Jean-François Hubert group

Our research project aims at better understanding the molecular basis for muscle membrane scaffolding in normal and DMD or BMD cells by in vitro and in silico structure to function approaches. We first showed that dystrophin interacts strongly with membrane phospholipids, a binding which is involved in the resistance to sarcolemmal rupture during muscle contraction (Sarkis et al., 2013).

Major publications

  • Dystrophin's central domain forms a complex filament that becomes disorganized by in-frame deletions.
    Delalande O, Molza AE, Dos Santos Morais R, Chéron A, Pollet E, Raguénès-Nicol C, Tascon C, Giudice E, Guilbaud M, Nicolas A, Bondon A, Leturcq F, Ferey N,
     Baaden M, Perez J, Roblin P, Pietri-Rouxel F, Hubert JF, Czjzek M, Le Rumeur E
    J Biol Chem 293, 6637-6646, 2018, PMID: 29535188

    Contrast-Matched Isotropic Bicelles: A Versatile Tool to Specifically Probe the Solution Structure of Peripheral Membrane Proteins Using SANS.
    Dos Santos Morais R, Delalande O, Perez J, Mouret L, Bondon A, Martel A, Appavou MS, Le Rumeur E, Hubert JF, Combet S
    Langmuir 33(26), 6572-6580, 2017, PMID: 28581294

    Abnormal splicing switch of DMD's penultimate exon compromises muscle fibre maintenance in myotonic dystrophy.
    Rau F, Laine J, Ramanoudjame L, Ferry A, Arandel L, Delalande O, Jollet A, Dingli F, Lee KY, Peccate C, Lorain S, Kabashi E, Athanasopoulos T, Koo T, Loew D, Swanson MS, Le Rumeur E, Dickson G, Allamand V, Marie J, Furling D
    Nat Commun 6, 7205, 2015, PMID: 26018658

    Becker muscular dystrophy severity is linked to the structure of dystrophin.
    Nicolas A, Raguenes-Nicol C, Ben Yaou R, Ameziane-Le Hir S, Chéron A, Vié V, Claustres M, Leturcq F, Delalande O, Hubert JF, Tuffery-Giraud S, Giudice E, Le Rumeur E
    Hum Mol Genet 24, 1267-1279, 2015, PMID: 25348330

    Structural Basis of Neuronal Nitric-Oxide Synthase Interaction with Dystrophin Repeats 16 and 17.
    Molza AE, Mangat K, Le Rumeur E, Hubert JF, Menhart N, Delalande O
    J Biol Chem 290(49), 29531-41, 2015, PMID: 26378238

    Clinical phenotypes as predictors of the outcome of skipping around DMD exon 45.
    Findlay AR, Wein N, Kaminoh Y, Taylor LE, Dunn DM, Mendell JR, King WM, Pestronk A, Florence JM, Mathews KD, Finkel RS, Swoboda KJ, Howard MT, Day JW, McDonald C, Nicolas A, Le Rumeur E, Weiss RB, Flanigan KM
    Ann Neurol 77(4), 668-674, 2015, PMID: 25612243

    Resisting sarcolemmal rupture: dystrophin repeats increase membrane-actin stiffness.
    Sarkis J, Vié V, Winder SJ, Renault A, Le Rumeur E, Hubert JF
    FASEB J 27, 359-367, 2013, PMID: 23033320