Search Results - "Hoch, Lucile"

Targeting of Smoothened for therapeutic gain by Ruat, Martial, Hoch, Lucile, Faure, Hélène, Rognan, Didier

“…Highlights • Smo antagonists have potential for cancer therapy. • Positive modulators of Smo may also be therapeutics. • Models of canonical and noncanonical…”
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Generation of three induced pluripotent stem cell lines from patients with glycogen storage disease type III by Rossiaud, Lucille, Pellier, Emilie, Benabides, Manon, Nissan, Xavier, Ronzitti, Giuseppe, Hoch, Lucile

“…Glycogen storage disease type III (GSDIII) is an autosomal recessive disorder characterized by a deficiency of glycogen debranching enzyme (GDE) leading to…”
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Identification of thiostrepton as a pharmacological approach to rescue misfolded alpha-sarcoglycan mutant proteins from degradation by Hoch, Lucile, Henriques, Sara F., Bruge, Celine, Marsolier, Justine, Benabides, Manon, Bourg, Nathalie, Tournois, Johana, Mahé, Gurvan, Morizur, Lise, Jarrige, Margot, Bigot, Anne, Richard, Isabelle, Nissan, Xavier

“…Limb-girdle muscular dystrophy type 2D (LGMD2D) is characterized by a progressive proximal muscle weakness. LGMD2D is caused by mutations in the gene encoding…”
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Dual Blockade of Misfolded Alpha-Sarcoglycan Degradation by Bortezomib and Givinostat Combination by Hoch, Lucile, Bourg, Nathalie, Degrugillier, Fanny, Bruge, Céline, Benabides, Manon, Pellier, Emilie, Tournois, Johana, Mahé, Gurvan, Maignan, Nicolas, Dawe, Jack, Georges, Maxime, Papazian, David, Subramanian, Nik, Simon, Stéphanie, Fanen, Pascale, Delevoye, Cédric, Richard, Isabelle, Nissan, Xavier

“…Limb-girdle muscular dystrophy type R3 (LGMD R3) is a rare genetic disorder characterized by a progressive proximal muscle weakness and caused by mutations in…”
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Skeletal Muscle Cells Derived from Induced Pluripotent Stem Cells: A Platform for Limb Girdle Muscular Dystrophies by Bruge, Celine, Geoffroy, Marine, Benabides, Manon, Pellier, Emilie, Gicquel, Evelyne, Dhiab, Jamila, Hoch, Lucile, Richard, Isabelle, Nissan, Xavier

“…Limb girdle muscular dystrophies (LGMD), caused by mutations in 29 different genes, are the fourth most prevalent group of genetic muscle diseases. Although…”
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Pathological modeling of glycogen storage disease type III with CRISPR/Cas9 edited human pluripotent stem cells by Rossiaud, Lucille, Fragner, Pascal, Barbon, Elena, Gardin, Antoine, Benabides, Manon, Pellier, Emilie, Cosette, Jérémie, El Kassar, Lina, Giraud-Triboult, Karine, Nissan, Xavier, Ronzitti, Giuseppe, Hoch, Lucile

“…Glycogen storage disease type III (GSDIII) is a rare genetic disease caused by mutations in the gene encoding the glycogen debranching enzyme (GDE). The…”
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MRT‐92 inhibits Hedgehog signaling by blocking overlapping binding sites in the transmembrane domain of the Smoothened receptor by Hoch, Lucile, Faure, Helene, Roudaut, Hermine, Schoenfelder, Angele, Mann, Andre, Girard, Nicolas, Bihannic, Laure, Ayrault, Olivier, Petricci, Elena, Taddei, Maurizio, Rognan, Didier, Ruat, Martial

“…ABSTRACT The Smoothened (Smo) receptor, a member of class F G protein‐coupled receptors, is the main transducer of the Hedgehog (Hh) signaling pathway…”
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A functional mini-GDE transgene corrects impairment in models of glycogen storage disease type III by Gardin, Antoine, Rouillon, Jérémy, Montaivo-Romera, Valle, Rossiaud, Lucille, Vidal, Patrice, Launay, Romain, Vie, Mallaury, Benchekroun, Youssef Krimi, Cosette, Jérémie, Bertin, Bérangére, La Bella, Tiziana, Dubreuil, Guillaume, Nozi, Justine, Jauze, Louisa, Fragnoud, Romain, Daniele, Nathalie, Van Wittenberghe, Laetitia, Esque, Jérémy, André, Isabelle, Nissan, Xavier, Hoch, Lucile, Ronzitti, Giuseppe

“…Glycogen storage disease type III (CSDIII) is a rare inborn error of metabolism affecting liver, skeletal muscle, and heart due to mutations of the AGŁ gene…”
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A functional mini-GDE transgene corrects impairment in models of glycogen storage disease type III by Gardin, Antoine, Rouillon, Jérémy, Montalvo-Romeral, Valle, Rossiaud, Lucille, Vidal, Patrice, Launay, Romain, Vie, Mallaury, Krimi Benchekroun, Youssef, Cosette, Jérémie, Bertin, Bérangère, La Bella, Tiziana, Dubreuil, Guillaume, Nozi, Justine, Jauze, Louisa, Fragnoud, Romain, Daniele, Nathalie, Van Wittenberghe, Laetitia, Esque, Jérémy, André, Isabelle, Nissan, Xavier, Hoch, Lucile, Ronzitti, Giuseppe

“…Glycogen storage disease type III (GSDIII) is a rare inborn error of metabolism affecting liver, skeletal muscle, and heart due to mutations of the AGL gene…”
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Hedgehog associated to microparticles inhibits adipocyte differentiation via a non-canonical pathway by Fleury, Audrey, Hoch, Lucile, Martinez, M. Carmen, Faure, Hélène, Taddei, Maurizio, Petricci, Elena, Manetti, Fabrizio, Girard, Nicolas, Mann, André, Jacques, Caroline, Larghero, Jérôme, Ruat, Martial, Andriantsitohaina, Ramaroson, Le Lay, Soazig

“…Hedgehog (Hh) is a critical regulator of adipogenesis. Extracellular vesicles are natural Hh carriers, as illustrated by activated/apoptotic lymphocytes…”
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Design, synthesis and biological characterization of a new class of osteogenic (1H)-quinolone derivatives by Manetti, Fabrizio, Petricci, Elena, Gabrielli, Annalisa, Mann, Andrè, Faure, Hélène, Gorojankina, Tatiana, Brasseur, Laurent, Hoch, Lucile, Ruat, Martial, Taddei, Maurizio

“…Smoothened (Smo) is the signal transducer of the Hedgehog (Hh) pathway and its stimulation is considered a potential powerful tool in regenerative medicine to…”
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Pathological modelling of pigmentation disorders associated with Hutchinson-Gilford Progeria Syndrome (HGPS) revealed an impaired melanogenesis pathway in iPS-derived melanocytes by Lo Cicero, Alessandra, Saidani, Manoubia, Allouche, Jennifer, Egesipe, Anne Laure, Hoch, Lucile, Bruge, Celine, Sigaudy, Sabine, De Sandre-Giovannoli, Annachiara, Levy, Nicolas, Baldeschi, Christine, Nissan, Xavier

“…Hutchinson-Gilford Progeria Syndrome (HGPS) is a rare genetic disorder that leads to premature aging. In this study, we used induced pluripotent stem cells to…”
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Discovery, molecular and pharmacological characterization of GSA-10, a novel small-molecule positive modulator of Smoothened by Gorojankina, Tatiana, Hoch, Lucile, Faure, Hélène, Roudaut, Hermine, Traiffort, Elisabeth, Schoenfelder, Angèle, Girard, Nicolas, Mann, André, Manetti, Fabrizio, Solinas, Antonio, Petricci, Elena, Taddei, Maurizio, Ruat, Martial

“…Activation of the Smoothened (Smo) receptor mediates Hedgehog (Hh) signaling. Hh inhibitors are in clinical trials for cancer, and small-molecule Smo agonists…”
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Synergism of dual AAV gene therapy and rapamycin rescues GSDIII phenotype in muscle and liver by Jauze, Louisa, Vie, Mallaury, Miagoux, Quentin, Rossiaud, Lucille, Vidal, Patrice, Montalvo-Romeral, Valle, Saliba, Hanadi, Jarrige, Margot, Polveche, Helene, Nozi, Justine, Le Brun, Pierre-Romain, Bocchialini, Luca, Francois, Amandine, Cosette, Jérémie, Rouillon, Jérémy, Collaud, Fanny, Bordier, Fanny, Bertil-Froidevaux, Emilie, Georger, Christophe, van Wittenberghe, Laetitia, Miranda, Adeline, Daniele, Nathalie F, Gross, David-Alexandre, Hoch, Lucile, Nissan, Xavier, Ronzitti, Giuseppe

“…Glycogen storage disease type III (GSDIII) is a rare metabolic disorder due to glycogen debranching enzyme (GDE) deficiency. Reduced GDE activity leads to…”
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Structure of the Smoothened receptor by Ruat, Martial, Hoch, Lucile, Faure, Hélène, Rognan, Didier

“…The Smoothened (Smo) receptor is a major component involved in signal transduction of the Hedgehog (Hh) morphogens both during embryogenesis and in the adult…”
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