Friedreich's Ataxia, Frataxin, PIP5K1B: Echo of a Distant Fracas

by Aurélien Bayot, Pierre Rustin
Published 2013-01-01

Genetic and biochemical intricacy shapes mitochondrial cytopathies

by Douglass M. Turnbull, Pierre Rustin
Published 2016-08-01

Correction: DAPIT Over-Expression Modulates Glucose Metabolism and Cell Behaviour in HEK293T Cells.

by Heidi Kontro, Giuseppe Cannino, Pierre Rustin, Eric Dufour, Heikki Kainulainen
Published 2015-01-01

DAPIT Over-Expression Modulates Glucose Metabolism and Cell Behaviour in HEK293T Cells.

by Heidi Kontro, Giuseppe Cannino, Pierre Rustin, Eric Dufour, Heikki Kainulainen
Published 2015-01-01

Correction: DAPIT Over-Expression Modulates Glucose Metabolism and Cell Behaviour in HEK293T Cells.

by Heidi Kontro, Giuseppe Cannino, Pierre Rustin, Eric Dufour, Heikki Kainulainen
Published 2015-01-01

The variability of the harlequin mouse phenotype resembles that of human mitochondrial-complex I-deficiency syndromes.

by Paule Bénit, Sergio Goncalves, Emmanuel Philippe Dassa, Jean-Jacques Brière, Pierre Rustin
Published 2008-01-01

An Effective, Versatile, and Inexpensive Device for Oxygen Uptake Measurement

by Paule Bénit, Dominique Chrétien, Mathieu Porceddu, Constantin Yanicostas, Malgorzata Rak, Pierre Rustin
Published 2017-06-01

PGC-1α is dispensable for exercise-induced mitochondrial biogenesis in skeletal muscle.

by Glenn C Rowe, Riyad El-Khoury, Ian S Patten, Pierre Rustin, Zolt Arany
Published 2012-01-01

Mouse studies to shape clinical trials for mitochondrial diseases: high fat diet in Harlequin mice.

by Manuel Schiff, Paule Bénit, Riyad El-Khoury, Dimitri Schlemmer, Jean-François Benoist, Pierre Rustin
Published 2011-01-01

Impaired nuclear Nrf2 translocation undermines the oxidative stress response in Friedreich ataxia.

by Vincent Paupe, Emmanuel P Dassa, Sergio Goncalves, Françoise Auchère, Maria Lönn, Arne Holmgren, Pierre Rustin
Published 2009-01-01

ANT2-defective fibroblasts exhibit normal mitochondrial bioenergetics

by Dolly Prabhu, Amy C. Goldstein, Riyad El-Khoury, Malgorzata Rak, Lia Edmunds, Pierre Rustin, Jerry Vockley, Manuel Schiff
Published 2015-06-01

Paradoxical Inhibition of Glycolysis by Pioglitazone Opposes the Mitochondriopathy Caused by AIF Deficiency

by Paule Bénit, Alice Pelhaître, Elise Saunier, Sylvie Bortoli, Assetou Coulibaly, Malgorzata Rak, Manuel Schiff, Guido Kroemer, Massimo Zeviani, Pierre Rustin
Published 2017-03-01

Use of human cancer cell lines mitochondria to explore the mechanisms of BH3 peptides and ABT-737-induced mitochondrial membrane permeabilization.

by Nelly Buron, Mathieu Porceddu, Magali Brabant, Diana Desgué, Cindy Racoeur, Myriam Lassalle, Christine Péchoux, Pierre Rustin, Etienne Jacotot, Annie Borgne-Sanchez
Published 2010-03-01

Mitochondria are physiologically maintained at close to 50 °C.

by Dominique Chrétien, Paule Bénit, Hyung-Ho Ha, Susanne Keipert, Riyad El-Khoury, Young-Tae Chang, Martin Jastroch, Howard T Jacobs, Pierre Rustin, Malgorzata Rak
Published 2018-01-01

Induced pluripotent stem cells-derived neurons from patients with Friedreich ataxia exhibit differential sensitivity to resveratrol and nicotinamide

by Pauline Georges, Maria-Gabriela Boza-Moran, Jacqueline Gide, Georges Arielle Pêche, Benjamin Forêt, Aurélien Bayot, Pierre Rustin, Marc Peschanski, Cécile Martinat, Laetitia Aubry
Published 2019-10-01

Pitfalls in Monitoring Mitochondrial Temperature Using Charged Thermosensitive Fluorophores

by Dominique Chrétien, Paule Bénit, Christine Leroy, Riyad El-Khoury, Sunyou Park, Jung Yeol Lee, Young-Tae Chang, Guy Lenaers, Pierre Rustin, Malgorzata Rak
Published 2020-12-01

Translocator Protein-Mediated Stabilization of Mitochondrial Architecture during Inflammation Stress in Colonic Cells.

by Leeyah Issop, Mariano A Ostuni, Sunghoon Lee, Mireille Laforge, Gabriel Péranzi, Pierre Rustin, Jean-François Benoist, Jérome Estaquier, Vassilios Papadopoulos, Jean-Jacques Lacapère
Published 2016-01-01

Evolutionarily conserved susceptibility of the mitochondrial respiratory chain to SDHI pesticides and its consequence on the impact of SDHIs on human cultured cells.

by Paule Bénit, Agathe Kahn, Dominique Chretien, Sylvie Bortoli, Laurence Huc, Manuel Schiff, Anne-Paule Gimenez-Roqueplo, Judith Favier, Pierre Gressens, Malgorzata Rak, Pierre Rustin
Published 2019-01-01

Alternative oxidase expression in the mouse enables bypassing cytochrome c oxidase blockade and limits mitochondrial ROS overproduction.

by Riyad El-Khoury, Eric Dufour, Malgorzata Rak, Nelina Ramanantsoa, Nicolas Grandchamp, Zsolt Csaba, Bertrand Duvillié, Paule Bénit, Jorge Gallego, Pierre Gressens, Chamsy Sarkis, Howard T Jacobs, Pierre Rustin
Published 2013-01-01

Arabidopsis thaliana alternative dehydrogenases: a potential therapy for mitochondrial complex I deficiency? Perspectives and pitfalls

by Alessia Catania, Arcangela Iuso, Juliette Bouchereau, Laura S. Kremer, Marina Paviolo, Caterina Terrile, Paule Bénit, Allan G. Rasmusson, Thomas Schwarzmayr, Valeria Tiranti, Pierre Rustin, Malgorzata Rak, Holger Prokisch, Manuel Schiff
Published 2019-10-01