Search Results - "Parone, Philippe A."

Preventing mitochondrial fission impairs mitochondrial function and leads to loss of mitochondrial DNA by Parone, Philippe A, Da Cruz, Sandrine, Tondera, Daniel, Mattenberger, Yves, James, Dominic I, Maechler, Pierre, Barja, François, Martinou, Jean-Claude

“…Mitochondria form a highly dynamic tubular network, the morphology of which is regulated by frequent fission and fusion events. However, the role of…”
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ALS-linked TDP-43 mutations produce aberrant RNA splicing and adult-onset motor neuron disease without aggregation or loss of nuclear TDP-43 by Arnold, Eveline S, Ling, Shuo-Chien, Huelga, Stephanie C, Lagier-Tourenne, Clotilde, Polymenidou, Magdalini, Ditsworth, Dara, Kordasiewicz, Holly B, McAlonis-Downes, Melissa, Platoshyn, Oleksandr, Parone, Philippe A, Da Cruz, Sandrine, Clutario, Kevin M, Swing, Debbie, Tessarollo, Lino, Marsala, Martin, Shaw, Christopher E, Yeo, Gene W, Cleveland, Don W

“…Transactivating response region DNA binding protein (TDP-43) is the major protein component of ubiquitinated inclusions found in amyotrophic lateral sclerosis…”
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Enhancing mitochondrial calcium buffering capacity reduces aggregation of misfolded SOD1 and motor neuron cell death without extending survival in mouse models of inherited amyotrophic lateral sclerosis by Parone, Philippe A, Da Cruz, Sandrine, Han, Joo Seok, McAlonis-Downes, Melissa, Vetto, Anne P, Lee, Sandra K, Tseng, Eva, Cleveland, Don W

“…Mitochondria have been proposed as targets for toxicity in amyotrophic lateral sclerosis (ALS), a progressive, fatal adult-onset neurodegenerative disorder…”
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Elevated PGC-1α Activity Sustains Mitochondrial Biogenesis and Muscle Function without Extending Survival in a Mouse Model of Inherited ALS by Da Cruz, Sandrine, Parone, Philippe A., Lopes, Vanda S., Lillo, Concepción, McAlonis-Downes, Melissa, Lee, Sandra K., Vetto, Anne P., Petrosyan, Susanna, Marsala, Martin, Murphy, Anne N., Williams, David S., Spiegelman, Bruce M., Cleveland, Don W.

“…The transcriptional coactivator PGC-1α induces multiple effects on muscle, including increased mitochondrial mass and activity. Amyotrophic lateral sclerosis…”
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hFis1, a Novel Component of the Mammalian Mitochondrial Fission Machinery by James, Dominic I., Parone, Philippe A., Mattenberger, Yves, Martinou, Jean-Claude

“…The balance between the fission and fusion mechanisms regulate the morphology of mitochondria. In this study we have identified a mammalian protein that we…”
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Fused in Sarcoma (FUS) Protein Lacking Nuclear Localization Signal (NLS) and Major RNA Binding Motifs Triggers Proteinopathy and Severe Motor Phenotype in Transgenic Mice by Shelkovnikova, Tatyana A., Peters, Owen M., Deykin, Alexey V., Connor-Robson, Natalie, Robinson, Hannah, Ustyugov, Alexey A., Bachurin, Sergey O., Ermolkevich, Tatyana G., Goldman, Igor L., Sadchikova, Elena R., Kovrazhkina, Elena A., Skvortsova, Veronica I., Ling, Shuo-Chien, Da Cruz, Sandrine, Parone, Philippe A., Buchman, Vladimir L., Ninkina, Natalia N.

“…Dysfunction of two structurally and functionally related proteins, FUS and TAR DNA-binding protein of 43 kDa (TDP-43), implicated in crucial steps of cellular…”
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Overriding FUS autoregulation in mice triggers gain-of-toxic dysfunctions in RNA metabolism and autophagy-lysosome axis by Ling, Shuo-Chien, Dastidar, Somasish Ghosh, Tokunaga, Seiya, Ho, Wan Yun, Lim, Kenneth, Ilieva, Hristelina, Parone, Philippe A, Tyan, Sheue-Houy, Tse, Tsemay M, Chang, Jer-Cherng, Platoshyn, Oleksandr, Bui, Ngoc B, Bui, Anh, Vetto, Anne, Sun, Shuying, McAlonis-Downes, Melissa, Han, Joo Seok, Swing, Debbie, Kapeli, Katannya, Yeo, Gene W, Tessarollo, Lino, Marsala, Martin, Shaw, Christopher E, Tucker-Kellogg, Greg, La Spada, Albert R, Lagier-Tourenne, Clotilde, Da Cruz, Sandrine, Cleveland, Don W

“…Mutations in coding and non-coding regions of FUS cause amyotrophic lateral sclerosis (ALS). The latter mutations may exert toxicity by increasing FUS…”
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Inhibiting the Mitochondrial Fission Machinery Does Not Prevent Bax/Bak-Dependent Apoptosis by Parone, Philippe A, James, Dominic I, Da Cruz, Sandrine, Mattenberger, Yves, Donzé, Olivier, Barja, François, Martinou, Jean-Claude

“…Article Usage Stats Services MCB Citing Articles Google Scholar PubMed Related Content Social Bookmarking CiteULike Delicious Digg Facebook Google+ Mendeley…”
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ALS/FTD-Linked Mutation in FUS Suppresses Intra-axonal Protein Synthesis and Drives Disease Without Nuclear Loss-of-Function of FUS by López-Erauskin, Jone, Tadokoro, Takahiro, Baughn, Michael W., Myers, Brian, McAlonis-Downes, Melissa, Chillon-Marinas, Carlos, Asiaban, Joshua N., Artates, Jonathan, Bui, Anh T., Vetto, Anne P., Lee, Sandra K., Le, Ai Vy, Sun, Ying, Jambeau, Mélanie, Boubaker, Jihane, Swing, Deborah, Qiu, Jinsong, Hicks, Geoffrey G., Ouyang, Zhengyu, Fu, Xiang-Dong, Tessarollo, Lino, Ling, Shuo-Chien, Parone, Philippe A., Shaw, Christopher E., Marsala, Martin, Lagier-Tourenne, Clotilde, Cleveland, Don W., Da Cruz, Sandrine

“…Through the generation of humanized FUS mice expressing full-length human FUS, we identify that when expressed at near endogenous murine FUS levels, both…”
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Misfolded SOD1 is not a primary component of sporadic ALS by Da Cruz, Sandrine, Bui, Anh, Saberi, Shahram, Lee, Sandra K., Stauffer, Jennifer, McAlonis-Downes, Melissa, Schulte, Derek, Pizzo, Donald P., Parone, Philippe A., Cleveland, Don W., Ravits, John

“…A common feature of inherited and sporadic ALS is accumulation of abnormal proteinaceous inclusions in motor neurons and glia. SOD1 is the major protein…”
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ALS/FTD-Linked Mutation in FUS Suppresses Intra-axonal Protein Synthesis and Drives Disease Without Nuclear Loss-of-Function of FUS by López-Erauskin, Jone, Tadokoro, Takahiro, Baughn, Michael W., Myers, Brian, McAlonis-Downes, Melissa, Chillon-Marinas, Carlos, Asiaban, Joshua N., Artates, Jonathan, Bui, Anh T., Vetto, Anne P., Lee, Sandra K., Le, Ai Vy, Sun, Ying, Jambeau, Mélanie, Boubaker, Jihane, Swing, Deborah, Qiu, Jinsong, Hicks, Geoffrey G., Ouyang, Zhengyu, Fu, Xiang-Dong, Tessarollo, Lino, Ling, Shuo-Chien, Parone, Philippe A., Shaw, Christopher E., Marsala, Martin, Lagier-Tourenne, Clotilde, Cleveland, Don W., Da Cruz, Sandrine

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Mitochondrial fission and apoptosis: An ongoing trial by Parone, Philippe A., Martinou, Jean-Claude

“…Apoptosis is a form of programmed cell death that is essential for the development and tissue homeostasis in all metazoan animals. Mitochondria play a critical…”
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SLP-2 interacts with prohibitins in the mitochondrial inner membrane and contributes to their stability by Da Cruz, Sandrine, Parone, Philippe A., Gonzalo, Philippe, Bienvenut, Willy V., Tondera, Daniel, Jourdain, Alexis, Quadroni, Manfredo, Martinou, Jean-Claude

“…Stomatin is a member of a large family of proteins including prohibitins, HflK/C, flotillins, mechanoreceptors and plant defense proteins, that are thought to…”
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SLP-2 negatively modulates mitochondrial sodium–calcium exchange by Da Cruz, Sandrine, De Marchi, Umberto, Frieden, Maud, Parone, Philippe A, Martinou, Jean-Claude, Demaurex, Nicolas

“…Abstract Mitochondria play a major role in cellular calcium homeostasis. Despite decades of studies, the molecules that mediate and regulate the transport of…”
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Building the mitochondrial proteome by Da Cruz, Sandrine, Parone, Philippe A, Martinou, Jean-Claude

“…Mitochondria are essential organelles for cellular homeostasis. A variety of pathologies including cancer, myopathies, diabetes, obesity, aging and…”
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Mitochondrial Isolation and Purification from Mouse Spinal Cord by Philippe Parone, Sandrine Cruz, Don Cleveland

“…Mitochondria are eukaryotic organelles that play a crucial role in several cellular processes, including energy production, β-oxidation of fatty acids and…”
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Mechanisms of mitochondrial outer membrane permeabilization by James, Dominic, Parone, Philippe A, Terradillos, Olivier, Lucken-Ardjomande, Safa, Montessuit, Sylvie, Martinou, Jean-Claude

“…In response to many apoptotic stimuli, Bcl-2 family pro-apoptotic members, such as Bax and Bak, are activated. This results in their oligomerization,…”
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ALS-linked TDP-43 mutations produce aberrant RNA splicing and adult-onset motor neuron disease without aggregation or loss of nuclear TDP-43 by Arnold, Eveline S., Ling, Shuo-Chien, Huelga, Stephanie C., Lagier-Tourenne, Clotilde, Polymenidou, Magdalini, Ditsworth, Dara, Kordasiewicz, Holly B., McAlonis-Downes, Melissa, Platoshyn, Oleksandr, Parone, Philippe A., Da Cruz, Sandrine, Clutario, Kevin M., Swing, Debbie, Tessarollo, Lino, Marsala, Martin, Shaw, Christopher E., Yeo, Gene W., Cleveland, Don W.

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hFis1, a novel component of the mammalian mitochondrial fission machinery. Vol. 278 (2003) 36373–36379 by James, Dominic I., Parone, Philippe A., Mattenberger, Yves, Martinou, Jean-Claude

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