Mitochondrial dysfunction in ACM pathogenesis.

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  • Project duration: -
  • Project status: ongoing
  • Funding:
    Provincial Joint Programme – IT-FWF (Province BZ funding / Project)

Arrhythmogenic cardiomyopathy (ACM) is characterized by arrhythmia and progressive fibro-fatty substitution of the cardiac tissue leading to ventricular dysfunction. Previously published research provided convincing evidence for the involvement of Reactive Oxygen Species (ROS) in tissue degeneration, with our preliminary data also supporting a broader role for mitochondria in ACM development. The present project, built as a collaboration between EURAC (EURAC, Bolzano/Bozen, South-Tyrol, Italy) and the Medical University of Innsbruck (MUI, Innsbruck, Austria) will:

1) Provide a detailed analysis of structural and functional mitochondrial changes in human ACM cell models;

2) Assess the contribution of mitochondrial and non-mitochondrial ROS sources to the ACM pathogenesis;

3)  Obtain evidence that targeting mitochondria can reverse ACM-associated alterations (i.e. increased lipid accumulation and higher ROS production).

Evidence of mitochondrial alterations in primary cardiac stromal cells from arrhythmogenic cardiomyopathy hearts
Philippe R, Volani C, Medici A, Texler B, Pagliaro A, Stadiotti I, Meraviglia V, De Bortoli M, Guarino A, Blumer M, Pompilio G, Pramstaller PP, Sommariva E, Troppmair J, Rossini A (2022)

Conference: Frontiers in CardioVascular Biomedicine 2022 | Budapest | 29.4.2022 - 1.5.2022

Mitochondriale Dysfunktion in der Pathogenese der Arrhytmischen Kardiomyopthie = Disfunzione mitoconriale nella patogenesi della cardiomiopatia aritmogena
Rossini A, Troppmair J, Volani C, Philippe R (2022)

Conference: "Joint Projects" Treffen mit DFG, FWF und SNF | Bozen | 10.8.2022 - 10.8.2022

GCN5 contributes to intracellular lipid accumulation in human primary cardiac stromal cells from patients affected by Arrhythmogenic cardiomyopathy
Volani C, Pagliaro A, Rainer J, Paglia G, Porro B, Stadiotti I, Foco L, Cogliati E, Paolin A, Lagrasta C, Frati C, Corradini E, Falco A, Matzinger T, Picard A, Ermon B, Piazza S, De Bortoli M, Tondo C, Philippe R, Medici A, Lavdas AA., Blumer MJF, Pompilio G, Sommariva E, Pramstaller PP, Troppmair J, Meraviglia V, Rossini A (2022)
Journal article
Journal of Cellular and Molecular Medicine

More information:

GCN5 contributes to intracellular lipid accumulation in human primary cardiac stromal cells from ACM patients
Volani C, Pagliaro A, Meraviglia V, De Bortoli M, Ermon B, Matzinger T, Cogliati E, Paolin A, Pompilio G, Rainer J, Paglia G, Pramstaller PP, Sommariva E, Rossini A (2021)

Conference: 6th world congress of the Tissue Engineering and Regenerative Medicine International Society (TERMIS) | Maastricht (virtual event) | 15.11.2021 - 19.11.2021

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Epigenetics of cellular senescence in aging

Funding: Italy-Austria 2021-2027 (EUTC / EU ...

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