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Tissue-specific mitochondrial signaling and adaptations to mistranslation

Total Cost €


EC-Contrib. €






Project "MISTRANSMITO" data sheet

The following table provides information about the project.


Organization address
postcode: 14

contact info
title: n.a.
name: n.a.
surname: n.a.
function: n.a.
email: n.a.
telephone: n.a.
fax: n.a.

 Coordinator Country Finland [FI]
 Total cost 1˙354˙507 €
 EC max contribution 1˙354˙507 € (100%)
 Programme 1. H2020-EU.1.1. (EXCELLENT SCIENCE - European Research Council (ERC))
 Code Call ERC-2014-STG
 Funding Scheme ERC-STG
 Starting year 2015
 Duration (year-month-day) from 2015-07-01   to  2020-06-30


Take a look of project's partnership.

# participants  country  role  EC contrib. [€] 


 Project objective

Mitochondria play a central role in the energy metabolism of our bodies and their defects give rise to a large variety of clinical phenotypes that can affect practically any tissue. The mechanisms for the tissue-specific outcomes of mitochondrial diseases are poorly understood. Mitochondrial energy production relies on two separate protein synthesis machineries, cytoplasmic and mitochondrial, but the mechanisms regulating the concerted actions between the two are largely to be discovered. Defects in either protein synthesis system that lead to accumulation of mistranslated mitochondrial proteins, intrinsic or imported from the cytoplasm, result in stress signals from mitochondria and in adaptive responses within the organelle and the entire cell. My hypothesis is that some of these signals and adaptive mechanisms are tissue-specific. My group will test the hypothesis by 1) generating and characterizing mouse models of cytoplasmic and mitochondrial mistranslation to be able to address our questions in different tissues. 2) We will develop methods for detection of ribosome stalling in mouse tissues to identify the consequences of mistranslation for individual proteins. 3) We will use systems biology approaches to identify stress signal responses to mitochondrial and/or cytoplasmic mistranslation using different tissues of our models, to identify those that are unique or global. 4) Our previous study has identified an interesting candidate responder to mistranslation stress and we will test the role of this factor in knockout animal models and by crossing with the mistranslation mice. I expect to gain important new knowledge of in vivo responses to mistranslation and execution of quality control. This proposal investigates key questions in understanding differential tissue involvement in metabolic defects, and will provide new directions for utilization of tissue-specific adaptations in finding interventions for mitochondrial diseases.


year authors and title journal last update
List of publications.
2017 Taru Hilander, Xiao-Long Zhou, Svetlana Konovalova, Fu-Ping Zhang, Liliya Euro, Dmitri Chilov, Matti Poutanen, Joseph Chihade, En-Duo Wang, Henna Tyynismaa
Editing activity for eliminating mischarged tRNAs is essential in mammalian mitochondria
published pages: 849-860, ISSN: 0305-1048, DOI: 10.1093/nar/gkx1231
Nucleic Acids Research 46/2 2019-05-28
2018 Svetlana Konovalova, Xiaonan Liu, Pooja Manjunath, Sundar Baral, Nirajan Neupane, Taru Hilander, Yang Yang, Diego Balboa, Mügen Terzioglu, Liliya Euro, Markku Varjosalo, Henna Tyynismaa
Redox regulation of GRPEL2 nucleotide exchange factor for mitochondrial HSP70 chaperone
published pages: 37-45, ISSN: 2213-2317, DOI: 10.1016/j.redox.2018.07.024
Redox Biology 19 2019-05-22

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