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Information on the principal investigator and host institution

Information of the project and the call

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The role of the mitochondrial dysfunction-related intracellular oxidative stress in pathogenesis of mitochondrial disorders

2014/15/B/NZ1/00490

Keywords:

mitochondria oxidative stress mitochondrial dysfunction mitochondrial disorders

Descriptors:

  • NZ1_2:
  • NZ1_1:

Panel:

NZ1 - Molecular biology, structural biology, biotechnology: molecular biology, structural biology, biotechnology

Host institution :

Instytut Biologii Doświadczalnej im. M. Nenckiego PAN

woj. mazowieckie

Other projects carried out by the institution 

Principal investigator (from the host institution):

prof. Mariusz Roman Więckowski 

Number of co-investigators in the project: 5

Call: OPUS 8 - announced on 2014-09-15

Amount awarded: 1 200 000 PLN

Project start date (Y-m-d): 2015-07-13

Project end date (Y-m-d): 2019-07-12

Project duration:: 48 months (the same as in the proposal)

Project status: Project settled

Information in the final report

  • Publication in academic press/journals (11)
  • Book publications / chapters in book publications (3)
  1. Targeting mitochondria for cardiovascular disorders: therapeutic potential and obstacles.
    Authors:
    Bonora M, Wieckowski MR, Sinclair DA, Kroemer G, Pinton P, Galluzzi L.
    Academic press:
    Nature Reviews Cardiology (rok: 2019, tom: 16(1), strony: 33-55), Wydawca: Springer Nature
    Status:
    Published
    DOI:
    10.1038/s41569-018-0074-0 - link to the publication
  2. Mitochondrial permeability transition involves dissociation of F1FO ATP synthase dimers and C-ring conformation.
    Authors:
    Bonora M, Morganti C, Morciano G, Pedriali G, Lebiedzinska-Arciszewska M, Aquila G, Giorgi C, Rizzo P, Campo G, Ferrari R, Kroemer G, Wieckowski MR, Galluzzi L, Pinton P
    Academic press:
    EMBO Reports (rok: 2017, tom: 18(7), strony: 1077-1089), Wydawca: EMBOpress
    Status:
    Published
    DOI:
    10.15252/embr.201643602 - link to the publication
  3. NDUFS4 deletion triggers loss of NDUFA12 in Ndufs4-/- mice and Leigh syndrome patients: A stabilizing role for NDUFAF2.
    Authors:
    Adjobo-Hermans MJW, de Haas R, Willems PHGM, Wojtala A, van Emst-de Vries SE, Wagenaars JA, van den Brand M, Rodenburg RJ, Smeitink JAM, Nijtmans LG, Sazanov LA, Wieckowski MR $, Koopman WJH. $ ($ co-corresponding authors)
    Academic press:
    Biochim Biophys Acta - Bioenergetics (rok: 2020, tom: 1861, strony: 45668), Wydawca: Elsevier
    Status:
    Published
    DOI:
    10.1016/j.bbabio.2020.148213 - link to the publication
  4. Comprehensive analysis of mitochondrial permeability transition pore activity in living cells using fluorescence-imaging-based techniques.
    Authors:
    Bonora M, Morganti C, Morciano G, Giorgi C, Wieckowski MR, Pinton P
    Academic press:
    Nature Protocols (rok: 2016, tom: 11 (6), strony: 1067-1080), Wydawca: Macmillan Publishers Limited, part of Springer Nature
    Status:
    Published
    DOI:
    10.1038/nprot.2016.064 - link to the publication
  5. Modulation of mitochondrial dysfunction-related oxidative stress in fibroblasts of patients with Leigh syndrome by inhibition of prooxidative p66Shc pathway.
    Authors:
    Wojtala A, Karkucinska-Wieckowska A, Sardao VA, Szczepanowska J, Kowalski P, Pronicki M, Duszynski J, Wieckowski MR.
    Academic press:
    Mitochondrion (rok: 2017, tom: 37, strony: 62-79), Wydawca: Elsevier
    Status:
    Published
    DOI:
    10.1016/j.mito.2017.07.002 - link to the publication
  6. Mitochondrial alterations accompanied by oxidative stress conditions in skin fibroblasts of Huntington's disease patients
    Authors:
    Jędrak P, Mozolewski P, Węgrzyn G, Więckowski MR
    Academic press:
    Metabolic Brain Disease (rok: 2018, tom: 33(6), strony: 2005-2017), Wydawca: Springer
    Status:
    Published
    DOI:
    10.1007/s11011-018-0308-1 - link to the publication
  7. Quantifying ROS levels using CM-H2DCFDA and HyPer
    Authors:
    Monika Oparka, Jarosław Walczak, Dominika Malinska, Lisanne M.P.E. van Oppen, Joanna Szczepanowska, Werner J.H. Koopman and Mariusz R. Wieckowski
    Academic press:
    Methods (rok: 2016, tom: 109, strony: 45727), Wydawca: ELSEVIER
    Status:
    Published
    DOI:
    10.1016/j.ymeth.2016.06.008 - link to the publication
  8. Dietary Polyphenols and Mitochondrial Function: Role in Health and Disease.
    Authors:
    Teixeira J, Chavarria D, Borges F, Wojtczak L, Wieckowski MR, Karkucińska-Wieckowska A, Oliveira PJ
    Academic press:
    Current Medicinal Chemistry (rok: 2019, tom: 26(19), strony: 3376-3406), Wydawca: Bentham Science Publishers
    Status:
    Published
    DOI:
    10.2174/0929867324666170529101810 - link to the publication
  9. Mitochondrial Reactive Oxigen Species and inflammation: molecular mechanisms, diseases and promising therapies.
    Authors:
    Alessandro Rimessi, Maurizio Previati, Federica Nigro, Mariusz R. Wieckowski and Paolo Pinton
    Academic press:
    The International Journal of Biochemistry & Cell Biology (rok: 2016, tom: 81, strony: 281-293), Wydawca: ELSEVIER
    Status:
    Published
    DOI:
    10.1016/j.biocel.2016.06.015 - link to the publication
  10. Interaction of Mitochondria with the Endoplasmic Reticulum and Plasma Membrane in Calcium Homeostasis, Lipid Trafficking and Mitochondrial Structure.
    Authors:
    Jędrzej Szymański, Justyna Janikiewicz, Bernadeta Michalska, Paulina Patalas-Krawczyk, Mariasole Perrone, Wiesław Ziółkowski, Jerzy Duszynski, Paolo Pinton, Agnieszka Dobrzyń and Mariusz R. Wieckowski
    Academic press:
    International Journal of Molecular Sciences (rok: 2017, tom: 18(7), strony: 45713), Wydawca: MDPI
    Status:
    Published
    DOI:
    10.3390/ijms18071576 - link to the publication
  11. A naturally occurring mutation in ATP synthase subunit c is associated with increased damage following hypoxia/reoxygenation in STEMI patients
    Authors:
    Giampaolo Morciano, Gaia Pedriali, Massimo Bonora, Rita Pavasini, Elisa Mikus, Simone Calvi, Matteo Bovolenta, Magdalena Lebiedzinska-Arciszewska, Mirko Pinotti, Alberto Albertini, Mariusz R Wieckowski, Carlotta Giorgi, Roberto Ferrari, Lorenzo Galluzzi, Gianluca Campo, Paolo Pinton
    Academic press:
    Cell Reports (rok: 2021, tom: 35(2), strony: 45679), Wydawca: Cell Press
    Status:
    Published
    DOI:
    10.1016/j.celrep.2021.108983 - link to the publication
  1. Recovering mitochondrial function in patients' fibroblasts
    Authors:
    Mariusz R. Wieckowski, Alberto Danese, Giampaolo Morciano, Maciej Pronicki, Jerzy Duszynski, Paolo Pinton and Agnieszka Karkucinska-Wieckowska
    Book:
    Mitochondrial Biology and Experimental Therapeutics (rok: 2018, tom: brak informacji, strony: 359-378), Wydawca: Springer International Publishing
    Status:
    Published
  2. Mitochondria and Reactive Oxygen Species in Aging and Age-Related Diseases
    Authors:
    Giorgi C, Marchi S, Simoes ICM, Ren Z, Morciano G, Perrone M, Patalas-Krawczyk P, Borchard S, Jędrak P, Pierzynowska K, Szymański J, Wang DQ, Portincasa P, Węgrzyn G, Zischka H, Dobrzyn P, Bonora M, Duszynski J, Rimessi A, Karkucinska-Wieckowska A, Dobrzyn A, Szabadkai G, Zavan B, Oliveira PJ, Sardao VA, Pinton P, Wieckowski MR.
    Book:
    International Review of Cell and Molecular Biology (rok: 2018, tom: 340, strony: 209-344), Wydawca: ELSEVIER
    Status:
    Published
  3. Mitochondria in Multiple Sclerosis: Molecular Mechanisms of Pathogenesis
    Authors:
    S.Patergnani, V.Fossati, M.Bonora, Giorgi, Marchi, Missiroli, T.Rusielewicz, M.R.Wieckowski, P.Pinton
    Book:
    International Review of Cell and Molecular Biology (rok: 2017, tom: 328, strony: 49-103), Wydawca: Elsevier
    Status:
    Published

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