R. E. Hershberger, S. , and J. D. , Update 2011: Clinical and Genetic Issues in Familial Dilated Cardiomyopathy, Journal of the American College of Cardiology, vol.57, issue.16, pp.1641-1649, 2011.
DOI : 10.1016/j.jacc.2011.01.015

B. Yaou, R. Gueneau, L. Demay, L. Stora, S. Chikhaoui et al., Heart involvement in lamin A/C related diseases, Arch. Mal. Coeur Vaiss, vol.99, pp.848-855, 2006.

D. Fatkin, C. Macrae, T. Sasaki, M. R. Wolff, M. Porcu et al., Missense Mutations in the Rod Domain of the Lamin A/C Gene as Causes of Dilated Cardiomyopathy and Conduction-System Disease, New England Journal of Medicine, vol.341, issue.23, pp.1715-1724, 1999.
DOI : 10.1056/NEJM199912023412302

H. J. Worman and G. Bonne, ???Laminopathies???: A wide spectrum of human diseases, Experimental Cell Research, vol.313, issue.10, pp.2121-2133, 2007.
DOI : 10.1016/j.yexcr.2007.03.028

S. Quijano-roy, B. Mbieleu, C. G. Bonnemann, P. Y. Jeannet, J. Colomer et al., mutations cause a new form of congenital muscular dystrophy, Annals of Neurology, vol.30, issue.2, pp.177-186, 2008.
DOI : 10.1002/ana.21417

G. Bonne, D. Barletta, M. R. Varnous, S. Becane, H. M. Hammouda et al., Mutations in the gene encoding lamin A/C cause autosomal dominant Emery-Dreifuss muscular dystrophy, Nature Genetics, vol.21, issue.3, pp.285-288, 1999.
DOI : 10.1038/6799

A. Muchir, G. Bonne, A. J. Van-der-kooi, M. Van-meegen, F. Baas et al., Identification of mutations in the gene encoding lamins A/C in autosomal dominant limb girdle muscular dystrophy with atrioventricular conduction disturbances (LGMD1B), Human Molecular Genetics, vol.9, issue.9, pp.1453-1459, 2000.
DOI : 10.1093/hmg/9.9.1453

F. Lin and H. J. Worman, Structural organization of the human gene encoding nuclear lamin A and nuclear lamin C, J. Biol. Chem, vol.268, pp.16321-16326, 1993.

J. L. Broers, F. C. Ramaekers, G. Bonne, R. B. Yaou, and C. J. Hutchison, Nuclear Lamins: Laminopathies and Their Role in Premature Ageing, Physiological Reviews, vol.86, issue.3, pp.967-1008, 2006.
DOI : 10.1152/physrev.00047.2005

URL : http://repository.tue.nl/647298

E. C. Schirmer and R. Foisner, Proteins that associate with lamins: Many faces, many functions, Experimental Cell Research, vol.313, issue.10, pp.2167-2179, 2007.
DOI : 10.1016/j.yexcr.2007.03.012

C. Favreau, E. Dubosclard, C. Ostlund, C. Vigouroux, J. Capeau et al., Expression of Lamin A Mutated in the Carboxyl-Terminal Tail Generates an Aberrant Nuclear Phenotype Similar to That Observed in Cells from Patients with Dunnigan-Type Partial Lipodystrophy and Emery-Dreifuss Muscular Dystrophy, Experimental Cell Research, vol.282, issue.1, pp.14-23, 2003.
DOI : 10.1006/excr.2002.5669

Y. Wang, A. J. Herron, and H. J. Worman, Pathology and nuclear abnormalities in hearts of transgenic mice expressing M371K lamin A encoded by an LMNA mutation causing Emery-Dreifuss muscular dystrophy, Human Molecular Genetics, vol.15, issue.16, pp.2479-2489, 2006.
DOI : 10.1093/hmg/ddl170

V. Nikolova, C. Leimena, A. C. Mcmahon, J. C. Tan, S. Chandar et al., Defects in nuclear structure and function promote dilated cardiomyopathy in lamin A/C???deficient mice, Journal of Clinical Investigation, vol.113, issue.3, pp.357-369, 2004.
DOI : 10.1172/JCI200419448

C. M. Wolf, L. Wang, R. Alcalai, A. Pizard, P. G. Burgon et al., Lamin A/C haploinsufficiency causes dilated cardiomyopathy and apoptosis-triggered cardiac conduction system disease, Journal of Molecular and Cellular Cardiology, vol.44, issue.2, pp.293-303, 2008.
DOI : 10.1016/j.yjmcc.2007.11.008

A. T. Bertrand, L. Renou, A. Papadopoulos, M. Beuvin, E. Lacene et al., DelK32-lamin A/C has abnormal location and induces incomplete tissue maturation and severe metabolic defects leading to premature death, Human Molecular Genetics, vol.21, issue.5, pp.1037-1048, 2012.
DOI : 10.1093/hmg/ddr534

URL : https://hal.archives-ouvertes.fr/hal-00672148

K. L. Rock, C. Gramm, L. Rothstein, K. Clark, R. Stein et al., Inhibitors of the proteasome block the degradation of most cell proteins and the generation of peptides presented on MHC class I molecules, Cell, vol.78, issue.5, pp.761-771, 1994.
DOI : 10.1016/S0092-8674(94)90462-6

K. Lindsten, V. Menendez-benito, M. G. Masucci, and N. P. Dantuma, A transgenic mouse model of the ubiquitin/proteasome system, Nature Biotechnology, vol.21, issue.8, pp.897-902, 2003.
DOI : 10.1038/nbt851

A. Hansen, A. Eder, M. Bonstrup, M. Flato, M. Mewe et al., Development of a Drug Screening Platform Based on Engineered Heart Tissue, Circulation Research, vol.107, issue.1, pp.35-44, 2010.
DOI : 10.1161/CIRCRESAHA.109.211458

H. M. Becane, G. Bonne, S. Varnous, A. Muchir, V. Ortega et al., High Incidence of Sudden Death with Conduction System and Myocardial Disease Due to Lamins A and C Gene Mutation, Pacing and Clinical Electrophysiology, vol.23, issue.11, pp.1661-1666, 2000.
DOI : 10.1046/j.1460-9592.2000.01661.x

G. Suurmeijer, A. J. White, S. J. Den-dunnen, J. T. Te-meerman, G. J. Vos et al., Severe myocardial fibrosis caused by a deletion of the 5' end of the lamin A/C gene, J. Am. Coll. Cardiol, vol.49, pp.2430-2439, 2007.

M. Pasotti, C. Klersy, A. Pilotto, N. Marziliano, C. Rapezzi et al., Long-Term Outcome and Risk Stratification in Dilated Cardiolaminopathies, Journal of the American College of Cardiology, vol.52, issue.15, pp.1250-1260, 2008.
DOI : 10.1016/j.jacc.2008.06.044

P. Gupta, Z. T. Bilinska, N. Sylvius, E. Boudreau, J. P. Veinot et al., Genetic and ultrastructural studies in dilated cardiomyopathy patients: a large deletion in the lamin A/C gene is associated with cardiomyocyte nuclear envelope disruption, Basic Research in Cardiology, vol.338, issue.3, pp.365-377, 2010.
DOI : 10.1007/s00395-010-0085-4

D. Jahn, S. Schramm, M. Schnolzer, C. J. Heilmann, C. G. De-koster et al., mouse line, mouse line: Implications for the understanding of laminopathies, pp.463-474, 2012.
DOI : 10.1038/nmeth0607-465

S. Schlossarek and L. Carrier, The ubiquitin???proteasome system in cardiomyopathies, Current Opinion in Cardiology, vol.26, issue.3, pp.190-195, 2011.
DOI : 10.1097/HCO.0b013e32834598fe

P. Vicart, A. Caron, P. Guicheney, Z. Li, M. C. Prevost et al., A missense mutation in the alphaBcrystallin chaperone gene causes a desmin-related myopathy, Nature Genetics, vol.20, issue.1, pp.92-95, 1998.
DOI : 10.1038/1765

P. Richard, P. Charron, L. Carrier, C. Ledeuil, T. Cheav et al., Hypertrophic cardiomyopathy. Distribution of disease genes, spectrum of mutations, and implications for a molecular diagnosis strategy, ACC Current Journal Review, vol.12, issue.4, pp.2227-2232, 2003.
DOI : 10.1016/S1062-1458(03)00298-8

URL : https://hal.archives-ouvertes.fr/hal-01110561

N. Vignier, S. Schlossarek, B. Fraysse, G. Mearini, E. Kramer et al., Nonsense-Mediated mRNA Decay and Ubiquitin-Proteasome System Regulate Cardiac Myosin-Binding Protein C Mutant Levels in Cardiomyopathic Mice, Circulation Research, vol.105, issue.3, pp.239-248
DOI : 10.1161/CIRCRESAHA.109.201251

A. Sanbe, H. Osinska, J. E. Saffitz, C. G. Glabe, R. Kayed et al., Desmin-related cardiomyopathy in transgenic mice: A cardiac amyloidosis, Proceedings of the National Academy of Sciences, vol.101, issue.27, pp.10132-10136, 2004.
DOI : 10.1073/pnas.0401900101

S. Kandert, M. Wehnert, C. R. Muller, B. Buendia, and M. C. Dabauvalle, Impaired nuclear functions lead to increased senescence and inefficient differentiation in human myoblasts with a dominant p.R545C mutation in the LMNA gene, European Journal of Cell Biology, vol.88, issue.10, 2009.
DOI : 10.1016/j.ejcb.2009.06.002

D. M. Eble, M. L. Spragia, A. G. Ferguson, and A. M. Samarel, Sarcomeric myosin heavy chain is degraded by the proteasome, Cell & Tissue Research, vol.296, issue.3, pp.541-548, 1999.
DOI : 10.1007/s004410051315

S. K. Shenoy, P. H. Mcdonald, T. A. Kohout, and R. J. Lefkowitz, Regulation of Receptor Fate by Ubiquitination of Activated beta 2-Adrenergic Receptor and beta -Arrestin, Science, vol.294, issue.5545, pp.1307-1313, 2001.
DOI : 10.1126/science.1063866

P. Penela, A. Ruiz-gomez, J. G. Castano, M. , and F. Jr, Degradation of the G Protein-coupled Receptor Kinase 2 by the Proteasome Pathway, Journal of Biological Chemistry, vol.273, issue.52, pp.35238-35244, 1998.
DOI : 10.1074/jbc.273.52.35238

B. Skaug, X. Jiang, C. , and Z. J. , The Role of Ubiquitin in NF-??B Regulatory Pathways, Annual Review of Biochemistry, vol.78, issue.1, pp.769-796, 2009.
DOI : 10.1146/annurev.biochem.78.070907.102750

H. Aberle, A. Bauer, J. Stappert, A. Kispert, and R. Kemler, ??-catenin is a target for the ubiquitin???proteasome pathway, The EMBO Journal, vol.16, issue.13, pp.3797-3804, 1997.
DOI : 10.1093/emboj/16.13.3797

E. J. Birks, N. Latif, K. Enesa, T. Folkvang, A. Luong-le et al., Elevated p53 expression is associated with dysregulation of the ubiquitin-proteasome system in dilated cardiomyopathy, Cardiovascular Research, vol.79, issue.3, pp.472-480, 2008.
DOI : 10.1093/cvr/cvn083

E. M. Bank, K. Ben-harush, N. Wiesel-motiuk, R. Barkan, N. Feinstein et al., A laminopathic mutation disrupting lamin filament assembly causes disease-like phenotypes in Caenorhabditis elegans, Molecular Biology of the Cell, vol.22, issue.15, pp.2716-2728, 2011.
DOI : 10.1091/mbc.E11-01-0064

N. Tanaka, N. Dalton, L. Mao, H. A. Rockman, K. L. Peterson et al., Transthoracic Echocardiography in Models of Cardiac Disease in the Mouse, Circulation, vol.94, issue.5, pp.1109-1117, 1996.
DOI : 10.1161/01.CIR.94.5.1109

C. Riviere, O. Danos, and A. M. Douar, Long-term expression and repeated administration of AAV type 1, 2 and 5 vectors in skeletal muscle of immunocompetent adult mice, Gene Therapy, vol.1, issue.17, pp.1300-1308, 2006.
DOI : 10.1038/sj.gt.3302766

A. Shevchenko, H. Tomas, J. Havlis, J. V. Olsen, and M. Mann, In-gel digestion for mass spectrometric characterization of proteins and proteomes, Nature Protocols, vol.5, issue.6, 2006.
DOI : 10.1038/nprot.2006.468

J. Rappsilber, M. Mann, and Y. Ishihama, Protocol for micro-purification, enrichment, pre-fractionation and storage of peptides for proteomics using StageTips, Nature Protocols, vol.4, issue.8, 2007.
DOI : 10.1038/nmeth1005

J. Cox and M. Mann, MaxQuant enables high peptide identification rates, individualized p.p.b.-range mass accuracies and proteome-wide protein quantification, Nature Biotechnology, vol.7, issue.12, 2008.
DOI : 10.1038/nprot.2007.261

C. A. Luber, J. Cox, H. Lauterbach, B. Fancke, M. Selbach et al., Quantitative Proteomics Reveals Subset-Specific Viral Recognition in Dendritic Cells, Immunity, vol.32, issue.2, pp.279-289, 2010.
DOI : 10.1016/j.immuni.2010.01.013

J. Hansen, T. J. Corydon, J. Palmfeldt, A. Durr, B. Fontaine et al., Decreased expression of the mitochondrial matrix proteases Lon and ClpP in cells from a patient with hereditary spastic paraplegia (SPG13), Neuroscience, vol.153, issue.2, pp.474-482, 2008.
DOI : 10.1016/j.neuroscience.2008.01.070

T. Arimura, A. Helbling-leclerc, C. Massart, S. Varnous, F. Niel et al., Mouse model carrying H222P-Lmna mutation develops muscular dystrophy and dilated cardiomyopathy similar to human striated muscle laminopathies, Human Molecular Genetics, vol.14, issue.1, pp.155-169, 2005.
DOI : 10.1093/hmg/ddi017

URL : https://hal.archives-ouvertes.fr/hal-00165763

*. Pwd, . Lvm, and . Bw, 49 ** IVSs (mm) 1.06 0.22 1.10 0.19 0.93 0

*. Pwd, . Lvm, and . Bw, 50 ** IVSs (mm) 0