Analysis of MT-ATP8 gene variants reported in patients by modeling in silico and in yeast model organism

por Panja, Chiranjit, Niedzwiecka, Katarzyna, Baranowska, Emilia, Poznanski, Jaroslaw, Kucharczyk, Roza
Publicado 2023

Ancestral State Reconstruction of the Apoptosis Machinery in the Common Ancestor of Eukaryotes

por Klim, Joanna, Gładki, Arkadiusz, Kucharczyk, Roza, Zielenkiewicz, Urszula, Kaczanowski, Szymon
Publicado 2018

ATP synthase interactome analysis identifies a new subunit l as a modulator of permeability transition pore in yeast

por Panja, Chiranjit, Wiesyk, Aneta, Niedźwiecka, Katarzyna, Baranowska, Emilia, Kucharczyk, Roza
Publicado 2023

5,6-diiodo-1H-benzotriazole: new TBBt analogue that minutely affects mitochondrial activity

por Paprocki, Daniel, Winiewska-Szajewska, Maria, Speina, Elżbieta, Kucharczyk, Róża, Poznański, Jarosław
Publicado 2021

ATP Synthase Diseases of Mitochondrial Genetic Origin

por Dautant, Alain, Meier, Thomas, Hahn, Alexander, Tribouillard-Tanvier, Déborah, di Rago, Jean-Paul, Kucharczyk, Roza
Publicado 2018

Revisiting Mitochondrial pH with an Improved Algorithm for Calibration of the Ratiometric 5(6)-carboxy-SNARF-1 Probe Reveals Anticooperative Reaction with H(+) Ions and Warrants Fu...

por Żurawik, Tomasz Michał, Pomorski, Adam, Belczyk-Ciesielska, Agnieszka, Goch, Grażyna, Niedźwiedzka, Katarzyna, Kucharczyk, Róża, Krężel, Artur, Bal, Wojciech
Publicado 2016

Molecular basis of diseases induced by the mitochondrial DNA mutation m.9032T>C

por Baranowska, Emilia, Niedzwiecka, Katarzyna, Panja, Chiranjit, Charles, Camille, Dautant, Alain, di Rago, Jean-Paul, Tribouillard-Tanvier, Déborah, Kucharczyk, Roza
Publicado 2022

Molecular Basis of the Pathogenic Mechanism Induced by the m.9191T>C Mutation in Mitochondrial ATP6 Gene

por Su, Xin, Dautant, Alain, Godard, François, Bouhier, Marine, Zoladek, Teresa, Kucharczyk, Roza, di Rago, Jean-Paul, Tribouillard-Tanvier, Déborah
Publicado 2020

Probing the pathogenicity of patient-derived variants of MT-ATP6 in yeast

por Baranowska, Emilia, Niedzwiecka, Katarzyna, Panja, Chiranjit, Charles, Camille, Dautant, Alain, Poznanski, Jarosław, di Rago, Jean-Paul, Tribouillard-Tanvier, Déborah, Kucharczyk, Roza
Publicado 2023

The Suppressor of AAC2 Lethality SAL1 Modulates Sensitivity of Heterologously Expressed Artemia ADP/ATP Carrier to Bongkrekate in Yeast

por Wysocka-Kapcinska, Monika, Torocsik, Beata, Turiak, Lilla, Tsaprailis, George, David, Cynthia L., Hunt, Andrea M., Vekey, Karoly, Adam-Vizi, Vera, Kucharczyk, Roza, Chinopoulos, Christos
Publicado 2013

Arf1 coordinates fatty acid metabolism and mitochondrial homeostasis

por Enkler, Ludovic, Szentgyörgyi, Viktoria, Pennauer, Mirjam, Prescianotto-Baschong, Cristina, Riezman, Isabelle, Wiesyk, Aneta, Avraham, Reut Ester, Spiess, Martin, Zalckvar, Einat, Kucharczyk, Roza, Riezman, Howard, Spang, Anne
Publicado 2023

Perturbation of the yeast mitochondrial lipidome and associated membrane proteins following heterologous expression of Artemia-ANT

por Chen, Emily, Kiebish, Michael A., McDaniel, Justice, Niedzwiecka, Katarzyna, Kucharczyk, Roza, Ravasz, Dora, Gao, Fei, Narain, Niven R., Sarangarajan, Rangaprasad, Seyfried, Thomas N., Adam-Vizi, Vera, Chinopoulos, Christos
Publicado 2018

Decreasing cytosolic translation is beneficial to yeast and human Tafazzin-deficient cells

por de Taffin de Tilques, Maxence, Lasserre, Jean-Paul, Godard, François, Sardin, Elodie, Bouhier, Marine, Le Guedard, Marina, Kucharczyk, Roza, Petit, Patrice X., Testet, Eric, di Rago, Jean-Paul, Tribouillard-Tanvier, Déborah
Publicado 2018

Identification of G8969>A in mitochondrial ATP6 gene that severely compromises ATP synthase function in a patient with IgA nephropathy

por Wen, Shuzhen, Niedzwiecka, Katarzyna, Zhao, Weiwei, Xu, Shutian, Liang, Shaoshan, Zhu, Xiaodong, Xie, Honglang, Tribouillard-Tanvier, Déborah, Giraud, Marie-France, Zeng, Caihong, Dautant, Alain, Kucharczyk, Róża, Liu, Zhihong, di Rago, Jean-Paul, Chen, Huimei
Publicado 2016

Assigning mitochondrial localization of dual localized proteins using a yeast Bi-Genomic Mitochondrial-Split-GFP

por Bader, Gaétan, Enkler, Ludovic, Araiso, Yuhei, Hemmerle, Marine, Binko, Krystyna, Baranowska, Emilia, De Craene, Johan-Owen, Ruer-Laventie, Julie, Pieters, Jean, Tribouillard-Tanvier, Déborah, Senger, Bruno, di Rago, Jean-Paul, Friant, Sylvie, Kucharczyk, Roza, Becker, Hubert Dominique
Publicado 2020

Assembly-dependent translation of subunits 6 (Atp6) and 9 (Atp9) of ATP synthase in yeast mitochondria

por Kabala, Anna M, Binko, Krystyna, Godard, François, Charles, Camille, Dautant, Alain, Baranowska, Emilia, Skoczen, Natalia, Gombeau, Kewin, Bouhier, Marine, Becker, Hubert D, Ackerman, Sharon H, Steinmetz, Lars M, Tribouillard-Tanvier, Déborah, Kucharczyk, Roza, di Rago, Jean-Paul
Publicado 2022

Case Report: Identification of a Novel Variant (m.8909T>C) of Human Mitochondrial ATP6 Gene and Its Functional Consequences on Yeast ATP Synthase

por Ding, Qiuju, Kucharczyk, Róża, Zhao, Weiwei, Dautant, Alain, Xu, Shutian, Niedzwiecka, Katarzyna, Su, Xin, Giraud, Marie-France, Gombeau, Kewin, Zhang, Mingchao, Xie, Honglang, Zeng, Caihong, Bouhier, Marine, di Rago, Jean-Paul, Liu, Zhihong, Tribouillard-Tanvier, Déborah, Chen, Huimei
Publicado 2020

Yeast as a system for modeling mitochondrial disease mechanisms and discovering therapies

por Lasserre, Jean-Paul, Dautant, Alain, Aiyar, Raeka S., Kucharczyk, Roza, Glatigny, Annie, Tribouillard-Tanvier, Déborah, Rytka, Joanna, Blondel, Marc, Skoczen, Natalia, Reynier, Pascal, Pitayu, Laras, Rötig, Agnès, Delahodde, Agnès, Steinmetz, Lars M., Dujardin, Geneviève, Procaccio, Vincent, di Rago, Jean-Paul
Publicado 2015

Experimental Relocation of the Mitochondrial ATP9 Gene to the Nucleus Reveals Forces Underlying Mitochondrial Genome Evolution

por Bietenhader, Maïlis, Martos, Alexandre, Tetaud, Emmanuel, Aiyar, Raeka S., Sellem, Carole H., Kucharczyk, Roza, Clauder-Münster, Sandra, Giraud, Marie-France, Godard, François, Salin, Bénédicte, Sagot, Isabelle, Gagneur, Julien, Déquard-Chablat, Michelle, Contamine, Véronique, Denmat, Sylvie Hermann-Le, Sainsard-Chanet, Annie, Steinmetz, Lars M., di Rago, Jean-Paul
Publicado 2012

Mitochondrial protein sorting as a therapeutic target for ATP synthase disorders

por Aiyar, Raeka S., Bohnert, Maria, Duvezin-Caubet, Stéphane, Voisset, Cécile, Gagneur, Julien, Fritsch, Emilie S., Couplan, Elodie, von der Malsburg, Karina, Funaya, Charlotta, Soubigou, Flavie, Courtin, Florence, Suresh, Sundari, Kucharczyk, Roza, Evrard, Justine, Antony, Claude, St.Onge, Robert P., Blondel, Marc, di Rago, Jean-Paul, van der Laan, Martin, Steinmetz, Lars M.
Publicado 2014