Search Results - "Fougerousse, Françoise"

Rescue of Dystrophic Muscle through U7 snRNA-Mediated Exon Skipping by Goyenvalle, Aurélie, Vulin, Adeline, Fougerousse, Françoise, Leturcq, France, Kaplan, Jean-Claude, Garcia, Luis, Danos, Olivier

“…Most mutations in the dystrophin gene create a frameshift or a stop in the mRNA and are associated with severe Duchenne muscular dystrophy. Exon skipping that…”
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Efficient recovery of dysferlin deficiency by dual adeno-associated vector-mediated gene transfer by Lostal, William, Bartoli, Marc, Bourg, Nathalie, Roudaut, Carinne, Bentaïb, Azeddine, Miyake, Katsuya, Guerchet, Nicolas, Fougerousse, Françoise, McNeil, Paul, Richard, Isabelle

“…Deficiency of the dysferlin protein presents as two major clinical phenotypes: limb–girdle muscular dystrophy type 2B and Miyoshi myopathy. Dysferlin is known…”
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Unraveling the relationships between alpha- and beta-adrenergic modulation and the risk of heart failure by Baudier, Claire, Fougerousse, Françoise, Asselbergs, Folkert W., Guedj, Mickael, Komajda, Michel, Kotecha, Dipak, Thomas Lumbers, R., Schmidt, Amand F., Tyl, Benoît

“…Background The effects of α and ß adrenergic receptor modulation on the risk of developing heart failure (HF) remains uncertain due to a lack of randomized…”
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Natural history of LGMD2A for delineating outcome measures in clinical trials by Richard, Isabelle, Hogrel, Jean‐Yves, Stockholm, Daniel, Payan, Christine A. M., Fougerousse, Françoise, Eymard, Bruno, Mignard, Claude, Lopez de Munain, Adolfo, Fardeau, Michel, Urtizberea, Jon Andoni

“…Objective Limb‐girdle muscular dystophy 2A (LGMD2A, OMIM) is a slowly progressive myopathy caused by the deficiency in calpain 3, a calcium‐dependent cysteine…”
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AAV-mediated intramuscular delivery of myotubularin corrects the myotubular myopathy phenotype in targeted murine muscle and suggests a function in plasma membrane homeostasis by Buj-Bello, Anna, Fougerousse, Françoise, Schwab, Yannick, Messaddeq, Nadia, Spehner, Danièle, Pierson, Christopher R., Durand, Muriel, Kretz, Christine, Danos, Olivier, Douar, Anne-Marie, Beggs, Alan H., Schultz, Patrick, Montus, Marie, Denèfle, Patrice, Mandel, Jean-Louis

“…Myotubular myopathy (XLMTM, OMIM 310400) is a severe congenital muscular disease due to mutations in the myotubularin gene (MTM1) and characterized by the…”
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Dynamic Molecular Combing: Stretching the Whole Human Genome for High-Resolution Studies by Michalet, Xavier, Ekong, Rosemary, Fougerousse, Françoise, Rousseaux, Sophie, Schurra, Catherine, Hornigold, Nick, van Slegtenhorst, Marjon, Wolfe, Jonathan, Povey, Sue, Beckmann, Jacques S., Bensimon, Aaron

“…DNA in amounts representative of hundreds of eukaryotic genomes was extended on silanized surfaces by dynamic molecular combing. The precise measurement of…”
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Transient heart rate reduction improves acute decompensated heart failure‐induced left ventricular and coronary dysfunction by Peschanski, Nicolas, Harouki, Najah, Soulie, Matthieu, Lachaux, Marianne, Nicol, Lionel, Remy‐Jouet, Isabelle, Henry, Jean‐Paul, Dumesnil, Anais, Renet, Sylvanie, Fougerousse, Françoise, Brakenhielm, Ebba, Ouvrard‐Pascaud, Antoine, Thuillez, Christian, Richard, Vincent, Roussel, Jérôme, Mulder, Paul

“…Aims Acute decompensated heart failure (ADHF), a live‐threatening complication of heart failure (HF), associates a further decrease of the already by…”
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Heart rate as an independent predictor of long term mortality of acute heart failure patients in sinus rhythm according to their ejection fraction: data from the AHEAD registry by Jarkovsky, Jiri, Spinar, Jindrich, Tyl, Benoit, Fougerousse, Françoise, Vitovec, Jiri, Linhart, Ales, Widimsky, Petr, Miklik, Roman, Spinarova, Lenka, Belohlavek, Jan, Malek, Filip, Felsoci, Marian, Kettner, Jiri, Ostadal, Petr, Vaclavik, Jan, Dusek, Ladislav, Lokaj, Petr, Mebazaa, Alexandre, Cohen Solal, Alain, Parenica, Jiri

“…•HR appears to be an prognostic parameter in AHF patients in sinus rythm•There are different prognostic cut-off points for HR depending on ejection…”
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Series of Exon-Skipping Events in the Elastic Spring Region of Titin as the Structural Basis for Myofibrillar Elastic Diversity by Freiburg, Alexandra, Trombitas, Karoly, Hell, Wolfgang, Cazorla, Olivier, Fougerousse, Francoise, Centner, Thomas, Kolmerer, Bernhard, Witt, Christian, Beckmann, Jaques S, Gregorio, Carol C, Granzier, Henk, Labeit, Siegfried

“…Titins are megadalton-sized filamentous polypeptides of vertebrate striated muscle. The I-band region of titin underlies the myofibrillar passive tension…”
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Phenotypic Correction of α-Sarcoglycan Deficiency by Intra-arterial Injection of a Muscle-specific Serotype 1 rAAV Vector by Fougerousse, Francoise, Bartoli, Marc, Poupiot, Jérôme, Arandel, Ludovic, Durand, Muriel, Guerchet, Nicolas, Gicquel, Evelyne, Danos, Olivier, Richard, Isabelle

“…α-Sarcoglycanopathy (limb-girdle muscular dystrophy type 2D, LGMD2D) is a recessive muscular disorder caused by deficiency in α-sarcoglycan, a transmembrane…”
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Arginine butyrate: a therapeutic candidate for Duchenne muscular dystrophy by Vianello, Sara, Yu, Hua, Voisin, Vincent, Haddad, Hafedh, He, Xun, Foutz, Arthur S., Sebrié, Catherine, Gillet, Brigitte, Roulot, Morgane, Fougerousse, Françoise, Perronnet, Caroline, Vaillend, Cyrille, Matecki, Stefan, Escolar, Diana, Bossi, Laura, Israël, Maurice, Porte, Sabine

“…As a strategy to treat Duchenne muscular dystrophy, we used arginine butyrate, which combines two pharmacological activities: nitric oxide pathway activation,…”
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Cardiac Characterization of mdx Mice Using High-Resolution Doppler Echocardiography by Fayssoil, A., Renault, G., Guerchet, N., Marchiol-Fournigault, C., Fougerousse, F., Richard, I.

“…OBJECTIVESDuchenne muscular dystrophy is an X-linked neuromuscular disorder. The heart is traditionally involved, leading to heart failure. The mdx mouse is a…”
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Safety and Efficacy of AAV-Mediated Calpain 3 Gene Transfer in a Mouse Model of Limb-Girdle Muscular Dystrophy Type 2A by Bartoli, Marc, Roudaut, Carinne, Martin, Samia, Fougerousse, Françoise, Suel, Laurence, Poupiot, Jérôme, Gicquel, Evelyne, Noulet, Fanny, Danos, Olivier, Richard, Isabelle

“…Calpainopathy (limb-girdle muscular dystrophy type 2A, LGMD2A) is a recessive muscular disorder caused by deficiency in the calcium-dependent cysteine protease…”
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Cardiac Characterization of mdx Mice Using High‐Resolution Doppler Echocardiography by Fayssoil, Abdallah, Renault, Gilles, Guerchet, Nicolas, Marchiol-Fournigault, Carmen, Fougerousse, Françoise, Richard, Isabelle

“…Objectives Duchenne muscular dystrophy is an X‐linked neuromuscular disorder. The heart is traditionally involved, leading to heart failure. The mdx mouse is a…”
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Cardiac Characterization of sgca-Null Mice Using High Resolution Echocardiography by Fayssoil, Abdallah, Renault, Gilles, Guerchet, Nicolas, Marchiol-Fournigault, Carmen, Fougerousse, Françoise, Richard, Isabelle

“…Limb-girdle muscular dystrophy 2D (LGMD2D) is an inherited myogenic disorder belonging to the group of muscular dystrophies. Sgca-null mouse is a knock-out…”
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Mutations in the proteolytic enzyme calpain 3 cause limb-girdle muscular dystrophy type 2A by Richard, Isabelle, Broux, Odile, Allamand, Valéerie, Fougerousse, Françoise, Chiannilkulchai, Nuchanard, Bourg, Nathalie, Brenguier, Lydie, Devaud, Catherine, Pasturaud, Patricia, Roudaut, Carinne, Hillaire, Dominique, Passos-Bueno, Maria-Rita, Zatz, Mayana, Tischfield, Jay A, Fardeau, Michel, Jackson, Charles E, Cohen, Daniel, Beckmann, Jacques S

“…Limb-girdle muscular dystrophies (LGMDs) are a group of inherited diseases whose genetic etiology has yet to be elucidated. The autosomal recessive forms…”
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Genetic ablation of acetylcholinesterase alters muscle function in mice by Vignaud, Alban, Fougerousse, Françoise, Mouisel, Etienne, Bertrand, Christelle, Bonafos, Beatrice, Molgo, Jordi, Ferry, Arnaud, Chatonnet, Arnaud

“…Although acetylcholinesterase (AChE) knockout mice survive, they have abnormal neuromuscular function. We analysed further the effects of the mutation on hind…”
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Dysferlin is a Plasma Membrane Protein and is Expressed Early in Human Development by Anderson, Louise V. B., Davison, Keith, Moss, Jennifer A., Young, Carol, Cullen, Michael J., Walsh, John, Johnson, Margaret A., Bashir, Rumaisa, Britton, Stephen, Keers, Sharon, Argov, Zohar, Mahjneh, Ibrahim, Fougerousse, Françoise, Beckmann, Jacques S., Bushby, Kate M. D.

“…Recently, a single gene, DYSF, has been identified which is mutated in patients with limb-girdle muscular dystrophy type 2B (LGMD2B) and with Miyoshi myopathy…”
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Expression and Functional Characteristics of Calpain 3 Isoforms Generated through Tissue-Specific Transcriptional and Posttranscriptional Events by Herasse, Muriel, Ono, Yasuko, Fougerousse, Françoise, Kimura, Ei-ichi, Stockholm, Daniel, Beley, Cyriaque, Montarras, Didier, Pinset, Christian, Sorimachi, Hiroyuki, Suzuki, Koichi, Beckmann, Jacques S., Richard, Isabelle

“…Article Usage Stats Services MCB Citing Articles Google Scholar PubMed Related Content Social Bookmarking CiteULike Delicious Digg Facebook Google+ Mendeley…”
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Force impairment in calpain 3-deficient mice is not correlated with mechanical disruption by Fougerousse, Françoise, Gonin, Patrick, Durand, Muriel, Richard, Isabelle, Raymackers, Jean-Marc

“…Defects in human calpain 3 are responsible for limb‐girdle muscular dystrophy type 2A, an autosomal‐recessive disorder characterized mainly by late‐onset…”
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