Search Results - "Rustin, Pierre"

Mitochondria are physiologically maintained at close to 50 °C by Chrétien, Dominique, Bénit, Paule, Ha, Hyung-Ho, Keipert, Susanne, El-Khoury, Riyad, Chang, Young-Tae, Jastroch, Martin, Jacobs, Howard T, Rustin, Pierre, Rak, Malgorzata

“…In endothermic species, heat released as a product of metabolism ensures stable internal temperature throughout the organism, despite varying environmental…”
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Imbalanced OPA1 processing and mitochondrial fragmentation cause heart failure in mice by Wai, Timothy, García-Prieto, Jaime, Baker, Michael J, Merkwirth, Carsten, Benit, Paule, Rustin, Pierre, Rupérez, Francisco Javier, Barbas, Coral, Ibañez, Borja, Langer, Thomas

“…Mitochondrial morphology is shaped by fusion and division of their membranes. Here, we found that adult myocardial function depends on balanced mitochondrial…”
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Mitochondrial cytochrome c oxidase deficiency by Rak, Malgorzata, Bénit, Paule, Chrétien, Dominique, Bouchereau, Juliette, Schiff, Manuel, El-Khoury, Riyad, Tzagoloff, Alexander, Rustin, Pierre

“…As with other mitochondrial respiratory chain components, marked clinical and genetic heterogeneity is observed in patients with a cytochrome c oxidase…”
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SDH Mutations Establish a Hypermethylator Phenotype in Paraganglioma by Letouzé, Eric, Martinelli, Cosimo, Loriot, Céline, Burnichon, Nelly, Abermil, Nasséra, Ottolenghi, Chris, Janin, Maxime, Menara, Mélanie, Nguyen, An Thach, Benit, Paule, Buffet, Alexandre, Marcaillou, Charles, Bertherat, Jérôme, Amar, Laurence, Rustin, Pierre, De Reyniès, Aurélien, Gimenez-Roqueplo, Anne-Paule, Favier, Judith

“…Paragangliomas are neuroendocrine tumors frequently associated with mutations in RET, NF1, VHL, and succinate dehydrogenase (SDHx) genes. Methylome analysis of…”
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Evolutionarily conserved susceptibility of the mitochondrial respiratory chain to SDHI pesticides and its consequence on the impact of SDHIs on human cultured cells by Bénit, Paule, Kahn, Agathe, Chretien, Dominique, Bortoli, Sylvie, Huc, Laurence, Schiff, Manuel, Gimenez-Roqueplo, Anne-Paule, Favier, Judith, Gressens, Pierre, Rak, Malgorzata, Rustin, Pierre

“…Succinate dehydrogenase (SDH) inhibitors (SDHIs) are used worldwide to limit the proliferation of molds on plants and plant products. However, as SDH, also…”
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Spermidine and resveratrol induce autophagy by distinct pathways converging on the acetylproteome by Morselli, Eugenia, Mariño, Guillermo, Bennetzen, Martin V, Eisenberg, Tobias, Megalou, Evgenia, Schroeder, Sabrina, Cabrera, Sandra, Bénit, Paule, Rustin, Pierre, Criollo, Alfredo, Kepp, Oliver, Galluzzi, Lorenzo, Shen, Shensi, Malik, Shoaib Ahmad, Maiuri, Maria Chiara, Horio, Yoshiyuki, López-Otín, Carlos, Andersen, Jens S, Tavernarakis, Nektarios, Madeo, Frank, Kroemer, Guido

“…Autophagy protects organelles, cells, and organisms against several stress conditions. Induction of autophagy by resveratrol requires the nicotinamide adenine…”
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A critical approach to the therapy of mitochondrial respiratory chain and oxidative phosphorylation diseases by DiMauro, Salvatore, Rustin, Pierre

“…Taking advantage of a series of questions raised by an association of patients with mitochondrial disease, this review, after a brief overview of basic…”
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CHCHD2 accumulates in distressed mitochondria and facilitates oligomerization of CHCHD10 by Huang, Xiaoping, Wu, Beverly P, Nguyen, Diana, Liu, Yi-Ting, Marani, Melika, Hench, Jürgen, Bénit, Paule, Kozjak-Pavlovic, Vera, Rustin, Pierre, Frank, Stephan, Narendra, Derek P

“…Abstract Mutations in paralogous mitochondrial proteins CHCHD2 and CHCHD10 cause autosomal dominant Parkinson Disease (PD) and Amyotrophic Lateral…”
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Defects in succinate dehydrogenase in gastrointestinal stromal tumors lacking KIT and PDGFRA mutations by Janeway, Katherine A, Kim, Su Young, Lodish, Maya, Nosé, Vânia, Rustin, Pierre, Gaal, José, Dahia, Patricia L.M, Liegl, Bernadette, Ball, Evan R, Raygada, Margarita, Lai, Angela H, Kelly, Lorna, Hornick, Jason L, O'Sullivan, Maureen, de Krijger, Ronald R, Dinjens, Winand N.M, Demetri, George D, Antonescu, Cristina R, Fletcher, Jonathan A, Helman, Lee, Stratakis, Constantine A

“…Carney-Stratakis syndrome, an inherited condition predisposing affected individuals to gastrointestinal stromal tumor (GIST) and paraganglioma, is caused by…”
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PGC-1α is dispensable for exercise-induced mitochondrial biogenesis in skeletal muscle by Rowe, Glenn C, El-Khoury, Riyad, Patten, Ian S, Rustin, Pierre, Arany, Zolt

“…Exercise confers numerous health benefits, many of which are thought to stem from exercise-induced mitochondrial biogenesis (EIMB) in skeletal muscle. The…”
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Genetic and biochemical intricacy shapes mitochondrial cytopathies by Turnbull, Douglass M, Rustin, Pierre

“…Abstract The major progress made in the identification of the molecular bases of mitochondrial disease has revealed the huge diversity of their origin. Today…”
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Acute and chronic mitochondrial respiratory chain deficiency differentially regulate lysosomal biogenesis by Fernández-Mosquera, Lorena, Diogo, Cátia V., Yambire, King Faisal, Santos, Gabriela L., Luna Sánchez, Marta, Bénit, Paule, Rustin, Pierre, Lopez, Luis Carlos, Milosevic, Ira, Raimundo, Nuno

“…Mitochondria are key cellular signaling platforms, affecting fundamental processes such as cell proliferation, differentiation and death. However, it remains…”
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Interaction between AIF and CHCHD4 Regulates Respiratory Chain Biogenesis by Hangen, Emilie, Féraud, Olivier, Lachkar, Sylvie, Mou, Haiwei, Doti, Nunzianna, Fimia, Gian Maria, Lam, Ngoc-vy, Zhu, Changlian, Godin, Isabelle, Muller, Kevin, Chatzi, Afroditi, Nuebel, Esther, Ciccosanti, Fabiola, Flamant, Stéphane, Bénit, Paule, Perfettini, Jean-Luc, Sauvat, Allan, Bennaceur-Griscelli, Annelise, Ser-Le Roux, Karine, Gonin, Patrick, Tokatlidis, Kostas, Rustin, Pierre, Piacentini, Mauro, Ruvo, Menotti, Blomgren, Klas, Kroemer, Guido, Modjtahedi, Nazanine

“…Apoptosis-inducing factor (AIF) is a mitochondrial flavoprotein that, beyond its apoptotic function, is required for the normal expression of major respiratory…”
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Respiratory-chain diseases related to complex III deficiency by Bénit, Paule, Lebon, Sophie, Rustin, Pierre

“…Complex III deficiencies are among the least common respiratory-chain abnormalities identified to date in humans. Nevertheless, their unexplained tissue…”
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SLC25A32 Mutations and Riboflavin-Responsive Exercise Intolerance by Schiff, Manuel, Veauville-Merllié, Alice, Su, Chen Hsien, Tzagoloff, Alexander, Rak, Malgorzata, Ogier de Baulny, Hélène, Boutron, Audrey, Smedts-Walters, Hélène, Romero, Norma B, Rigal, Odile, Rustin, Pierre, Vianey-Saban, Christine, Acquaviva-Bourdain, Cécile

“…A patient with late-onset exercise intolerance had haploinsufficiency of SLC25A32, which encodes the human mitochondrial flavin adenine dinucleotide…”
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Author Correction: miR-379 links glucocorticoid treatment with mitochondrial response in Duchenne muscular dystrophy by Sanson, Mathilde, Vu Hong, Ai, Massourides, Emmanuelle, Bourg, Nathalie, Suel, Laurence, Amor, Fatima, Corre, Guillaume, Bénit, Paule, Barthelemy, Inès, Blot, Stephane, Bigot, Anne, Pinset, Christian, Rustin, Pierre, Servais, Laurent, Voit, Thomas, Richard, Isabelle, Israeli, David

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Alternative oxidase expression in the mouse enables bypassing cytochrome c oxidase blockade and limits mitochondrial ROS overproduction by El-Khoury, Riyad, Dufour, Eric, Rak, Malgorzata, Ramanantsoa, Nelina, Grandchamp, Nicolas, Csaba, Zsolt, Duvillié, Bertrand, Bénit, Paule, Gallego, Jorge, Gressens, Pierre, Sarkis, Chamsy, Jacobs, Howard T, Rustin, Pierre

“…Cyanide-resistant non-phosphorylating respiration is known in mitochondria from plants, fungi, and microorganisms but is absent in mammals. It results from the…”
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DAPIT Over-Expression Modulates Glucose Metabolism and Cell Behaviour in HEK293T Cells by Kontro, Heidi, Cannino, Giuseppe, Rustin, Pierre, Dufour, Eric, Kainulainen, Heikki

“…Diabetes Associated Protein in Insulin-sensitive Tissues (DAPIT) is a subunit of mitochondrial ATP synthase and has also been found to associate with the…”
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Mitochondrial temperature homeostasis resists external metabolic stresses by Terzioglu, Mügen, Veeroja, Kristo, Montonen, Toni, Ihalainen, Teemu O, Salminen, Tiina S, Bénit, Paule, Rustin, Pierre, Chang, Young-Tae, Nagai, Takeharu, Jacobs, Howard T

“…Based on studies with a fluorescent reporter dye, Mito Thermo Yellow (MTY), and the genetically encoded gTEMP ratiometric fluorescent temperature indicator…”
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Impaired nuclear Nrf2 translocation undermines the oxidative stress response in Friedreich ataxia by Paupe, Vincent, Dassa, Emmanuel P, Goncalves, Sergio, Auchère, Françoise, Lönn, Maria, Holmgren, Arne, Rustin, Pierre

“…Friedreich ataxia originates from a decrease in mitochondrial frataxin, which causes the death of a subset of neurons. The biochemical hallmarks of the disease…”
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