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收藏分享 收藏 Sengers syndrome caused by biallelic TIMM29 variants and RNAi silencing in Drosophila orthologue recapitulates the human phenotype
Shalata, Adel; Saada, Ann; Mahroum, Mohammed; Hadid, Yarin; Furman, Chaya; Shalata, Zaher Eldin; Desnick, Robert J.; Lorber, Avraham; Khoury, Asaad; Higazi, Adnan; Shaag, Avraham; Barash, Varda; Spiegel, Ronen; Vlodavsky, Euvgeni; Rustin, Pierre; Pietrokovski, Shmuel; Manov, Irena; Gieger, Dan; Tal, Galit; Salzberg, Adi; Mandel, Hanna 分享 收藏 分享 收藏 miR-379 links glucocorticoid treatment with mitochondrial response in Duchenne muscular dystrophy (vol 10, 9139, 2020)Sanson, Mathilde; Vu Hong, Ai; Massourides, Emmanuelle; Bourg, Nathalie; Suel, Laurence; Amor, Fatima; Corre, Guillaume; Benit, Paule; Barthelemy, Ines; Blot, Stephane; Bigot, Anne; Pinset, Christian; Rustin, Pierre; Servais, Laurent; Voit, Thomas; Richard, Isabelle; Israeli, David 分享 收藏 A homoeostatic switch causing glycerol-3-phosphate and phosphoethanolamine accumulation triggers senescence by rewiring lipid metabolism引起甘油-3-磷酸和磷酸乙醇胺积累的同型平衡开关通过重新连接脂质代谢触发衰老Tighanimine, Khaled; Freitas, Jose Americo Nabuco Leva Ferreira; Nemazanyy, Ivan; Bankole, Alexia; Benarroch-Popivker, Delphine; Brodesser, Susanne; Dore, Gregory; Robinson, Lucas; Benit, Paule; Ladraa, Sophia; Saada, Yara Bou; Friguet, Bertrand; Bertolino, Philippe; Bernard, David; Canaud, Guillaume; Rustin, Pierre; Gilson, Eric; Bischof, Oliver; Fumagalli, Stefano; Pende, Mario 分享 收藏 Mitochondrial temperature homeostasis resists external metabolic stressesTerzioglu, Muegen; Veeroja, Kristo; Montonen, Toni; Ihalainen, Teemu O.; Salminen, Tiina S.; Benit, Paule; Rustin, Pierre; Chang, Young-Tae; Nagai, Takeharu; Jacobs, Howard T. 分享 收藏 Muscle-specific loss of apoptosis-inducing factor leads to mitochondrial dysfunction, skeletal muscle atrophy, and dilated cardiomyopathyJoza, N; Oudit, GY; Brown, D; Bénit, P; Kassiri, Z; Vahsen, N; Benoit, L; Patel, MM; Nowikovsky, K; Vassault, A; Backx, PH; Wada, T; Kroerner, G; Rustin, P; Penninger, JA 分享 收藏 Cells Lacking the Fumarase Tumor Suppressor Are Protected from Apoptosis through a Hypoxia-Inducible Factor-Independent, AMPK-Dependent MechanismBardella, Chiara; Olivero, Martina; Lorenzato, Annalisa; Geuna, Massimo; Adam, Julie; O'Flaherty, Linda; Rustin, Pierre; Tomlinson, Ian; Pollard, Patrick J.; Di Renzo, Maria Flavia 分享 收藏 Relevance of the TRIAP1/p53 axis in colon cancer cell proliferation and adaptation to glutamine deprivationNedara, Kenza; Reinhardt, Camille; Lebraud, Emilie; Arena, Giuseppe; Gracia, Celine; Buard, Valerie; Pioche-Durieu, Catherine; Castelli, Florence; Colsch, Benoit; Benit, Paule; Rustin, Pierre; Albaud, Benoit; Gestraud, Pierre; Baulande, Sylvain; Servant, Nicolas; Deutsch, Eric; Verbavatz, Jean-Marc; Brenner, Catherine; Milliat, Fabien; Modjtahedi, Nazanine 分享 收藏 分享 收藏 Succinate Dehydrogenase, Succinate, and Superoxides: A Genetic, Epigenetic, Metabolic, Environmental Explosive CrossroadBenit, Paule; Goncalves, Judith; El Khoury, Riyad; Rak, Malgorzata; Favier, Judith; Gimenez-Roqueplo, Anne-Paule; Rustin, Pierre 分享 收藏 Actinomycin D Targets NPM1c-Primed Mitochondria to Restore PML-Driven Senescence in AML Therapy放线菌素d靶向NPM1c-Primed线粒体以恢复AML治疗中PML驱动的衰老Wu, Hsin-Chieh; Rerolle, Domitille; Berthier, Caroline; Hleihel, Rita; Sakamoto, Takashi; Quentin, Samuel; Benhenda, Shirine; Morganti, Claudia; Wu, Chengchen; Conte, Lidio; Rimsky, Sylvie; Sebert, Marie; Clappier, Emmanuelle; Souquere, Sylvie; Gachet, Stephanie; Soulier, Jean; Durand, Sylvere; Trowbridge, Jennifer J.; Benit, Paule; Rustin, Pierre; El Hajj, Hiba; Raffoux, Emmanuel; Ades, Lionel; Itzykson, Raphael; Dombret, Herve; Fenaux, Pierre; Espeli, Olivier; Kroemer, Guido; Brunetti, Lorenzo; Mak, Tak W.; Lallemand-Breitenbach, Valerie; Bazarbachi, Ali; Falini, Brunangelo; Ito, Keisuke; Martelli, Maria Paola; de The, Hugues 分享 收藏 Pitfalls in Monitoring Mitochondrial Temperature Using Charged Thermosensitive FluorophoresChretien, Dominique; Benit, Paule; Leroy, Christine; El-Khoury, Riyad; Park, Sunyou; Lee, Jung Yeol; Chang, Young-Tae; Lenaers, Guy; Rustin, Pierre; Rak, Malgorzata 分享 收藏 miR-379 links glucocorticoid treatment with mitochondrial response in Duchenne muscular dystrophySanson, M.; Vu Hong, A.; Massourides, E.; Bourg, N.; Suel, L.; Amor, F.; Corre, G.; Benit, P.; Barthelemy, I; Blot, S.; Bigot, A.; Pinset, C.; Rustin, P.; Servais, L.; Voit, T.; Richard, I; Israeli, D. 分享 收藏 Cell Metabolic Alterations due to Mcph1 Mutation in Microcephaly小头畸形中Mcph1突变引起的细胞代谢改变Journiac, Nathalie; Gilabert-Juan, Javier; Cipriani, Sara; Benit, Paule; Liu, Xiaoqian; Jacquier, Sandrine; Faivre, Valerie; Delahaye-Duriez, Andree; Csaba, Zsolt; Hourcade, Tristan; Melinte, Eliza; Lebon, Sophie; Violle-Poirsier, Celine; Oury, Jean-Francois; Adle-Biassette, Homa; Wang, Zhao-Qi; Mani, Shyamala; Rustin, Pierre; Gressens, Pierre; Nardelli, Jeannette 分享 收藏 Succinate detection using in vivo 1H-MR spectroscopy identifies germline and somatic SDHx mutations in paragangliomasLussey-Lepoutre, Charlotte; Bellucci, Alexandre; Burnichon, Nelly; Amar, Laurence; Buffet, Alexandre; Drossart, Tom; Fontaine, Sebastien; Clement, Olivier; Benit, Paule; Rustin, Pierre; Groussin, Lionel; Meatchi, Tchao; Gimenez-Roqueplo, Anne-Paule; Tavitian, Bertrand; Favier, Judith 分享 收藏 Arabidopsis thaliana alternative dehydrogenases: a potential therapy for mitochondrial complex I deficiency? Perspectives and pitfalls拟南芥替代脱氢酶: 线粒体复合物I缺乏症的潜在疗法？观点和陷阱Catania, Alessia; Iuso, Arcangela; Bouchereau, Juliette; Kremer, Laura S.; Paviolo, Marina; Terrile, Caterina; Benit, Paule; Rasmusson, Allan G.; Schwarzmayr, Thomas; Tiranti, Valeria; Rustin, Pierre; Rak, Malgorzata; Prokisch, Holger; Schiff, Manuel 分享 收藏 Induced pluripotent stem cells-derived neurons from patients with Friedreich ataxia exhibit differential sensitivity to resveratrol and nicotinamideGeorges, Pauline; Boza-Moran, Maria-Gabriela; Gide, Jacqueline; Peche, Georges Arielle; Foret, Benjamin; Bayot, Aurelien; Rustin, Pierre; Peschanski, Marc; Martinat, Cecile; Aubry, Laetitia 分享 收藏 Report on three additional patients and genotype-phenotype correlation in SLC25A22-related disorders groupLemattre, Camille; Imbert-Bouteille, Marion; Gatinois, Vincent; Benit, Paule; Sanchez, Elodie; Guignard, Thomas; Mau-Them, Frederic Tran; Haquet, Emmanuelle; Rivier, Francois; Carme, Emilie; Roubertie, Agathe; Boland, Anne; Lechner, Doris; Meyer, Vincent; Thevenon, Julien; Duffourd, Yannis; Riviere, Jean-Baptiste; Deleuze, Jean-Francois; Wells, Constance; Molinari, Florence; Rustin, Pierre; Blanchet, Patricia; Genevieve, David 分享 收藏 
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