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Prion Respiration SIGNED

The Role of Complex I Assembly Factors during Prion Diseases: Insights into Mitochondrial Neurobiology

Total Cost €


EC-Contrib. €






 Prion Respiration project word cloud

Explore the words cloud of the Prion Respiration project. It provides you a very rough idea of what is the project "Prion Respiration" about.

transport    broad    insights    disorders    structure    characterization    structural    stresses    mitochondria    apoptosis    encoded    afs    cells    age    syndrome    altered    cell    mutations    proteins    functional    identification    nuclear    energy    demonstrates    em    biology    aberrant    strategies    survival    decrease    saxs    prions    ndufaf1    components    disease    levels    interfering    respiration    respiratory    childhood    declines    cryo    denoted    prion    neurons    function    cellular    models    generate    chain    leigh    constitute    found    animal    prevent    diminish    ecsit    neurodegenerative    complexes    correct    integrity    subsequently    drug    neurological    lived    mitochondrial    exacerbated    neurodegeneration    energetic    mechanisms    alzheimer    underlying    aging    diseases    assembly    dysfunctions    defects    neuronal    electron    biological    dieseas    spectrum    responsible    helping    oxidative    misassembly    acad9    parkinson   

Project "Prion Respiration" data sheet

The following table provides information about the project.


Organization address
address: VIA CALEPINA 14
city: TRENTO
postcode: 38122

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 Italy [IT]
 Total cost 171˙473 €
 EC max contribution 171˙473 € (100%)
 Programme 1. H2020-EU.1.3.2. (Nurturing excellence by means of cross-border and cross-sector mobility)
 Code Call H2020-MSCA-IF-2018
 Funding Scheme MSCA-IF-EF-ST
 Starting year 2019
 Duration (year-month-day) from 2019-10-01   to  2021-09-30


Take a look of project's partnership.

# participants  country  role  EC contrib. [€] 
1    UNIVERSITA DEGLI STUDI DI TRENTO IT (TRENTO) coordinator 171˙473.00


 Project objective

Mitochondria have a crucial role in cell survival and apoptosis, in particular in long-lived cells such as neurons. Mitochondria generate energetic potential through respiratory complexes I to IV, which constitute the electron transport chain. A number of studies demonstrates that mitochondrial integrity declines as a function of aging and dysfunctions may be exacerbated by age-related neurodegenerative diseases. Altered levels of complex I proteins have been found to be directly responsible for a decrease in energy production in Alzheimer’s and Parkinson's disease. Recently, a number of nuclear encoded mitochondrial proteins, denoted as “assembly factors” (AFs), have been identified as crucial components helping the complex I assembly. Defects in AFs (due to mutations or aberrant AFs processing) may cause complex I misassembly and mitochondrial dysfunctions leading to a broad spectrum of diseases, including neurological disorders in childhood-related dieseas as Leigh syndrome. The functional role of AFs during neurodegeneration has not been investigated yet. With the “Prion Respiration” project I propose to develop a research approach aimed at the identification of the biological role of three crucial complex I AFs (namely ECSIT, NDUFAF1 and ACAD9) in cellular and animal models using prion diseases as robust system to study neurodegenerative diseases. Subsequently, the role of prions in interfering the with the correct assembly of the AFs will be investigated using structural biology approaches, as SAXS and cryo-EM methods. The structural and functional characterization of key proteins involved in mitochondrial respiration will provide insights into the underlying mechanisms involved in neuronal function and may represent novel targets for structure-based drug design strategies to prevent or diminish the oxidative stresses underlying neurodegeneration.

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The information about "PRION RESPIRATION" are provided by the European Opendata Portal: CORDIS opendata.

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