User talk:Beno Marija/REMIX

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User talk:Beno Marija/REMIX

REMIX - Regulation of mitochondrial expression

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REMIX: H2020-MSCA-ITN-2016 - Contents and Aims


Mitochondria are essential organelles found in every eukaryotic cell, required to convert food into usable energy. The mitochondrial oxidative phosphorylation (OXPHOS) system, which produces the majority of cellular energy in the form of ATP, is controlled by two distinct genomes: the nuclear and the mitochondrial genome (mtDNA). Mutations in mitochondrial genes encoded by either genome could cause diseases affecting OXPHOS system, called mitochondrial diseases, whose prevalence has been estimated to be ≈1:8500. Moreover, dysfunction of mitochondrial OXPHOS system has emerged as a key factor in a myriad of “common” diseases, including neurodegenerative and metabolic disorders like Parkinson’s and Alzheimer’s Disease, Type 2 Diabetes, and was linked to aging process. Despite all this, it is surprising that our understanding of the mechanisms governing the mitochondrial gene expression and its associated pathologies remain superficial and therapeutic interventions unexplored. The basic machineries for mtDNA replication, mtDNA transcription and mitochondrial translation are known, but the regulation of these processes in response to metabolic demands is poorly understood. The complex nature of mitochondrial gene expression that relies on two different genomes calls for a multidisciplinary approach where different teams of researchers join forces. Studies in this area are not only of basic scientific interest but may also provide new avenues towards treatment of mitochondrial dysfunction in a variety of human diseases.
The key aim of the REMIX Network is combine the skills of European research groups to provide strategic training of the next generation of scientists through a programme that will progress in the elucidation of the molecular mechanisms and pathways that regulate mitochondrial gene expression.


  • Work Package 1 will cover the management of the REMIX ITN. The overall aim of this Work Package is to manage all project, financial, communication, and human reseource-related aspects of this Network.
  • The main objective of this Work Package is to provide a comprehensive and multidisciplinary training program for early stage reseacher in mitochondrial biology.
  • The overall aim of this Work Package is to coordinate the exploitation of the project results, to coordinate the dissemination of the project's outputs to academia, poliy makers and genreal public
  • The main objective of this Work Package is to identify and characterize novel factors involved in maintenance of mitochondrial genome.
  • The objective of this Work Package is to further characterize mitochondrial transcription and RNA processing.
  • The overall aim of this Work Package is to identify novel factors involved in mitochondrial translation and ribosomal assembly and to understand molecular mechanisms that govern their mode of action.

Specific objectives
  • Defects in the synthesis, import, and modification of mitochondrial polypeptides as well as defective assembly of multi-subunit OXPHOS complexes result in mitochondrial diseases that primarily affect tissues with a high metabolic demand, such as the nervous, muscular, cardiac, ocular and endocrine systems.
  • REMIX ITN is therefore designed to bring together a multidisciplinary team, which harnesses the different expertise (genetics, molecular and cell biology, biochemistry and structural biology) of researchers across Europe, aimed to elucidate precise molecular mechanisms of mitochondrial gene expression.
  • The consortium will facilitate this effort by pooling research tools and resources, stimulating researcher exchange and training-promoting knowledge transfer, and enhancing scientific communication between researchers with complementary expertise

OROBOROS project involvement

Project Duration

Start: 1st Oktober 2016 2020-09-30
End: 30th September 2020
Ongoing project


REMIX Marie Skłodowska-Curie Participants/Beneficiaries

Selected publications

Research innovation products: OROBOROS Oxygraph-2k (O2k) for high-resolution respirometry, O2k-Fluorometer.
  1. Gnaiger E (2014) 4th ed. Mitochondr Physiol Network 19.12. OROBOROS MiPNet Publications, Innsbruck: 80 pp. ISBN 978-3-9502399-8-0
  2. Pesta D, et al. Methods Mol Biol 810:25-58. (2012)
  3. Kristiansen G, et al. J Biol Chem 286: 43417-43428. (2011)
  4. Eberhart K et al. Biochim Biophys Acta 1807: 719-725. (2011)
  5. Scandurra FM, et al. Adv Exp Med Biol 662: 7-25. (2010)