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Activated dendritic cells modulate proliferation and differentiation of human myoblasts.

Ladislau L, Portilho DM, Courau T, Solares-Pérez A, Negroni E, Lainé J, Klatzmann D, Bonomo A, Allenbach Y, Benveniste O, Riederer I, Savino W, Mouly V, Butler-Browne G, Benjamim CF.

Cell Death Dis. 2018 May 10;9(5):551. doi: 10.1038/s41419-018-0426-z.


JAK inhibitor improves type I interferon induced damage: proof of concept in dermatomyositis.

Ladislau L, Suárez-Calvet X, Toquet S, Landon-Cardinal O, Amelin D, Depp M, Rodero MP, Hathazi D, Duffy D, Bondet V, Preusse C, Bienvenu B, Rozenberg F, Roos A, Benjamim CF, Gallardo E, Illa I, Mouly V, Stenzel W, Butler-Browne G, Benveniste O, Allenbach Y.

Brain. 2018 May 8. doi: 10.1093/brain/awy105. [Epub ahead of print]


Combined Therapies for Duchenne Muscular Dystrophy to Optimize Treatment Efficacy.

Cordova G, Negroni E, Cabello-Verrugio C, Mouly V, Trollet C.

Front Genet. 2018 Apr 10;9:114. doi: 10.3389/fgene.2018.00114. eCollection 2018. Review.


A novel long non-coding RNA Myolinc regulates myogenesis through TDP-43 and Filip1.

Militello G, Hosen MR, Ponomareva Y, Gellert P, Weirick T, John D, Hindi SM, Mamchaoui K, Mouly V, Döring C, Zhang L, Nakamura M, Kumar A, Fukada SI, Dimmeler S, Uchida S.

J Mol Cell Biol. 2018 Apr 1;10(2):102-117. doi: 10.1093/jmcb/mjy025.


Reversible immortalisation enables genetic correction of human muscle progenitors and engineering of next-generation human artificial chromosomes for Duchenne muscular dystrophy.

Benedetti S, Uno N, Hoshiya H, Ragazzi M, Ferrari G, Kazuki Y, Moyle LA, Tonlorenzi R, Lombardo A, Chaouch S, Mouly V, Moore M, Popplewell L, Kazuki K, Katoh M, Naldini L, Dickson G, Messina G, Oshimura M, Cossu G, Tedesco FS.

EMBO Mol Med. 2018 Feb;10(2):254-275. doi: 10.15252/emmm.201607284.


Skeletal Muscle Regenerative Potential of Human MuStem Cells following Transplantation into Injured Mice Muscle.

Lorant J, Saury C, Schleder C, Robriquet F, Lieubeau B, Négroni E, Leroux I, Chabrand L, Viau S, Babarit C, Ledevin M, Dubreil L, Hamel A, Magot A, Thorin C, Guevel L, Delorme B, Péréon Y, Butler-Browne G, Mouly V, Rouger K.

Mol Ther. 2018 Feb 7;26(2):618-633. doi: 10.1016/j.ymthe.2017.10.013. Epub 2017 Oct 20.


Flow Cytometry-Defined CD49d Expression in Circulating T-Lymphocytes Is a Biomarker for Disease Progression in Duchenne Muscular Dystrophy.

Savino W, Pinto-Mariz F, Mouly V.

Methods Mol Biol. 2018;1687:219-227. doi: 10.1007/978-1-4939-7374-3_16.


HGF potentiates extracellular matrix-driven migration of human myoblasts: involvement of matrix metalloproteinases and MAPK/ERK pathway.

González MN, de Mello W, Butler-Browne GS, Silva-Barbosa SD, Mouly V, Savino W, Riederer I.

Skelet Muscle. 2017 Oct 10;7(1):20. doi: 10.1186/s13395-017-0138-6.


Quantitative Antisense Screening and Optimization for Exon 51 Skipping in Duchenne Muscular Dystrophy.

Echigoya Y, Lim KRQ, Trieu N, Bao B, Miskew Nichols B, Vila MC, Novak JS, Hara Y, Lee J, Touznik A, Mamchaoui K, Aoki Y, Takeda S, Nagaraju K, Mouly V, Maruyama R, Duddy W, Yokota T.

Mol Ther. 2017 Nov 1;25(11):2561-2572. doi: 10.1016/j.ymthe.2017.07.014. Epub 2017 Jul 28.


MicroRNA-542 Promotes Mitochondrial Dysfunction and SMAD Activity and Is Elevated in Intensive Care Unit-acquired Weakness.

Garros RF, Paul R, Connolly M, Lewis A, Garfield BE, Natanek SA, Bloch S, Mouly V, Griffiths MJ, Polkey MI, Kemp PR.

Am J Respir Crit Care Med. 2017 Dec 1;196(11):1422-1433. doi: 10.1164/rccm.201701-0101OC.


Obestatin Increases the Regenerative Capacity of Human Myoblasts Transplanted Intramuscularly in an Immunodeficient Mouse Model.

Santos-Zas I, Negroni E, Mamchaoui K, Mosteiro CS, Gallego R, Butler-Browne GS, Pazos Y, Mouly V, Camiña JP.

Mol Ther. 2017 Oct 4;25(10):2345-2359. doi: 10.1016/j.ymthe.2017.06.022. Epub 2017 Jul 24.


Obestatin controls the ubiquitin-proteasome and autophagy-lysosome systems in glucocorticoid-induced muscle cell atrophy.

Cid-Díaz T, Santos-Zas I, González-Sánchez J, Gurriarán-Rodríguez U, Mosteiro CS, Casabiell X, García-Caballero T, Mouly V, Pazos Y, Camiña JP.

J Cachexia Sarcopenia Muscle. 2017 Dec;8(6):974-990. doi: 10.1002/jcsm.12222. Epub 2017 Jul 3.


Correction of the Exon 2 Duplication in DMD Myoblasts by a Single CRISPR/Cas9 System.

Lattanzi A, Duguez S, Moiani A, Izmiryan A, Barbon E, Martin S, Mamchaoui K, Mouly V, Bernardi F, Mavilio F, Bovolenta M.

Mol Ther Nucleic Acids. 2017 Jun 16;7:11-19. doi: 10.1016/j.omtn.2017.02.004. Epub 2017 Feb 10.


Obestatin controls skeletal muscle fiber-type determination.

Santos-Zas I, Cid-Díaz T, González-Sánchez J, Gurriarán-Rodriguez U, Seoane-Mosteiro C, Porteiro B, Nogueiras R, Casabiell X, Luis Relova J, Gallego R, Mouly V, Pazos Y, Camiña JP.

Sci Rep. 2017 May 18;7(1):2137. doi: 10.1038/s41598-017-02337-4.


Quiescence of human muscle stem cells is favored by culture on natural biopolymeric films.

Monge C, DiStasio N, Rossi T, Sébastien M, Sakai H, Kalman B, Boudou T, Tajbakhsh S, Marty I, Bigot A, Mouly V, Picart C.

Stem Cell Res Ther. 2017 May 2;8(1):104. doi: 10.1186/s13287-017-0556-8.


PABPN1 gene therapy for oculopharyngeal muscular dystrophy.

Malerba A, Klein P, Bachtarzi H, Jarmin SA, Cordova G, Ferry A, Strings V, Espinoza MP, Mamchaoui K, Blumen SC, St Guily JL, Mouly V, Graham M, Butler-Browne G, Suhy DA, Trollet C, Dickson G.

Nat Commun. 2017 Mar 31;8:14848. doi: 10.1038/ncomms14848.


Pathogenic role of anti-signal recognition protein and anti-3-Hydroxy-3-methylglutaryl-CoA reductase antibodies in necrotizing myopathies: Myofiber atrophy and impairment of muscle regeneration in necrotizing autoimmune myopathies.

Arouche-Delaperche L, Allenbach Y, Amelin D, Preusse C, Mouly V, Mauhin W, Tchoupou GD, Drouot L, Boyer O, Stenzel W, Butler-Browne G, Benveniste O.

Ann Neurol. 2017 Apr;81(4):538-548. doi: 10.1002/ana.24902.


Immortalized human myotonic dystrophy muscle cell lines to assess therapeutic compounds.

Arandel L, Polay Espinoza M, Matloka M, Bazinet A, De Dea Diniz D, Naouar N, Rau F, Jollet A, Edom-Vovard F, Mamchaoui K, Tarnopolsky M, Puymirat J, Battail C, Boland A, Deleuze JF, Mouly V, Klein AF, Furling D.

Dis Model Mech. 2017 Apr 1;10(4):487-497. doi: 10.1242/dmm.027367. Epub 2017 Feb 10.


CRISPR/Cas9-Induced (CTG⋅CAG)n Repeat Instability in the Myotonic Dystrophy Type 1 Locus: Implications for Therapeutic Genome Editing.

van Agtmaal EL, André LM, Willemse M, Cumming SA, van Kessel IDG, van den Broek WJAA, Gourdon G, Furling D, Mouly V, Monckton DG, Wansink DG, Wieringa B.

Mol Ther. 2017 Jan 4;25(1):24-43. doi: 10.1016/j.ymthe.2016.10.014. Epub 2017 Jan 4.


Skeletal muscle characteristics are preserved in hTERT/cdk4 human myogenic cell lines.

Thorley M, Duguez S, Mazza EMC, Valsoni S, Bigot A, Mamchaoui K, Harmon B, Voit T, Mouly V, Duddy W.

Skelet Muscle. 2016 Dec 8;6(1):43. doi: 10.1186/s13395-016-0115-5.


Functional Characterization of a Central Core Disease RyR1 Mutation (p.Y4864H) Associated with Quantitative Defect in RyR1 Protein.

Cacheux M, Blum A, Sébastien M, Wozny AS, Brocard J, Mamchaoui K, Mouly V, Roux-Buisson N, Rendu J, Monnier N, Krivosic R, Allen P, Lacour A, Lunardi J, Fauré J, Marty I.

J Neuromuscul Dis. 2015 Nov 20;2(4):421-432.


Nuclear poly(A)-binding protein aggregates misplace a pre-mRNA outside of SC35 speckle causing its abnormal splicing.

Klein P, Oloko M, Roth F, Montel V, Malerba A, Jarmin S, Gidaro T, Popplewell L, Perie S, Lacau St Guily J, de la Grange P, Antoniou MN, Dickson G, Butler-Browne G, Bastide B, Mouly V, Trollet C.

Nucleic Acids Res. 2016 Dec 15;44(22):10929-10945. Epub 2016 Aug 9.


Novel small molecules potentiate premature termination codon readthrough by aminoglycosides.

Baradaran-Heravi A, Balgi AD, Zimmerman C, Choi K, Shidmoossavee FS, Tan JS, Bergeaud C, Krause A, Flibotte S, Shimizu Y, Anderson HJ, Mouly V, Jan E, Pfeifer T, Jaquith JB, Roberge M.

Nucleic Acids Res. 2016 Aug 19;44(14):6583-98. doi: 10.1093/nar/gkw638. Epub 2016 Jul 12.


MyoD reprogramming requires Six1 and Six4 homeoproteins: genome-wide cis-regulatory module analysis.

Santolini M, Sakakibara I, Gauthier M, Ribas-Aulinas F, Takahashi H, Sawasaki T, Mouly V, Concordet JP, Defossez PA, Hakim V, Maire P.

Nucleic Acids Res. 2016 Oct 14;44(18):8621-8640. Epub 2016 Jun 14.


Membrane repair of human skeletal muscle cells requires Annexin-A5.

Carmeille R, Bouvet F, Tan S, Croissant C, Gounou C, Mamchaoui K, Mouly V, Brisson AR, Bouter A.

Biochim Biophys Acta. 2016 Sep;1863(9):2267-79. doi: 10.1016/j.bbamcr.2016.06.003. Epub 2016 Jun 7.


The absence of dysferlin induces the expression of functional connexin-based hemichannels in human myotubes.

Cea LA, Bevilacqua JA, Arriagada C, Cárdenas AM, Bigot A, Mouly V, Sáez JC, Caviedes P.

BMC Cell Biol. 2016 May 24;17 Suppl 1:15. doi: 10.1186/s12860-016-0096-6.


Laminopathies disrupt epigenomic developmental programs and cell fate.

Perovanovic J, Dell'Orso S, Gnochi VF, Jaiswal JK, Sartorelli V, Vigouroux C, Mamchaoui K, Mouly V, Bonne G, Hoffman EP.

Sci Transl Med. 2016 Apr 20;8(335):335ra58. doi: 10.1126/scitranslmed.aad4991.


Knockdown of Lmo7 inhibits chick myogenesis.

Possidonio AC, Soares CP, Fontenele M, Morris ER, Mouly V, Costa ML, Mermelstein C.

FEBS Lett. 2016 Feb;590(3):317-29. doi: 10.1002/1873-3468.12067. Epub 2016 Feb 1.


Selection-free gene repair after adenoviral vector transduction of designer nucleases: rescue of dystrophin synthesis in DMD muscle cell populations.

Maggio I, Stefanucci L, Janssen JM, Liu J, Chen X, Mouly V, Gonçalves MA.

Nucleic Acids Res. 2016 Feb 18;44(3):1449-70. doi: 10.1093/nar/gkv1540. Epub 2016 Jan 13.


Spell Checking Nature: Versatility of CRISPR/Cas9 for Developing Treatments for Inherited Disorders.

Wojtal D, Kemaladewi DU, Malam Z, Abdullah S, Wong TW, Hyatt E, Baghestani Z, Pereira S, Stavropoulos J, Mouly V, Mamchaoui K, Muntoni F, Voit T, Gonorazky HD, Dowling JJ, Wilson MD, Mendoza-Londono R, Ivakine EA, Cohn RD.

Am J Hum Genet. 2016 Jan 7;98(1):90-101. doi: 10.1016/j.ajhg.2015.11.012. Epub 2015 Dec 10.


CD49d is a disease progression biomarker and a potential target for immunotherapy in Duchenne muscular dystrophy.

Pinto-Mariz F, Rodrigues Carvalho L, Prufer De Queiroz Campos Araujo A, De Mello W, Gonçalves Ribeiro M, Cunha Mdo C, Cabello PH, Riederer I, Negroni E, Desguerre I, Veras M, Yada E, Allenbach Y, Benveniste O, Voit T, Mouly V, Silva-Barbosa SD, Butler-Browne G, Savino W.

Skelet Muscle. 2015 Dec 10;5:45. doi: 10.1186/s13395-015-0066-2. eCollection 2015.


Cellular Therapies for Muscular Dystrophies: Frustrations and Clinical Successes.

Negroni E, Bigot A, Butler-Browne GS, Trollet C, Mouly V.

Hum Gene Ther. 2016 Feb;27(2):117-26. doi: 10.1089/hum.2015.139. Epub 2016 Jan 25. Review.


Post-transcriptional modulation of interleukin 8 by CNOT6L regulates skeletal muscle differentiation.

Polesskaya A, Pinna G, Sassi Y, Vandamme M, Bigot A, Mouly V, Morozova N, Harel-Bellan A, Degerny C.

Biochim Biophys Acta. 2016 Feb;1863(2):263-70. doi: 10.1016/j.bbamcr.2015.11.018. Epub 2015 Dec 1.


Age-Associated Methylation Suppresses SPRY1, Leading to a Failure of Re-quiescence and Loss of the Reserve Stem Cell Pool in Elderly Muscle.

Bigot A, Duddy WJ, Ouandaogo ZG, Negroni E, Mariot V, Ghimbovschi S, Harmon B, Wielgosik A, Loiseau C, Devaney J, Dumonceaux J, Butler-Browne G, Mouly V, Duguez S.

Cell Rep. 2015 Nov 10;13(6):1172-1182. doi: 10.1016/j.celrep.2015.09.067. Epub 2015 Oct 29.


Laminin therapy for the promotion of muscle regeneration.

Riederer I, Bonomo AC, Mouly V, Savino W.

FEBS Lett. 2015 Nov 14;589(22):3449-53. doi: 10.1016/j.febslet.2015.10.004. Epub 2015 Oct 10. Review.


Exon 32 Skipping of Dysferlin Rescues Membrane Repair in Patients' Cells.

Barthélémy F, Blouin C, Wein N, Mouly V, Courrier S, Dionnet E, Kergourlay V, Mathieu Y, Garcia L, Butler-Browne G, Lamaze C, Lévy N, Krahn M, Bartoli M.

J Neuromuscul Dis. 2015 Sep 2;2(3):281-290.


Changes in Communication between Muscle Stem Cells and their Environment with Aging.

Thorley M, Malatras A, Duddy W, Le Gall L, Mouly V, Butler Browne G, Duguez S.

J Neuromuscul Dis. 2015 Sep 2;2(3):205-217.


Engineering human 3D micromuscles with co-culture of fibroblasts and myoblasts.

Kalman B, Monge C, Bigot A, Mouly V, Picart C, Boudou T.

Comput Methods Biomech Biomed Engin. 2015;18 Suppl 1:1960-1. doi: 10.1080/10255842.2015.1069557. Epub 2015 Aug 21. No abstract available.


β-Arrestin scaffolds and signaling elements essential for the obestatin/GPR39 system that determine the myogenic program in human myoblast cells.

Santos-Zas I, Gurriarán-Rodríguez U, Cid-Díaz T, Figueroa G, González-Sánchez J, Bouzo-Lorenzo M, Mosteiro CS, Señarís J, Casanueva FF, Casabiell X, Gallego R, Pazos Y, Mouly V, Camiña JP.

Cell Mol Life Sci. 2016 Feb;73(3):617-35. doi: 10.1007/s00018-015-1994-z. Epub 2015 Jul 27.


Correlation between low FAT1 expression and early affected muscle in facioscapulohumeral muscular dystrophy.

Mariot V, Roche S, Hourdé C, Portilho D, Sacconi S, Puppo F, Duguez S, Rameau P, Caruso N, Delezoide AL, Desnuelle C, Bessières B, Collardeau S, Feasson L, Maisonobe T, Magdinier F, Helmbacher F, Butler-Browne G, Mouly V, Dumonceaux J.

Ann Neurol. 2015 Sep;78(3):387-400. doi: 10.1002/ana.24446. Epub 2015 Jul 3.


Mitochondrial dysfunction reveals the role of mRNA poly(A) tail regulation in oculopharyngeal muscular dystrophy pathogenesis.

Chartier A, Klein P, Pierson S, Barbezier N, Gidaro T, Casas F, Carberry S, Dowling P, Maynadier L, Bellec M, Oloko M, Jardel C, Moritz B, Dickson G, Mouly V, Ohlendieck K, Butler-Browne G, Trollet C, Simonelig M.

PLoS Genet. 2015 Mar 27;11(3):e1005092. doi: 10.1371/journal.pgen.1005092. eCollection 2015 Mar.


In silico screening based on predictive algorithms as a design tool for exon skipping oligonucleotides in Duchenne muscular dystrophy.

Echigoya Y, Mouly V, Garcia L, Yokota T, Duddy W.

PLoS One. 2015 Mar 27;10(3):e0120058. doi: 10.1371/journal.pone.0120058. eCollection 2015.


Cannabinoid receptor 1 and acute resistance exercise--In vivo and in vitro studies in human skeletal muscle.

Pekkala S, Wiklund P, Hulmi JJ, Pöllänen E, Marjomäki V, Munukka E, Pierre P, Mouly V, Mero A, Alén M, Cheng S.

Peptides. 2015 May;67:55-63. doi: 10.1016/j.peptides.2015.03.007. Epub 2015 Mar 18.


miRNA expression in control and FSHD fetal human muscle biopsies.

Portilho DM, Alves MR, Kratassiouk G, Roche S, Magdinier F, de Santana EC, Polesskaya A, Harel-Bellan A, Mouly V, Savino W, Butler-Browne G, Dumonceaux J.

PLoS One. 2015 Feb 18;10(2):e0116853. doi: 10.1371/journal.pone.0116853. eCollection 2015.


FUBP1: a new protagonist in splicing regulation of the DMD gene.

Miro J, Laaref AM, Rofidal V, Lagrafeuille R, Hem S, Thorel D, Méchin D, Mamchaoui K, Mouly V, Claustres M, Tuffery-Giraud S.

Nucleic Acids Res. 2015 Feb 27;43(4):2378-89. doi: 10.1093/nar/gkv086. Epub 2015 Feb 6.


Intramuscular sex steroid hormones are associated with skeletal muscle strength and power in women with different hormonal status.

Pöllänen E, Kangas R, Horttanainen M, Niskala P, Kaprio J, Butler-Browne G, Mouly V, Sipilä S, Kovanen V.

Aging Cell. 2015 Apr;14(2):236-48. doi: 10.1111/acel.12309. Epub 2015 Jan 20.


IL-6 and Akt are involved in muscular pathogenesis in myasthenia gravis.

Maurer M, Bougoin S, Feferman T, Frenkian M, Bismuth J, Mouly V, Clairac G, Tzartos S, Fadel E, Eymard B, Fuchs S, Souroujon MC, Berrih-Aknin S.

Acta Neuropathol Commun. 2015 Jan 15;3:1. doi: 10.1186/s40478-014-0179-6.


Invited review: Stem cells and muscle diseases: advances in cell therapy strategies.

Negroni E, Gidaro T, Bigot A, Butler-Browne GS, Mouly V, Trollet C.

Neuropathol Appl Neurobiol. 2015 Apr;41(3):270-87. doi: 10.1111/nan.12198. Review.


Predictive markers of clinical outcome in the GRMD dog model of Duchenne muscular dystrophy.

Barthélémy I, Pinto-Mariz F, Yada E, Desquilbet L, Savino W, Silva-Barbosa SD, Faussat AM, Mouly V, Voit T, Blot S, Butler-Browne G.

Dis Model Mech. 2014 Nov;7(11):1253-61. doi: 10.1242/dmm.016014. Epub 2014 Sep 26.


MMP-14 is necessary but not sufficient for invasion of three-dimensional collagen by human muscle satellite cells.

Lund DK, Mouly V, Cornelison DD.

Am J Physiol Cell Physiol. 2014 Jul 15;307(2):C140-9. doi: 10.1152/ajpcell.00032.2014. Epub 2014 Jun 4.

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