Projects funded by the NCN

Information on the principal investigator and host institution

Information of the project and the call

Keywords

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Understanding of the functioning of mitochondrial ATP synthase by studying in yeast the phenotype of human pathology related mutations in the mitochondrial DNA

2016/23/B/NZ3/02098

Keywords:

mitochondria mitochondrial diseases mitochondrial mutations MT-ATP6 MT-ATP8 ATP synthase

Descriptors:

  • NZ3_1: Cell biology
  • NZ1_2: Biochemistry
  • NZ5_3: Pathogenesis of human diseases

Panel:

NZ3 - Cellular and developmental biology: cell biology, developmental biology, ageing biology, neurobiology

Host institution :

Instytut Biochemii i Biofizyki Polskiej Akademii Nauk

woj. mazowieckie

Other projects carried out by the institution 

Principal investigator (from the host institution):

dr hab. Róża Kucharczyk 

Number of co-investigators in the project: 3

Call: OPUS 12 - announced on 2016-09-15

Amount awarded: 1 061 800 PLN

Project start date (Y-m-d): 2017-07-25

Project end date (Y-m-d): 2022-07-24

Project duration:: 60 months (the same as in the proposal)

Project status: Project settled

Project description

Download the project description in a pdf file

Note - project descriptions were prepared by the authors of the applications themselves and placed in the system in an unchanged form.

Equipment purchased [PL]

  1. zestaw komputerowy z oprogramowaniem (10 000 PLN)
  2. komputer przenośny (4 000 PLN)

Information in the final report

  • Publication in academic press/journals (10)
  • Articles in post-conference publications (2)
  • Book publications / chapters in book publications (1)
  1. Molecular basis of disease caused by the mtDNA mutation m.8969G>A in the subunit a of ATP synthase
    Authors:
    Natalia Skoczeń, Alain Dautant, Krystyna Binko, François Godard, Marine Bouhier, Xin Su, Jean-Paul Lasserre, Marie-France Giraud, Déborah Tribouillard-Tanvier, Huimei Chen, Jean-Paul di Rago, Roza Kucharczyk
    Academic press:
    BBA-Bioenergetics (rok: 2018, tom: 1859, strony: 602-611), Wydawca: Elsevier
    Status:
    Published
    DOI:
    10.1016/j.bbabio.2018.05.009 - link to the publication
  2. Probing the pathogenicity of patient-derived variants of MT-ATP6 in yeast
    Authors:
    Emilia Baranowska, Katarzyna Niedzwiecka, Chiranjit Panja, Camille Charles, Alain Dautant, Jarosław Poznanski, Jean-Paul di Rago, Deborah Tribouillard-Tanvier, Roza Kucharczyk
    Academic press:
    Disease Models and the Mechanisms (rok: 2023, tom: 16(4), strony: dmm049783), Wydawca: The Company of Biologists
    Status:
    Published
    DOI:
    10.1242/dmm.049783 - link to the publication
  3. The pathogenic m.8993T>G mutation in mitochondrial ATP6 gene prevents proton release from the subunit c-ring rotor of ATP synthase
    Authors:
    Xin Su, Alain Dautant, Malgorzata Rak, François Godard, Nahia Ezkurdia, Marine Bouhier, Maïlis Bietenhader, David M. Mueller, Roza Kucharczyk, Jean-Paul di Rago, and Déborah Tribouillard-Tanvier
    Academic press:
    Human Molecular Genetics (rok: 2021, tom: 30, strony: 381-392), Wydawca: Oxford
    Status:
    Published
    DOI:
    10.1093/hmg/ddab043 - link to the publication
  4. Analysis of MT-ATP8 gene variants reported in patients by modeling in silico and in yeast model organism
    Authors:
    Chiranjit Panja, Katarzyna Niedzwiecka, Emilia Baranowska, Jaroslaw Poznanski, Roza Kucharczyk
    Academic press:
    Scientific Report , Wydawca: Nature
    Status:
    Submitted
    DOI:
    brak - link to the publication
  5. ATP synthase subunit a supports permeability transition in yeast lacking dimerization subunits and modulates yPTP conductance
    Authors:
    Katarzyna Niedziewska, Emilia Baranowska,Chiranjit Panja, Roza Kucharczyk
    Academic press:
    Cellular Physiology and Biochemistry (rok: 2020, tom: 54(2), strony: 211-229), Wydawca: Karger
    Status:
    Published
    DOI:
    10.33594/000000215 - link to the publication
  6. Molecular Basis of the Pathogenic Mechanism Induced by the m.9191T>C Mutation in Mitochondrial ATP6 Gene
    Authors:
    Xin Su , Alain Dautant , François Godard , Marine Bouhier , Teresa Zoladek , Roza Kucharczyk , Jean-Paul di Rago, Déborah Tribouillard-Tanvier
    Academic press:
    International Journal of Molecular Sciences (rok: 2020, tom: 21, strony: 5083), Wydawca: MDPI
    Status:
    Published
    DOI:
    10.3390/ijms21145083 - link to the publication
  7. Molekularne podłoże chorób spowodowanych mutacjami w genach kodujących podjednostki syntazy ATP
    Authors:
    Emilia Baranowska, Joanna Rytka, Roza Kucharczyk
    Academic press:
    Postepy biochemii (rok: 2018, tom: 64, strony: 304-317), Wydawca: PTBioch
    Status:
    Published
    DOI:
    10.18388/pb.2018_144 - link to the publication
  8. Molecular basis of diseases induced by the mitochondrial DNA mutation m.9032T>C
    Authors:
    Emilia Baranowska, Katarzyna Niedzwiecka, Chiranjit Panja, Camille Charles, Alain Dautant, Jean-Paul di Rag, Déborah Tribouillard-Tanvier, Roza Kucharczyk
    Academic press:
    Human Molecular Genetics (rok: 2023, tom: 32(8), strony: 1313-1323), Wydawca: Oxford Academic
    Status:
    Published
    DOI:
    10.1093/hmg/ddac292 - link to the publication
  9. Functional investigation of an universally conserved leucine residue in subunit a of ATP synthase targeted by the pathogenic m.9176T>G
    Authors:
    RozaKucharczyk, AlainDautant, FrançoisGodard, DéborahTribouillar-Tanvier, Jean-Pauldi Rago
    Academic press:
    BBA -Bioenergetics (rok: 2019, tom: 1860, strony: 52-59), Wydawca: Elsevier
    Status:
    Published
    DOI:
    10.1016/j.bbabio.2018.11.005 - link to the publication
  10. The pathogenic MT-ATP6 m.8851T>C mutation prevents proton movements within the n-side hydrophilic cleft of the membrane domain of ATP synthase
    Authors:
    Kucharczyk R, Dautant A, Gombeau K, Godard F, Tribouillard- Tanvier D, di Rago JP.
    Academic press:
    BBA-BIO (rok: 2019, tom: 1860, strony: 562-572), Wydawca: Elsevier
    Status:
    Published
    DOI:
    10.1016/jbbabio.2019.06.002 - link to the publication
  1. A disease-causing hydrogen bond in ATP synthase of mitochondrial genetic origin
    Authors:
    Roza Kucharczyk, Natalia Skoczeń, Alain Dautant, Marine Bouhier, Deborah Tribouillard-Tanvier, Jean-Paul di Rago
    Conference:
    European Bioenergetic Conference (rok: 2018, ), Wydawca: Elsevier-BBA
    Data:
    konferencja 25-30.08.
    Status:
    Published
  2. Yeast as model to study the pathogenic mechanisms of mutations in mitochondrial MT-ATP6 gene
    Authors:
    Roza Kucharczyka, Emilia Baranowskaa, Katarzyna Niedzwieckaa, Chiranjit Panjaa, Alain Dautantb, Jarosław Poznanskia, Jean-Paul di Ragob, Deborah Tribouillard-Tanvierb
    Conference:
    European Bioenergetic Conference 2022 (rok: 2022, ), Wydawca: Elsevier
    Data:
    konferencja 20-25-08.
    Status:
    Published
  1. Creation of Yeast Models for Evaluating the Pathogenicity of Mutations in the Human Mitochondrial Gene MT-ATP6 and Discovering Therapeutic Molecules
    Authors:
    Tribouillard-Tanvier De borah, Dautant Alain, Godard Franc ois, Panja Chiranjit, di Rago Jean-Paul, and Kucharczyk Roza
    Book:
    Methods in Molecular Biology (rok: 2022, tom: 2497, strony: 221-242), Wydawca: Springer Science +business Media
    Status:
    Published

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