Université Evry Val d'Essonne

Thèmes :

• Physiopathologie des dystrophies des ceintures.
• Evaluation de stratégies thérapeutiques de type pharmacologique ou transfert de gène dans les dystrophies musculaires.
• Etiologie génétique des dystrophies musculaires.
• Construction et caractérisation de modèles animaux.
• Transfert de gènes non viral : Evaluation de copolymères amphiphiles, de peptides cationiques et de  polymères de cyclodextrines.
• Développement d’approches thérapeutiques pour la Dystrophie Musculaire de Duchenne : saut d’exon, production de facteurs circulants anti-inflammatoires, augmentation de la masse musculaire.
•  Intégration génomique des transgènes véhiculés par les vecteurs de thérapie génique.
• Modification du tropisme des vecteurs utilisés (dérivés de lentivirus).
• Utilisation des introns de groupe II comme vecteurs de thérapie génique.
• Imagerie du petit animal : mesure de l’activité de protéase par l’utilisation de bio-sondes FRET chez la souris vivante.
• Développement d’outils pour l’évaluation de la réparation membranaire.
• Etude de l’hétérogénéité d’une population cellulaire clonale : analyse quantitative d’image de cellules en culture et simulation informatique du comportement cellulaire.
• Quantification de l’expression de transgène et de l’intégration des vecteurs dans le cadre de thérapie génique.
• Développement d’outils d’analyse d’image.

 Dystrophies des Ceintures

Responsable : Isabelle RICHARD, DR2 CNRS

Administration et Vectorisation Physico-chimie

Responsable : Antoine KICHLER, CR1 CNRS

Intégration et Contexte Chromatinien

Responsable : Javier PEREA, Professeur d’Université

Imagerie et Cytométrie

Responsable : Daniel STOCKHOLM, Responsable d’études scientifiques

Membres :

• Daniel SCHERMAN, Directeur Recherche Exploratoire, DRCE CNRS
• Mauro MEZZINA, Animation scientifique & Projets européens, DR2 CNRS
• Isabelle PERISSON, Assistante de Direction 

Dystrophies des Ceintures

• Responsable : Isabelle RICHARD, DR2 CNRS
• Azzedine BENTAIB           
• Nathalie BOURG-ALIBERT
• Karine CHARTON
• Nathalie DANIELE
• Evelyne GICQUEL-ZOUIDA
• Florence LE ROY
• William LOSTAL
• Sylvie MARCHAND
• François MONJARET
• Jérôme POUPIOT
• Carinne ROUDAUT
• Shabahang SOHEILI
• Laurence SUEL-PETAT

Administration et Vectorisation Physico-chimie

• Responsable : Antoine KICHLER, CR1 CNRS
• Debborah ALIMI
• Smahane CHALABI
• Jérôme DENARD
• Xavier GERARD
• Simon GUIRAUD
• David ISRAELI
• Bérangère LANGLET
• Christian LEBORGNE
• Xavier LECLERC
• Gilles MOULAY
• Magdalena SOZANSKA
• Fédor SVINARTCHOUK
• Ludivine VIGNAUD

Intégration et Contexte Chromatinien

• Responsable : Javier PEREA, Professeur d’Université
• Sophie COUTANT
• Nathalie HOLIC-BARLET
• Stéphanie MATRAT
• Sophie MONIOT
• Thi My Anh NEILDEZ
• Andras PALDI
• Julie PASQUET
• Madeleine ZERBATO
• Sofia ZIYYAT-BENKHELIFA

Imagerie et Cytométrie

• Responsable : Daniel STOCKHOLM, Responsable d’études scientifiques
• Perrine BOREL
• Mehdi CHERRAR
• Peggy SANATINE

1.  Benchaouir R, Rameau P, Decraene C, Dreyfus P, Israeli D, Pietu G, Danos O, Garcia L. Evidence for a resident subset of cells with SP phenotype in the C2C12 myogenic line: a tool to explore muscle stem cell biology.Exp Cell Res 294 (1): 254-68, (2004).
2. Decraene C, Garcon L, Lacout C, Sabri S, Auffray C, Vainchenker W, Dumenil D, Pietu G, Svinartchuk F. Zyxin is up-regulated during megakaryocytic differentiation of human UT-7/c-mpl cells.Biochem Biophys Res Commun 318 (2): 439-43, (2004).
3. Deltour L, Vandamme J, Jouvenot Y, Duvillie B, Kelemen K, Schaerly P, Jami J, Paldi A. Differential expression and imprinting status of Ins1 and Ins2 genes in extraembryonic tissues of laboratory mice.Gene Expr Patterns 5 (2): 297-300, (2004).
4. Haack K, Cockrell AS, Ma H, Israeli D, Ho SN, McCown TJ, Kafri T. Transactivator and structurally optimized inducible lentiviral vectors.Mol Ther 10 (3): 585-96, (2004).
5. Imamura T, Neildez TM, Thenevin C, Paldi A. Essential role for poly (ADP-ribosyl)ation in mouse preimplantation development.BMC Mol Biol 5: 4, (2004).
6. Israeli D, Benchaouir R, Ziaei S, Rameau P, Gruszczynski C, Peltekian E, Danos O, Garcia L. FGF6 mediated expansion of a resident subset of cells with SP phenotype in the C2C12 myogenic line.J Cell Physiol 201 (3): 409-19, (2004).
7. Millot GA, Vainchenker W, Dumenil D, Svinarchuk F. Differential signalling of NH2-terminal flag-labelled thrombopoietin receptor activated by TPO or anti-FLAG antibodies.Cell Signal 16 (3): 355-63, (2004).
8. Taveau M, Stockholm D, Marchand S, Roudaut C, Le Bert M, Richard I. Bidirectional transcriptional activity of the Pgk1 promoter and transmission ratio distortion in Capn3-deficient mice.Genomics 84 (3): 592-5, (2004).
9. Bartoli M, Richard I. Calpains in muscle wasting.Int J Biochem Cell Biol 37 (10): 2115-33, (2005).
10. Charrier S, Stockholm D, Seye K, Opolon P, Taveau M, Gross DA, Bucher-Laurent S, Delenda C, Vainchenker W, Danos O, Galy A. A lentiviral vector encoding the human Wiskott-Aldrich syndrome protein corrects immune and cytoskeletal defects in WASP knockout mice.Gene Ther 12 (7): 597-606, (2005).
11. Collins CA, Partridge TA. Self-renewal of the adult skeletal muscle satellite cell.Cell Cycle 4 (10): 1338-41, (2005).
12. Decraene C, Benchaouir R, Dillies MA, Israeli D, Bortoli S, Rochon C, Rameau P, Pitaval A, Tronik-Le Roux D, Danos O, Gidrol X, Garcia L, Pietu G. Global transcriptional characterization of SP and MP cells from the myogenic C2C12 cell line: effect of FGF6.Physiol Genomics 23 (2): 132-49, (2005).
13. Garcon L, Lacout C, Svinartchouk F, Le Couedic JP, Villeval JL, Vainchenker W, Dumenil D. Gfi-1B plays a critical role in terminal differentiation of normal and transformed erythroid progenitor cells.Blood 105 (4): 1448-55, (2005).
14. Imamura T, Kerjean A, Heams T, Kupiec JJ, Thenevin C, Paldi A. Dynamic CpG and non-CpG methylation of the Peg1/Mest gene in the mouse oocyte and preimplantation embryo.J Biol Chem 280 (20): 20171-5, (2005).
15. Israeli D, Ziaei S, Gonin P, Garcia L. A proposal for the physiological significance of mdr1 and Bcrp1/Abcg2 gene expression in normal tissue regeneration and after cancer therapy.J Theor Biol 232 (1): 41-5, (2005).
16. Kichler A, Leborgne C, Savage P, Danos O. Cationic steroid antibiotics demonstrate DNA delivery properties.J. Control. Release 107 (1): 174-82, (2005).
17. Kichler A, Leborgne C, Danos O. Dilution of reporter gene with stuffer DNA does not alter the transfection efficiency of polyethylenimines.J. Gene Med 7 (11): 1459-67, (2005).
18. Kissenpfennig A, Henri S, Dubois B, Laplace-Builhe C, Perrin P, Romani N, Tripp CH, Douillard P, Leserman L, Kaiserlian D, Saeland S, Davoust J, Malissen B. Dynamics and function of Langerhans cells in vivo: dermal dendritic cells colonize lymph node areas distinct from slower migrating Langerhans cells.Immunity 22 (5): 643-54, (2005).
19. Kocaefe YC, Israeli D, Ozguc M, Danos O, Garcia L. Myogenic program induction in mature fat tissue (with MyoD expression).Exp Cell Res 308 (2): 300-8, (2005).
20. Lange S, Xiang F, Yakovenko A, Vihola A, Hackman P, Rostkova E, Kristensen J, Brandmeier B, Franzen G, Hedberg B, Gunnarsson LG, Hughes SM, Marchand S, Sejersen T, Richard I, Edstrom L, Ehler E, Udd B, Gautel M. The kinase domain of titin controls muscle gene expression and protein turnover.Science 308 (5728): 1599-603, (2005).
21. Richard I. The genetic and molecular bases of monogenic disorders affecting proteolytic systems.J Med Genet 42 (7): 529-39, (2005).
22. Stockholm D, Bartoli M, Sillon G, Bourg N, Davoust J, Richard I. Imaging calpain protease activity by multiphoton FRET in living mice.J Mol Biol 346 (1): 215-22, (2005).
23. Udd B, Vihola A, Sarparanta J, Richard I, Hackman P. Titinopathies and extension of the M-line mutation phenotype beyond distal myopathy and LGMD2J.Neurology 64 (4): 636-42, (2005).
24. Vendome J, Letard S, Martin F, Svinarchuk F, Dubreuil P, Auclair C, Le Bret M. Molecular modeling of wild-type and D816V c-Kit inhibition based on ATP-competitive binding of ellipticine derivatives to tyrosine kinases.J Med Chem 48 (20): 6194-201, (2005).
25. Young J, Morbois-Trabut L, Couzinet B, Lascols O, Dion E, Bereziat V, Feve B, Richard I, Capeau J, Chanson P, Vigouroux C. Type A insulin resistance syndrome revealing a novel lamin A mutation.Diabetes 54 (6): 1873-8, (2005).
26. Aranyi T, Paldi A. The constant variation: DNA methylation changes during preimplantation development.FEBS Lett 580 (28-29): 6521-6, (2006).
27. Balci B, Aurino S, Haliloglu G, Talim B, Erdem S, Akcoren Z, Tan E, Caglar M, Richard I, Nigro V, Topaloglu H, Dincer P. Calpain-3 mutations in Turkey.Eur J Pediatr 165 (5): 293-8, (2006).
28. Barde I, Zanta-Boussif MA, Paisant S, Leboeuf M, Rameau P, Delenda C, Danos O. Efficient control of gene expression in the hematopoietic system using a single Tet-on inducible lentiviral vector.Mol Ther 13 (2): 382-90, (2006).
29. Barkats M, Horellou P, Colin P, Millecamps S, Faucon-Biguet N, Mallet J. 1-methyl-4-phenylpyridinium neurotoxicity is attenuated by adenoviral gene transfer of human Cu/Zn superoxide dismutase.J Neurosci Res. 83 (2): 233-242, (2006).
30. Bartoli M, Bourg N, Stockholm D, Raynaud F, Delevacque A, Han Y, Borel P, Seddik K, Armande N, Richard I. A mouse model for monitoring calpain activity under physiological and pathological conditions.J Biol Chem 281 (51): 39672-80, (2006).
31. Bartoli M, Poupiot J, Goyenvalle A, Perez N, Garcia L, Danos O, Richard I. Noninvasive monitoring of therapeutic gene transfer in animal models of muscular dystrophies.Gene Ther 13 (1): 20-8, (2006).
32. Bartoli M, Roudaut C, Martin S, Fougerousse F, Suel L, Poupiot J, Gicquel E, Noulet F, Danos O, Richard I. Safety and efficacy of AAV-Mediated Calpain 3 Gene Transfer in a Mouse Model of Limb-Girdle Muscular Dystrophy Type 2A.Mol Ther 13 (2): 250-259, (2006).
33. Beroud C, Tuffery-Giraud S, Matsuo M, Hamroun D, Humbertclaude V, Monnier N, Moizard MP, Voelckel MA, Calemard LM, Boisseau P, Blayau M, Philippe C, Cossee M, Pages M, Rivier F, Danos O, Garcia L, Claustres M. Multiexon skipping leading to an artificial DMD protein lacking amino acids from exons 45 through 55 could rescue up to 63% of patients with Duchenne muscular dystrophy.Hum Mutat 28 (2): 196 - 202, (2006).
34. Blewitt ME, Vickaryous NK, Paldi A, Koseki H, Whitelaw E. Dynamic reprogramming of DNA methylation at an epigenetically sensitive allele in mice.PLoS Genet 2 (4): e49, (2006).
35. Brissault B, Leborgne C, Guis C, Danos O, Cheradame H, Kichler A. Linear topology confers in vivo gene transfer activity to polyethylenimines.Bioconjug Chem 17 (3): 759-65, (2006).
36. Brissault B, Kichler A, Leborgne C, Danos O, Cheradame H, Gau J, Auvray L, Guis C. Synthesis, characterization, and gene transfer application of poly(ethylene glycol-b-ethylenimine) with high molar mass polyamine block.Biomacromolecules 7 (10): 2863-70, (2006).
37. Burckbuchler V, Wintgens V, Lecomte S, Percot A, Leborgne C, Danos O, Kichler A, Amiel C. DNA compaction into new DNA vectors based on cyclodextrin polymer: SERS characterization.Biopolymers 81 (5): 360-70, (2006).
38. Cabarrocas J, Cassan C, Magnusson F, Piaggio E, Mars L, Derbinski J, Kyewski B, Gross DA, Salomon BL, Khazaie K, Saoudi A, Liblau RS. Foxp3+ CD25+ regulatory T cells specific for a neo-self-antigen develop at the double-positive thymic stage.Proc Natl Acad Sci U S A 103 (22): 8453-8, (2006).
39. Capron C, Lecluse Y, Kaushik AL, Foudi A, Lacout C, Sekkai D, Godin I, Albagli O, Poullion I, Svinartchouk F, Schanze E, Vainchenker W, Sablitzky F, Bennaceur-Griscelli A, Dumenil D. The SCL relative LYL-1 is required for fetal and adult hematopoietic stem cell function and B-cell differentiation.Blood 107 (12): 4678-86, (2006).
40. Cheradame H, Brissault B, Guis C, Guégan P, Pomel C, Kichler A, Gau J, Auvray L. Improvement of synthetic vectors for gene therapy using ring-opening cationic polymerization.Macromolecular Symposia 240 (166-177), (2006).
41. Danos O, Svinartchouk F. Dialysis-assisted two-dimensional gel electrophoresis.Electrophoresis 27 (17): 3475 - 3479, (2006).
42. Duguez S, Bartoli M, Richard I. Calpain 3: a key regulator of the sarcomere?Febs J 273 (15): 3427-36, (2006).
43. Duque S, Marais T, Danos O, Barkats M. Optimisation du transfert de gène dans la moelle épinière. In: Congrès de la Société Française de Thérapie Génique et Cellulaire (SFTGC), 19-20 Juin, 2006.
44. Gross DA, Chappert P, Leboeuf M, Monteilhet V, Van Wittenberghe L, Danos O, Davoust J. Simple conditioning with mono-specific CD4+CD25+ regulatory T cells for bone marrow engraftment and tolerance to multiple gene products.Blood 108 (6): 1841-1848, (2006).
45. Imamura T, Paldi A. Genomic imprinting: Springer-Verlag, (2006).
46. Izmiryan A, Cheraud Y, Khanamiryan L, Leterrier JF, Federici T, Peltekian E, Moura-Neto V, Paulin D, Li Z, Xue ZG. Different expression of synemin isoforms in glia and neurons during nervous system development.Glia 54 (3): 204-213, (2006).
47. Kichler A, Mason AJ, Bechinger B. Cationic amphipathic histidine-rich peptides for gene delivery.Biochim Biophys Acta 1758 (3): 301-7, (2006).
48. Krahn M, Bernard R, Pecheux C, Hammouda el H, Eymard B, Lopez de Munain A, Cobo AM, Romero N, Urtizberea A, Leturcq F, Levy N. Screening of the CAPN3 gene in patients with possible LGMD2A.Clin Genet 69 (5): 444-9, (2006).
49. Lagresle-Peyrou C, Yates F, Malassis-Seris M, Hue C, Morillon E, Garrigue A, Liu A, Hajdari P, Stockholm D, Danos O, Lemercier B, Gougeon ML, Rieux-Laucat F, de Villartay JP, Fischer A, Cavazzana-Calvo M. Long term immune reconstitution in RAG-1 deficient mice treated by retroviral gene therapy: a balance between efficiency and toxicity.Blood 107 (1): 63-72, (2006).
50. Mantovani J, Holic N, Martinez K, Danos O, Perea J. A high throughput method for genome-wide analysis of retroviral integration.Nucleic Acids Res 34 (19): e134, (2006).
51. Mason AJ, Martinez A, Glaubitz C, Danos O, Kichler A, Bechinger B. The antibiotic and DNA transfecting peptide LAH4 selectively associates with, and disorders, anionic lipids in mixed membranes.FASEB Journal 20 (2): 320-2, (2006).
52. Mason AJ, Gasnier C, Kichler A, Prévost G, Aunis D, Metz-Boutigue M-H, Bechinger B. Designed histidine-rich peptides show pH dependent antibiotic action against pathogenic bacteria.Antimicrob Agents Chemother. 2006 (50): 3305-3311, (2006).
53. Olivier A, Lauret E, Gonin P, Galy A. The Notch ligand delta-1 is a hematopoietic development co-factor for plasmacytoid dendritic cells.Blood 107 (7): 2694-701, (2006).
54. Olivier A, Jeanson-Leh L, Bouma G, Compagno D, Blondeau J, Seye K, Charrier S, Burns S, Thrasher A, Danos O, Vainchenker W, Galy A. A partial down-regulation of WASP is sufficient to inhibit podosome formation in dendritic cells.Mol Ther 13 (4): 729-37, (2006).
55. Paldi A. Clonage des mammifères par greffe du noyau somatique et remodelage épigénétique de la chromatine.Médicine de la reproduction 8: 1-5, (2006).
56. Paldi A. Empreinte génomique.Métabolismes Hormones Diabètes et Nutrition 10 (1): 6-9, (2006).
57. Partridge T. Disciplining the stem cell into myogenesis.N Engl J Med 354 (17): 1844-5, (2006).
58. Philippe S, Sarkis C, Barkats M, Mammeri H, Ladroue C, Petit C, Mallet J, Serguera C. Lentiviral vectors with a defective integrase allow efficient and sustained transgene expression in vitro and in vivo.Proc Natl Acad Sci U S A 103 (47): 17684-9, (2006).
59. Richard I. Limb-girdle muscular dystrophies. In: Ganten DR, K., ed. Encyclopedic Reference of Genomics and Proteomics: Springer-Verlag, (2006).
60. Smet-Nocca C, Paldi A, Benecke A. De l'épigénomique à l'émergence morphogénétique. In: Bourgine P, Lesne A, eds. Morphogenèse. L'origine des formes. Paris: Belin, (2006).
61. t'Hoen PA, van der Wees CG, Aartsma-Rus A, Turk R, Goyenvalle A, Danos O, Garcia L, van Ommen GJ, den Dunnen JT, van Deutekom JC. Gene expression profiling to monitor therapeutic and adverse effects of antisense therapies for Duchenne muscular dystrophy.Pharmacogenomics 7 (3): 281-97, (2006).
62. Veron P, Boutin S, Bernard J, Danos O, Davoust J, Masurier C. Efficient transduction of monocyte- and CD34(+)- derived Langerhans cells with lentiviral vectors in the absence of phenotypic and functional maturation.J Gene Med 8 (8): 951 - 961, (2006).
63. Bartoli M, Poupiot J, Vulin A, Fougerousse F, Arandel L, Daniele N, Roudaut C, Noulet F, Garcia L, Danos O, Richard I. AAV-mediated delivery of a mutated myostatin propeptide ameliorates calpain 3 but not alpha-sarcoglycan deficiency.Gene Ther 14 (9): 733-40, (2007).
64. Benchaouir R, Picot J, Greppo N, Rameau P, Stockholm D, Garcia L, Paldi A, Laplace-Builhe C. Combination of quantification and observation methods for study of "Side Population" cells in their "in vitro" microenvironment.Cytometry A 71 (4): 251-7, (2007).
65. Brissault B, Kichler A, Leborgne C, Jarroux N, Cheradame H, Guis C. Amphiphilic Poly[(propylene glycol)-block-(2-methyl-2-oxazoline)] Copolymers for Gene Transfer in Skeletal Muscle.ChemMedChem 2 (8): 1202-1207, (2007).
66. Carmignac V, Salih MA, Quijano-Roy S, Marchand S, Al Rayess MM, Mukhtar MM, Urtizberea JA, Labeit S, Guicheney P, Leturcq F, Gautel M, Fardeau M, Campbell KP, Richard I, Estournet B, Ferreiro A. C-terminal titin deletions cause a novel early-onset myopathy with fatal cardiomyopathy.Ann Neurol 61 (4): 340-51, (2007).
67. Daniele N, Richard I, Bartoli M. Ins and outs of therapy in limb girdle muscular dystrophies.Int J Biochem Cell Biol 39 (9): 1608-24, (2007).
68. Deburgrave N, Daoud F, Llense S, Barbot JC, Recan D, Peccate C, Burghes AH, Beroud C, Garcia L, Kaplan JC, Chelly J, Leturcq F. Protein- and mRNA-based phenotype-genotype correlations in DMD/BMD with point mutations and molecular basis for BMD with nonsense and frameshift mutations in the DMD gene.Hum Mutat 28 (2): 183-95, (2007).
69. Faure O, Graff-Dubois S, Alves PM, Cornet S, Duffour MT, Scardino A, Gross DA, Miconnet I, Salcedo M, Chouaib S, Lemonnier FA, Abastado JP, Kosmatopoulos K. Induction of multiple CD8+ T cell responses against the inducible Hsp70 employing an Hsp70 oligoepitope peptide.Oncol Rep 17 (3): 679-85, (2007).
70. Fougerousse F, Bartoli M, Poupiot J, Arandel L, Durand M, Guerchet N, Gicquel E, Danos O, Richard I. Phenotypic correction of alpha-Sarcoglycan Deficiency by Intra-arterial Injection of a Muscle-specific Serotype 1 rAAV Vector.Mol Ther 15 (1): 53-61, (2007).
71. Griggs R, Vihola A, Hackman P, Talvinen K, Haravuori H, Faulkner G, Eymard B, Richard I, Selcen D, Engel A, Carpen O, Udd B. Zaspopathy in a large classic late-onset distal myopathy family.Brain 130 (Pt 6): 1477-84, (2007).
72. Israeli D, Ziaei S, Gjata B, Benchaouir R, Rameau P, Marais T, Fukada S, Segawa M, Yamamoto H, Gonin P, Danos O, Garcia L. Expression of mdr1 is required for efficient long term regeneration of dystrophic muscle.Exp Cell Res 313 (11): 2438-50, (2007).
73. Jaiswal JK, Marlow G, Summerill G, Mahjneh I, Mueller S, Hill M, Miyake K, Haase H, Anderson LV, Richard I, Kiuru-Enari S, McNeil PL, Simon SM, Bashir R. Patients with a non-dysferlin Miyoshi myopathy have a novel membrane repair defect.Traffic 8 (1): 77-88, (2007).
74. Kichler A, Leborgne C, Danos O, Bechinger B. Characterization of the gene transfer process mediated by histidine-rich peptides.J Mol Med 85 (2): 191-201, (2007).
75. Mars LT, Bauer J, Gross DA, Bucciarelli F, Firat H, Hudrisier D, Lemonnier F, Kosmatopoulos K, Liblau RS. CD8 T Cell Responses to Myelin Oligodendrocyte Glycoprotein-Derived Peptides in Humanized HLA-A*0201-Transgenic Mice.J Immunol 179 (8): 5090-8, (2007).
76. Mason AJ, Bertani P, Moulay G, Marquette A, Perrone B, Drake AF, Kichler A, Bechinger B. Membrane interaction of chrysophsin-1, a histidine-rich antimicrobial peptide from red sea bream.Biochemistry 46 (51): 15175-87, (2007).
77. Mason AJ, Leborgne C, Moulay G, Martinez A, Danos O, Bechinger B, Kichler A. Optimising histidine rich peptides for efficient DNA delivery in the presence of serum.J Control Release 118 (1): 95-104, (2007).
78. Mason AJ, Bechinger B, Kichler A. Rational design of vector and antibiotic peptides using solid-state NMR.Mini Rev Med Chem 7 (5): 491-7, (2007).
79. Maystadt I, Rezsohazy R, Barkats M, Duque S, Vannuffel P, Remacle S, Lambert B, Najimi M, Sokal E, Munnich A, Viollet L, Verellen-Dumoulin C. The Nuclear Factor kappa B-Activator Gene PLEKHG5 Is Mutated in a Form of Autosomal Recessive Lower Motor Neuron Disease with Childhood Onset.Am J Hum Genet 81 (1): 67-76, (2007).
80. Milic A, Daniele N, Lochmuller H, Mora M, Comi GP, Moggio M, Noulet F, Walter MC, Morandi L, Poupiot J, Roudaut C, Bittner RE, Bartoli M, Richard I. A third of LGMD2A biopsies have normal calpain 3 proteolytic activity as determined by an in vitro assay.Neuromuscul Disord 17 (2): 148-56, (2007).
81. Paldi A. Expression stochastique des gènes. Génétiquement indéterminé: le vivant auto-organisé: Editions QUAE - INRA, (2007).
82. Prongidi-Fix L, Sugawara M, Bertani P, Raya J, Leborgne C, Kichler A, Bechinger B. Self-Promoted Cellular Uptake of Peptide/DNA Transfection Complexes.Biochemistry 46 (40): 11253-62, (2007).
83. Quenneville SP, Chapdelaine P, Skuk D, Paradis M, Goulet M, Rousseau J, Xiao X, Garcia L, Tremblay JP. Autologous transplantation of muscle precursor cells modified with a lentivirus for muscular dystrophy: human cells and primate models.Mol Ther 15 (2): 431-8, (2007).
84. Robin P, Fritsch L, Philipot O, Svinarchuk F, Ait-Si-Ali S. Post-translational modifications of histones H3 and H4 associated with the histone methyltransferases Suv39h1 and G9a.Genome Biol 8 (12): R270, (2007).
85. Stockholm D, Benchaouir R, Picot J, Rameau P, Neildez TM, Landini G, Laplace-Builhe C, Paldi A. The origin of phenotypic heterogeneity in a clonal cell population in vitro.PLoS ONE 2: e394, (2007).
86. Tang Y, Liu X, Zoltoski RK, Novak LA, Herrera RA, Richard I, Kuszak JR, Kumar NM. Age-related cataracts in alpha3Cx46-knockout mice are dependent on a calpain 3 isoform.Invest Ophthalmol Vis Sci 48 (6): 2685-94, (2007).
87. Vassilopoulos S, Brocard J, Garcia L, Marty I, Bouron A. Retrograde regulation of store-operated calcium channels by the ryanodine receptor-associated protein triadin 95 in rat skeletal myotubes.Cell Calcium 41 (2): 179-85, (2007).
88. Bacquet C, Imamura T, Gonzalez CA, Conejeros I, Kausel G, Neildez-Nguyen TM, Paldi A, Gallardo MH. Epigenetic processes in a tetraploid mammal.Mamm Genome 19 (6): 439-47, (2008).
89. Bartoli M, Gicquel E, Barrault L, Soheili T, Malissen M, Malissen B, Vincent-Lacaze N, Perez N, Udd B, Danos O, Richard I. Mannosidase I inhibition rescues the human alpha-sarcoglycan R77C recurrent mutation.Hum Mol Genet 17 (9): 1214-21, (2008).
90. Benayoun B, Baghdiguian S, Lajmanovich A, Bartoli M, Daniele N, Gicquel E, Bourg N, Raynaud F, Pasquier MA, Suel L, Lochmuller H, Lefranc G, Richard I. NF-kappaB-dependent expression of the antiapoptotic factor c-FLIP is regulated by calpain 3, the protein involved in limb-girdle muscular dystrophy type 2A.Faseb J 22 (5): 1521-9, (2008).
91. Benjelloun F, Garrigue A, Demerens-de Chappedelaine C, Soulas-Sprauel P, Malassis-Seris M, Stockholm D, Hauer J, Blondeau J, Riviere J, Lim A, Le Lorc'h M, Romana S, Brousse N, Paques F, Galy A, Charneau P, Fischer A, de Villartay JP, Cavazzana-Calvo M. Stable and functional lymphoid reconstitution in artemis-deficient mice following lentiviral artemis gene transfer into hematopoietic stem cells.Mol Ther 16 (8): 1490-9, (2008).
92. Burckbuchler V, Wintgens V, Leborgne C, Lecomte S, Leygue N, Scherman D, Kichler A, Amiel C. Development and characterization of new cyclodextrin polymer-based DNA delivery systems.Bioconjugate Chemistry: In Press, (2008).
93. Campana-Salort E, Krahn M, Bartoli M, Richard I, Pouget J, Levy N. Dystrophies musculaires des ceintures: stratégie diagnostique, bases moléculaires.Revue du rhumatisme 75: 142-150, (2008).
94. Chappert P, Leboeuf M, Rameau P, Stockholm D, Liblau R, Danos O, Davoust JM, Gross DA. Antigen-driven interactions with dendritic cells and expansion of Foxp3+ regulatory T cells occur in the absence of inflammatory signals.J Immunol 180 (1): 327-34, (2008).
95. Coulis G, Becila S, Herrera-Mendez CH, Sentandreu MA, Raynaud F, Richard I, Benyamin Y, Ouali A. Calpain 1 binding capacities of the N1-line region of titin are significantly enhanced by physiological concentrations of calcium.Biochemistry 47 (35): 9174-83, (2008).
96. Gonnet F, Bouazza B, Millot GA, Ziaei S, Garcia L, Butler-Browne GS, Mouly V, Tortajada J, Danos O, Svinartchouk F. Proteome analysis of differentiating human myoblasts by dialysis-assisted two-dimensional gel electrophoresis (DAGE).Proteomics 8 (2): 264-78, (2008).
97. Hackman P, Marchand S, Sarparanta J, Vihola A, Penisson-Besnier I, Eymard B, Pardal-Fernandez JM, Hammouda EH, Richard I, Illa I, Udd B. Truncating mutations in C-terminal titin may cause more severe tibial muscular dystrophy (TMD).Neuromuscul Disord: [Epub ahead of print], (2008).
98. Khoury M, Escriou V, Courties G, Galy A, Yao R, Largeau C, Scherman D, Jorgensen C, Apparailly F. Efficient suppression of murine arthritis by combined anticytokine small interfering RNA lipoplexes.Arthritis Rheum 58 (8): 2356-2367, (2008).
99. Krahn M, Béroud C, Labelle V, Nguyen K, Bernard R, Bassez G, Figarella-Branger D, Fernandez C, Bouvenot J, Richard I, Ollagnon-Roman E, Bevilacqua JA, Salvo E, Attarian S, Chapon F, Pellissier JF, Pouget J, Hammouda EH, Laforêt P, Urtizberea JA, Eymard B, Leturcq F, Lévy N. Analysis of the DYSF Mutational Spectrum in a large cohort of patients.Human Mutation: In Press, (2008).
100. Krahn M, Levy N, Daniele N, Richard I, Bartoli M. Nouvelles stratégies thérapeutiques des dystrophies musculaires.Revue du rhumatisme 75: 181-186, (2008).
101. Kwiatkowski BA, Kirillova I, Richard RE, Israeli D, Yablonka-Reuveni Z. FGFR4 and its novel splice form in myogenic cells: Interplay of glycosylation and tyrosine phosphorylation.J Cell Physiol 215 (3): 803-17, (2008).
102. Lorain S, Gross DA, Goyenvalle A, Danos O, Davoust J, Garcia L. Transient immunomodulation allows repeated injections of AAV1 and correction of muscular dystrophy in multiple muscles.Mol Ther 16 (3): 541-7, (2008).
103. Mason AJ, Moussaoui W, Abdelrahman T, Boukhari A, Bertani P, Marquette A, Shooshtarizaheh P, Moulay G, Boehm N, Guerold B, Sawers RJ, Kichler A, Metz-Boutigue MH, Candofi E, Prevost G, Bechinger B. Structural determinants of antimicrobial and antiplasmodial activity and selectivity in histidine rich amphipathic cationic peptides.J Biol Chem: [Epub ahead of print], (2008).
104. Neildez-Nguyen TM, Parisot A, Vignal C, Rameau P, Stockholm D, Picot J, Allo V, Le Bec C, Laplace C, Paldi A. Epigenetic gene expression noise and phenotypic diversification of clonal cell populations.Differentiation 76 (1): 33-40, (2008).

Réseau de recherche

• Pr. Burkhard Bechinger : Chimie Physique Moleculaire et Spectroscopie, Faculté de chimie, Université Louis Pasteur LC3-UMR 7177, Strasbourg, France
• Dr. Luc Lebeau : Laboratoire de Chimie Organique Appliquée, UMR 7175 - LC1, Faculté de Pharmacie, Illkirch, France
• Dr. Nathalie Mignet : Laboratoire de Pharmacologie Chimique et Génétique, U640 INSERM UMR 8151 CNRS, Faculté de Sciences Pharmaceutiques et Biologiques, Paris, France
• Pr. Catherine Amiel : Laboratoire de Recherche sur les Polymères, Thiais, France
• Dr. Otto Merten : Généthon, Evry, France
• G. Butler-Browne : Inserm U787 "group myologie"- Université Pierre et Marie Curie-Institut de Myologie, Paris, France
• Dr Olivier Laprevote : Laboratoire de Spectrochimie de masse de Gif-sur-Yvette, France
• Florence Gonnet : Laboratoire Analyse et Modélisation pour la Biologie et l'Environnement (LAMBE), Université d'Evry Val d'Essonne, Evry, France

Collaborations internationales

• Réseau européen CONSERT (2004-2008)
• Réseau européen PERSIST (2008-2012)
• Dr James Mason : Pharmaceutical Science Research Division King’s College London, Londres, Angleterre

Formations attachées

Ecole doctorale

« Des génomes aux Organismes », Université d’Evry-Val-d’Essonne

Masters attachés

• Biothérapies Tissulaires Cellulaires et Géniques
• Biologie Intégrative et Moléculaire

Thèses en cours

• Madeleine ZERBATO (Bourse Ministère de l’Education Nationale)
• Debborah ALIMI
• Gilles MOULAY
• Simon GUIRAUD
• Xavier GERARD
• Karine CHARTON
• William LOSTAL
• Shabahang SOHEILI

Responsable du laboratoire : Daniel SCHERMAN

Tél. : 01 69 47 28 58

Courriel : scherman@genethon.fr