008778118, ISSN 0027-8424
Éditeur: National Academy of Sciences
DOI: 10.1073/pnas.2008778118

Deep sequencing of mitochondrial DNA and characterization of a novel POLG mutation in a patient with arPEO

Auteurs: Carola Hedberg-Oldfors, Bertil Macao, Swaraj Basu, Christopher Lindberg, Bradley Peter, Direnis Erdinc, Jay P. Uhler, Erik Larsson, Maria Falkenberg, Anders Oldfors
Publié dans: Neurology Genetics, Numéro 6/1, 2019, Page(s) e391, ISSN 2376-7839
Éditeur: American Academy of Neurology (AAN)
DOI: 10.1212/nxg.0000000000000391

Mitochondrial RNA granules are critically dependent on mtDNA replication factors Twinkle and mtSSB

Auteurs: Fenna Hensen, Alisa Potter, Selma L van Esveld, Aleix Tarrés-Solé, Arka Chakraborty, Maria Solà, Johannes N Spelbrink
Publié dans: Nucleic Acids Research, Numéro 47/7, 2019, Page(s) 3680-3698, ISSN 0305-1048
Éditeur: Oxford University Press
DOI: 10.1093/nar/gkz047

Duplexing complexome profiling with SILAC to study human respiratory chain assembly defects

Auteurs: Petra Páleníková, Michael E. Harbour, Federica Prodi, Michal Minczuk, Massimo Zeviani, Anna Ghelli, Erika Fernández-Vizarra
Publié dans: Biochimica et Biophysica Acta (BBA) - Bioenergetics, Numéro 1862/6, 2021, Page(s) 148395, ISSN 0005-2728
Éditeur: Elsevier BV
DOI: 10.1016/j.bbabio.2021.148395

Rapid Isolation of the Mitoribosome from HEK Cells

Auteurs: Shintaro Aibara, Juni Andréll, Vivek Singh, Alexey Amunts
Publié dans: Journal of Visualized Experiments, Numéro 140, 2018, ISSN 1940-087X
Éditeur: MYJoVE Corporation
DOI: 10.3791/57877

Visualizing Mitochondrial Ribosomal RNA and Mitochondrial Protein Synthesis in Human Cell Lines

Auteurs: Matthew Zorkau, Yasmin Proctor-Kent, Rolando Berlinguer-Palmini, Andrew Hamilton, Zofia M. Chrzanowska-Lightowlers, Robert N. Lightowlers
Publié dans: Mitochondrial Gene Expression - Methods and Protocols, Numéro 2192, 2021, Page(s) 159-181, ISBN 978-1-0716-0833-3
Éditeur: Springer US
DOI: 10.1007/978-1-0716-0834-0_13

Application of Cryo-EM for Visualization of Mitoribosomes

Auteurs: Vivek Singh, Alexey Amunts
Publié dans: Mitochondrial Gene Expression - Methods and Protocols, Numéro 2192, 2021, Page(s) 197-210, ISBN 978-1-0716-0833-3
Éditeur: Springer US
DOI: 10.1007/978-1-0716-0834-0_15

Visualization of Mitochondrial RNA Granules in Cultured Cells Using 5-Bromouridine Labeling

Auteurs: Vanessa Joanne Xavier, Jean-Claude Martinou
Publié dans: Mitochondrial Gene Expression - Methods and Protocols, Numéro 2192, 2021, Page(s) 69-73, ISBN 978-1-0716-0833-3
Éditeur: Springer US
DOI: 10.1007/978-1-0716-0834-0_6

A novel system to monitor mitochondrial translation in yeast

Auteurs: Tamara Suhm, Lukas Habernig, Magdalena Rzepka, Jayasankar Mohanakrishnan Kaimal, Claes Andréasson, Sabrina Buettner, Martin Ott
Publié dans: Microbial Cell, Numéro 5/3, 2018, Page(s) 158-164, ISSN 2311-2638
Éditeur: http://www.shared-science.org/
DOI: 10.15698/mic2018.03.621

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