Straight-forward synthesis of ringed particles von Roche, J., Loget, G., Zigah, D., Fattah, Z., Goudeau, B., Arbault, S., Bouffier, L., Kuhn, A.

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Cardiac arrhythmia and late-onset muscle weakness caused by a myofibrillar myopathy with unusual histopathological features due to a novel missense mutation in FLNC von Avila-Smirnow, D., Gueneau, L., Batonnet-Pichon, S., Delort, F., Bécane, H.-M., Claeys, K., Beuvin, M., Goudeau, B., Jais, J.-P., Nelson, I., Richard, P., Ben Yaou, R., Romero, N.B., Wahbi, K., Mathis, S., Voit, T., Furst, D., van der Ven, P., Gil, R., Vicart, P., Fardeau, M., Bonne, G., Behin, A.

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On noxious desmin: functional effects of a novel heterozygous desmin insertion mutation on the extrasarcomeric desmin cytoskeleton and mitochondria von Schröder, Rolf, Goudeau, Bertrand, Simon, Monique Casteras, Fischer, Dirk, Eggermann, Thomas, Clemen, Christoph S., Li, Zhenlin, Reimann, Jens, Xue, Zhigang, Rudnik-Schöneborn, Sabine, Zerres, Klaus, van der Ven, Peter F. M., Fürst, Dieter O., Kunz, Wolfram S., Vicart, Patrick

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Sealing hyaluronic acid microgels with oppositely-charged polypeptides: A simple strategy for packaging hydrophilic drugs with on-demand release von Labie, Hélène, Perro, Adeline, Lapeyre, Véronique, Goudeau, Bertrand, Catargi, Bogdan, Auzély, Rachel, Ravaine, Valérie

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P2.18 A novel missense FLNC mutation causes arrhythmia and late onset myofibrillar myopathy with particular histopathology features von Avila-Smirnow, D, Béhin, A, Gueneau, L, Claeys, K, Beuvin, M, Goudeau, B, Richard, P, Yaou, R. Ben, Romero, N.B, Mathis, S, Voit, T, Eymard, B, Gil, R, Fardeau, M, Bonne, G

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D.P.3.02 Desminopathies: What can we learn from a long term follow-up? von Behin, A, Stojkovic, T, Claeys, K, Wahbi, K, Duboc, D, Becane, H.M, Dubourg, O, Maisonobe, T, Stoltenburg, G, Fardeau, M, Richard, P, Goudeau, B, Vicart, P, Eymard, B

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Forebrain-Specific Neuronal Inhibition of Nuclear Factor-κB Activity Leads to Loss of Neuroprotection von Fridmacher, Valérie, Kaltschmidt, Barbara, Goudeau, Bertrand, Ndiaye, Delphine, Rossi, Francesco Mattia, Pfeiffer, Julia, Kaltschmidt, Christian, Israël, Alain, Mémet, Sylvie

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Straight-forward synthesis of ringed particlesElectronic supplementary information (ESI) available. See DOI: 10.1039/c3sc53329h von Roche, J, Loget, G, Zigah, D, Fattah, Z, Goudeau, B, Arbault, S, Bouffier, L, Kuhn, A

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Pathogenic effects of a novel heterozygous R350P desmin mutation on the assembly of desmin intermediate filaments in vivo and in vitro von Bär, Harald, Fischer, Dirk, Goudeau, Bertrand, Kley, Rudolf A., Clemen, Christoph S., Vicart, Patrick, Herrmann, Harald, Vorgerd, Matthias, Schröder, Rolf

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Variable pathogenic potentials of mutations located in the desmin alpha-helical domain von Goudeau, Bertrand, Rodrigues-Lima, Fernando, Fischer, Dirk, Casteras-Simon, Monique, Sambuughin, Nyamkhishig, de Visser, Marianne, Laforet, Pascal, Ferrer, Xavier, Chapon, Françoise, Sjöberg, Gunnar, Kostareva, Anna, Sejersen, Thomas, Dalakas, Marinos C., Goldfarb, Lev G., Vicart, Patrick

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IκBα/IκBε Deficiency Reveals That a Critical NF-κB Dosage Is Required for Lymphocyte Survival von Goudeau, Bertrand, Huetz, François, Samson, Sandrine, Di Santo, James P., Cumano, Ana, Beg, Amer, Israël, Alain, Mémet, Sylvie

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Cardiac arrhythmia and late-onset muscle weakness caused by a myofibrillar myopathy with unusual histopathological features due to a novel missense mutation in FLNC von Avila-Smirnow, D, Gueneau, L, Batonnet-Pichon, S, Delort, F, Bécane, H.-M, Claeys, Kristl, Beuvin, M, Goudeau, B, Jais, J.-P, Nelson, I, Richard, P, Ben Yaou, R, Romero, N.B, Wahbi, K, Mathis, S, Voit, T, Furst, D, van der Ven, P, Gil, Rodrigo, Vicart, P, Fardeau, M, Bonne, G, Behin, A

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Small deletions disturb desmin architecture leading to breakdown of muscle cells and development of skeletal or cardioskeletal myopathy von Kaminska, Anna, Strelkov, Sergei V, Goudeau, Bertrand, Olivé, Montse, Dagvadorj, Ayush, Fidzianska, Anna, Simon-Casteras, Monique, Shatunov, Alexey, Dalakas, Marinos C, Ferrer, Isidro, Kwiecinski, Hubert, Vicart, Patrick, Goldfarb, Lev G

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Hsp27-2D-gel electrophoresis is a diagnostic tool to differentiate primary desminopathies from myofibrillar myopathies von Clemen, Christoph S., Fischer, Dirk, Roth, Udo, Simon, Stéphanie, Vicart, Patrick, Kato, Kanefusa, Kaminska, Anna M., Vorgerd, Matthias, Goldfarb, Lev G., Eymard, Bruno, Romero, Norma B., Goudeau, Bertrand, Eggermann, Thomas, Zerres, Klaus, Noegel, Angelika A., Schröder, Rolf

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Structural and functional analysis of a new desmin variant causing desmin-related myopathy von Goudeau, Bertrand, Dagvadorj, Ayush, Rodrigues-Lima, Fernando, Nédellec, Patrick, Casteras-Simon, Monique, Perret, Emmanuelle, Langlois, Sylvie, Goldfarb, Lev, Vicart, Patrick

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Respiratory insufficiency in desminopathy patients caused by introduction of proline residues in desmin c-terminal α-helical segment von Dagvadorj, Ayush, Goudeau, Bertrand, Hilton-Jones, David, Blancato, Jan K., Shatunov, Alexey, Simon-Casteras, Monique, Squier, Waney, Nagle, James W., Goldfarb, Lev G., Vicart, Patrick

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Progressive skeletal myopathy, a phenotypic variant of desmin myopathy associated with desmin mutations von Dalakas, Marinos C., Dagvadorj, Ayush, Goudeau, Bertrand, Park, Kye-Yoon, Takeda, Kazuyo, Simon-Casteras, Monique, Vasconcelos, Olavo, Sambuughin, Nyamkhishig, Shatunov, Alexey, Nagle, James W., Sivakumar, Kumaraswamy, Vicart, Patrick, Goldfarb, Lev G.

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A novel desmin R355P mutation causes cardiac and skeletal myopathy von Fidziańska, Anna, Kotowicz, Jerzy, Sadowska, Marta, Goudeau, Bertrand, Walczak, Ewa, Vicart, Patrick, Hausmanowa-Petrusewicz, Irena

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Skeletal Muscles Express the Xenobiotic-metabolizing Enzyme Arylamine N-acetyltransferase von Rodrigues-Lima, Fernando, Cooper, Racquel N, Goudeau, Bertrand, Atmane, Noureddine, Chamagne, Anne-Marie, Butler-Browne, Gillian, Sim, Edith, Vicart, Patrick, Dupret, Jean-Marie

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On noxious desmin: functional effects of a novel heterozygous desmin insertion mutation on the extrasarcomeric desmin cytoskeleton and mitochondria von Schröder, Rolf, Goudeau, Bertrand, Simon, Monique Casteras, Fischer, Dirk, Eggermann, Thomas, Clemen, Christoph S., Li, Zhenlin, Reimann, Jens, Xue, Zhigang, Rudnik-Schöneborn, Sabine, Zerres, Klaus, van der Ven, Peter F. M., Fürst, Dieter O., Kunz, Wolfram S., Vicart, Patrick

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