Hubert group publications

25 publications relative to dystrophin since 2009


Delalande, O., Czogalla, A., Hubert, J.-F., Sikorski, A., and Le Rumeur, E. (2017) Dystrophin and spectrin, two highly dissimilar sisters of the same family, John Suire ed., Springer Books, Subcell Biochem 82, 373-403

Dos-Santos Morais, R., Delalande, O., Pérez, J., Mouret, L., Bondon, A., Martel, A., Appavou, M. S., Le Rumeur, E., Hubert, J.-F., and Combet, S. (2017) Contrast-matched isotropic bicelles: a versatile tool to specifically probe the solution structure of peripheral membrane proteins using SANS. Langmuir 33, 6572-80


Ameziane-Le Hir, S., Paboeuf, G., Tascon, C., Hubert, J.-F., Le Rumeur, E., Vié, V., and Raguénès-Nicol, C. (2016) Dystrophin Hot-Spot Mutants Leading to Becker Muscular Dystrophy Insert More Deeply into Membrane Models than the Native Protein. Biochemistry 82, 4018-26


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

Nicolas, A., Raguenes-Nicol, C., Ben Yaou, R., Ameziane-Le Hir, S., Cheron, A., Vie, V., Claustres, M., Leturcq, F., Delalande, O., Hubert, J. F., Tuffery-Giraud, S., Giudice, E., and Le Rumeur, E. (2015) Becker muscular dystrophy severity is linked to the structure of dystrophin. Hum Mol Genet 24, 1267-1279

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

Le Rumeur, E. (2015) Dystrophin and the two related genetic diseases, Duchenne and Becker muscular dystrophies. Bosn J Basic Med Sci 15(3), 14-20

Findlay, A. R., Wein, N., Kaminoh, Y., Taylor, L. E., Dunn, D. M., Mendell, J. R., King, W. M., Pestronk, A., Florence, J. M., Mathews, K. D., Finkel, R. S., Swoboda, K. J., Howard, M. T., Day, J. W., McDonald, C., Nicolas, A., Le Rumeur, E., Weiss, R. B., and Flanigan, K. M. (2015) Clinical phenotypes as predictors of the outcome of skipping around DMD exon 45. Ann Neurol 77(4), 668-674



Nicolas, A., Delalande, O., Hubert, J. F., and Le Rumeur, E. (2014) The spectrin family of proteins: A unique coiled-coil fold for various molecular surface properties. J Struct Biol 186, 392-401.

Molza, A.-E., Férey, N., Czjzek, M., Rumeur, E. L., Hubert, J.-F., Tek, A., Laurent, B., Baaden, M., and Delalande, O. (2014) Innovative Interactive Flexible Docking method for multi-scale reconstruction elucidates dystrophin molecular assembly. Faraday Discuss 169, 45-62

Ameziane-Le Hir, S., Raguénès-Nicol, C., Paboeuf, G., Nicolas, A., Chéron, A., Le Rumeur, E., and Vié, V. (2014) Cholesterol favors higher level of insertion and organization of spectrin-like repeat 16-21 of human dystrophin in membrane. Biochim Biophys Acta 1838, 1266-1273



Sarkis, J., Vié, V., Winder, S. J., Renault, A., Le Rumeur, E., and Hubert, J.-F. (2013) Resisting sarcolemmal rupture: dystrophin repeats increase membrane-actin stiffness. FASEB J 27, 359-367

Giudice, E., Molza, A.-E., Laurin, Y., Nicolas, A., Le Rumeur, E., and Delalande, O. (2013) Molecular clues about the dystrophin-neuronal nitric oxide synthase interaction: a theoretical approach. Biochemistry 52, 7777-7784



Sahni, N., Mangat, K., Le Rumeur, E., and Menhart, N. (2012) Exon edited dystrophin rods in the hinge 3 Region. Biochim Biophys Acta 1824, 1080-1089

Nicolas, A., Lucchetti-Miganeh, C., Ben Yaou, R., Kaplan, J. C., Chelly, J., Leturcq, F., Barloy-Hubler, F., and Le Rumeur, E. (2012) Assessment of the structural and functional impact of in-frame mutations of the DMD gene, using the tools included in the eDystrophin online database. Orphanet J Rare Dis 7(1), 45

Le Rumeur, E., Hubert, J.-F., and Winder, S. J. (2012) A new twist to coiled coil. FEBS letters 586, 2717-2722

Acsadi, G., Moore, S. A., Cheron, A., Delalande, O., Bennett, L., Kupsky, W., El-Baba, M., Le Rumeur, E., and Hubert, J. F. (2012) Novel mutation in spectrin-like repeat 1 of dystrophin central domain causes protein misfolding and mild Becker muscular dystrophy. J Biol Chem 287(22), 18153-18162



Sarkis, J., Hubert, J. F., Legrand, B., Robert, E., Cheron, A., Jardin, J., Hitti, E., Le Rumeur, E., and Vie, V. (2011) Spectrin-like repeats 11-15 of human dystrophin show adaptations to a lipidic environment. J Biol Chem 286(35), 30481-30491

Rouger, K., Larcher, T., Dubreil, L., Deschamps, J. Y., Le Guiner, C., Jouvion, G., Delorme, B., Lieubeau, B., Carlus, M., Fornasari, B., Theret, M., Orlando, P., Ledevin, M., Zuber, C., Leroux, I., Deleau, S., Guigand, L., Testault, I., Le Rumeur, E., Fiszman, M., and Cherel, Y. (2011) Systemic delivery of allogenic muscle stem cells induces long-term muscle repair and clinical efficacy in Duchenne muscular dystrophy dogs. Am J Pathol 179(5), 2501-2518

Legrand, B., Giudice, E., Nicolas, A., Delalande, O., and Le Rumeur, E. (2011) Computational Study of the Human Dystrophin Repeats: Interaction Properties and Molecular Dynamics. Plos One 6(8), e23819



Vié, V., Legardinier, S., Chieze, L., Le Bihan, O., Qin, Y., Sarkis, J., Hubert, J. F., Renault, A., Desbat, B., and Le Rumeur, E. (2010) Specific anchoring modes of two distinct dystrophin rod sub-domains interacting in phospholipid Langmuir films studied by atomic force microscopy and PM-IRRAS. Biochim Biophys Acta 1798(8), 1503-1511

Le Rumeur, E., Winder, S. J., and Hubert, J. F. (2010) Dystrophin: More than just the sum of its parts. Biochim Biophys Acta 1804(9), 1713-1722

Daval, S., Rocher, C., Cherel, Y., and Le Rumeur, E. (2010) Several dystrophin-glycoprotein complex members are present in crude surface membranes but they are sodium dodecyl sulphate invisible in KCl-washed microsomes from mdx mouse muscle. Cell Mol Biol Lett 15(1), 134-152



Legardinier, S., Raguénès-Nicol, C., Tascon, C., Rocher, C., Hardy, S., Hubert, J. F., and Le Rumeur, E. (2009) Mapping of the lipid-binding and stability properties of the central rod domain of human dystrophin. J Mol Biol 389, 546-558

Legardinier, S., Legrand, B., Raguénès-Nicol, C., Bondon, A., Hardy, S., Tascon, C., Le Rumeur, E., and Hubert, J. F. (2009) A two amino-acid mutation encountered in Duchenne muscular dystrophy decreases stability of the R23 spectrin-like repeat of dystrophin. J Biol Chem 284(13), 8822-8832


Other topics

Fouqué, A., Delalande, O., Jean, M., Castellano, R., Josselin, E., Malleter, M., Shoji, K. F., Hung, M. D., Rampanarivo, H., Collette, Y., van de Weghe, P. & Legembre, P., A novel covalent mTOR inhibitor, DHM25, shows in vivo antitumor activity against triple-negative breast cancer cells, J. Med. Chem., 58(16), 6559-73 (2015)

Paquet, F., Delalande, O., Goffinont, S., Culard, F., Loth, K., Asseline, U., Castaing, B. & Landon, C., Atypical DNA-protein complex of the architectural monomeric protein MC1 form Euryarchaea, PloS ONE, 9, e88809 (2014)

Simon, M., Metzinger, V., Chevance, S., Delalande, O., & Bondon, A., Versatility of non-native forms of human cytochrome c: pH and micellar concentration dependence, J. Biol. Inorg. Chem., 18(1), 27-38 (2013)

Da Costa, G., Bondon, A., Delalande, O., Mouret, L., & Monti, J.-P., Elucidation by NMR solution of neurotensin in Small Unilamellar Vesicle environment: molecular surveys for neurotensin receptor recognition, J. Biol. Struct. Dyn., 31(8), 809-17 (2013)

Ismed, F., Lohézic-Le Dévéhat, F., Delalande, O., Sinbandhit, S., Bakhtiar, A., & Boustie, J., Lobarin, a bioactive diphenyl ether from Stereocaulon massartianum, a Sumatran lichen, Fitoterapia, 83(8), 1693-98 (2012)

Garcin, P., Delalande, O., Zhang, J.-Y., Cassier-Chauvat, C., Chauvat, F., & Boulard, Y., A transcriptional-switch model for Slr1738-controlled gene expression in the cyanobacterium Synechocystis, BMC Sturct. Biol., 12 (1) (2012)

Delalande, O., Sacquin-Mora, S., & Baaden, M., Conformational substates of Mycobacterium Tuberculosis xguanylate kinase reveal structural fluctuations linked to enzymatic function, Biophys. J., 101, 1440-1449 (2011)

Delalande, O., Desvaux, H., Godat, E., Valleix, A., Junot, C., Labarre, J., & Boulard, Y., Cadmium-glutathione solution structures provide new insights into heavy metal detoxification, FEBS J., 277(24), 5086-96 (2010)

Sacquin-Mora, S., Delalande, O., & Baaden, M., Functional modes and residue flexibility control the anisotropic response of Guanylate Kinase to mechanical stress, Biophys. J., 99, 3412-3419 (2010)

Saladin, A., Amourda, C., Poulain, P., Férey, N., Baaden, M., Zacharias, M., Delalande, O., & Prévost, C., Modeling the early stage of DNA sequence recognition within RecA nucleoprotein filaments, Nucleic Acids Res., 38, 6313-23 (2010)

Delalande, O., Férey, N., Laurent, B., Gueroult, M., Hartmann, B., & Baaden, M., Multi-resolution approach for interactively locating functionally linked ion binding sites by steering small molecules into electrostatic potential maps using a haptic device,  Pacific Symposium on Biocomputing (PSB 2010), 205-215 (2010)

Delalande, O., Férey, N., Grasseau, G. & Baaden, M., Complex molecular assemblies at hand via interactive simulations, J. Comp. Chem., 30, 2375-87 (2009)