Projects funded by the NCN

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: Biochemistry
  • NZ1_1: Molecular biology

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 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 (14)
  • Book publications / chapters in book publications (3)
  1. Discovery of novel 1,3,8-triazaspiro[4.5]decane derivatives that target the c subunit of F1/FO-adenosine triphosphate (ATP) synthase for the treatment of reperfusion damage in myocardial infarction
    Authors:
    Morciano G, Preti D, Pedriali G, Aquila G, Missiroli S, Fantinati A, Caroccia N, Pacifico S, Bonora M, Talarico A, Morganti C, Rizzo P, Ferrari R, Wieckowski MR, Campo G, Giorgi C, Trapella C, Pinton P.
    Academic press:
    Journal of Medicinal Chemistry (rok: 2018, tom: 61(16), strony: 7131-7143.), Wydawca: ACS
    Status:
    Published
    DOI:
    10.1021/acs.jmedchem.8b00278 - link to the publication
  2. 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
  3. 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
  4. 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
  5. 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
  6. 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
  7. 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
  8. 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: 45302), Wydawca: Elsevier
    Status:
    Published
    DOI:
    10.1016/j.bbabio.2020.148213 - link to the publication
  9. 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: 45362), Wydawca: ELSEVIER
    Status:
    Published
    DOI:
    10.1016/j.ymeth.2016.06.008 - link to the publication
  10. 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: 45313), Wydawca: Cell Press
    Status:
    Published
    DOI:
    10.1016/j.celrep.2021.108983 - link to the publication
  11. 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: 45347), Wydawca: MDPI
    Status:
    Published
    DOI:
    10.3390/ijms18071576 - link to the publication
  12. Swim Training Modulates Skeletal Muscle Energy Metabolism, Oxidative Stress, and Mitochondrial Cholesterol Content in Amyotrophic Lateral Sclerosis Mice
    Authors:
    Flis DJ, Dzik K, Kaczor JJ, Halon-Golabek M, Antosiewicz J, Wieckowski MR, Ziolkowski W.
    Academic press:
    Oxidative Medicine and Cellular Longevity (rok: 2018, tom: eCollection, strony: 45303), Wydawca: Hindawi
    Status:
    Published
    DOI:
    10.1155/2018/5940748 - link to the publication
  13. 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
  14. Ndufs4 knockout mouse models of Leigh Syndrome: Pathophysiology and intervention
    Authors:
    Melissa van de Wal, Merel Adjobo-Hermans, Jaap Keijer, Tom Schirris, Judith Homberg, Mariusz R. Wieckowski, Sander Grefte, Evert M. van Schothorst, Clara van Karnebeek, Albert Quintana and Werner J.H. Koopman
    Academic press:
    Brain (rok: 2021, tom: not yet, strony: not yet available), Wydawca: Oxford University Press
    Status:
    Submitted
    DOI:
    not - link to the publication
  1. 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
  2. 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
  3. 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

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