Published in Hum Mol Genet on November 17, 2004
"Laminopathies": a wide spectrum of human diseases. Exp Cell Res (2007) 3.46
Blocking protein farnesyltransferase improves nuclear blebbing in mouse fibroblasts with a targeted Hutchinson-Gilford progeria syndrome mutation. Proc Natl Acad Sci U S A (2005) 3.30
Laminopathies and the long strange trip from basic cell biology to therapy. J Clin Invest (2009) 2.94
Nuclear lamin A/C deficiency induces defects in cell mechanics, polarization, and migration. Biophys J (2007) 2.51
A farnesyltransferase inhibitor improves disease phenotypes in mice with a Hutchinson-Gilford progeria syndrome mutation. J Clin Invest (2006) 2.39
Prelamin A and lamin A appear to be dispensable in the nuclear lamina. J Clin Invest (2006) 2.23
Bringing KASH under the SUN: the many faces of nucleo-cytoskeletal connections. J Cell Biol (2009) 2.16
Activation of MAPK pathways links LMNA mutations to cardiomyopathy in Emery-Dreifuss muscular dystrophy. J Clin Invest (2007) 1.87
When lamins go bad: nuclear structure and disease. Cell (2013) 1.84
Disruption of nesprin-1 produces an Emery Dreifuss muscular dystrophy-like phenotype in mice. Hum Mol Genet (2008) 1.83
Lamin A/C and emerin are critical for skeletal muscle satellite cell differentiation. Genes Dev (2006) 1.73
Nuclear lamins: key regulators of nuclear structure and activities. J Cell Mol Med (2009) 1.70
Inhibition of extracellular signal-regulated kinase signaling to prevent cardiomyopathy caused by mutation in the gene encoding A-type lamins. Hum Mol Genet (2008) 1.64
Nesprin-1 mutations in human and murine cardiomyopathy. J Mol Cell Cardiol (2009) 1.48
Lamin A/C haploinsufficiency causes dilated cardiomyopathy and apoptosis-triggered cardiac conduction system disease. J Mol Cell Cardiol (2007) 1.42
Progerin elicits disease phenotypes of progeria in mice whether or not it is farnesylated. J Clin Invest (2008) 1.40
Mouse models of the laminopathies. Exp Cell Res (2007) 1.40
Lamina-associated polypeptide 2alpha regulates cell cycle progression and differentiation via the retinoblastoma-E2F pathway. J Cell Biol (2006) 1.38
LINC complexes in health and disease. Nucleus (2011) 1.37
Lamin A/C-mediated neuromuscular junction defects in Emery-Dreifuss muscular dystrophy. J Cell Biol (2009) 1.36
Lamins at a glance. J Cell Sci (2012) 1.30
An accumulation of non-farnesylated prelamin A causes cardiomyopathy but not progeria. Hum Mol Genet (2010) 1.26
The tight junction protein CAR regulates cardiac conduction and cell-cell communication. J Exp Med (2008) 1.23
Mitogen-activated protein kinase inhibitors improve heart function and prevent fibrosis in cardiomyopathy caused by mutation in lamin A/C gene. Circulation (2010) 1.21
Temsirolimus activates autophagy and ameliorates cardiomyopathy caused by lamin A/C gene mutation. Sci Transl Med (2012) 1.19
Dystromirs as serum biomarkers for monitoring the disease severity in Duchenne muscular Dystrophy. PLoS One (2013) 1.16
Dependence of diffusional mobility of integral inner nuclear membrane proteins on A-type lamins. Biochemistry (2006) 1.12
The cardiac conduction system. Circulation (2011) 1.11
A-type lamin complexes and regenerative potential: a step towards understanding laminopathic diseases? Histochem Cell Biol (2005) 1.09
Emerin-lacking mice show minimal motor and cardiac dysfunctions with nuclear-associated vacuoles. Am J Pathol (2006) 1.05
Epidermal expression of the truncated prelamin A causing Hutchinson-Gilford progeria syndrome: effects on keratinocytes, hair and skin. Hum Mol Genet (2008) 1.03
Distinctive serum miRNA profile in mouse models of striated muscular pathologies. PLoS One (2013) 1.03
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Investigating the purpose of prelamin A processing. Nucleus (2011) 1.01
LMNA cardiomyopathy: cell biology and genetics meet clinical medicine. Dis Model Mech (2011) 1.01
Molecular genetic analysis of the nested Drosophila melanogaster lamin C gene. Genetics (2005) 1.01
Modeling of lamin A/C mutation premature cardiac aging using patient‐specific induced pluripotent stem cells. Aging (Albany NY) (2012) 1.00
Cellular mechanosensing: getting to the nucleus of it all. Prog Biophys Mol Biol (2014) 0.99
Attenuated hypertrophic response to pressure overload in a lamin A/C haploinsufficiency mouse. J Mol Cell Cardiol (2009) 0.99
Pharmacological inhibition of c-Jun N-terminal kinase signaling prevents cardiomyopathy caused by mutation in LMNA gene. Biochim Biophys Acta (2010) 0.98
Nuclear damages and oxidative stress: new perspectives for laminopathies. Eur J Histochem (2012) 0.93
The intricacy of nuclear membrane dynamics during nucleophagy. Nucleus (2010) 0.92
Tissue-specific defects are caused by loss of the Drosophila MAN1 LEM domain protein. Genetics (2008) 0.92
Disheveled hair and ear (Dhe), a spontaneous mouse Lmna mutation modeling human laminopathies. PLoS One (2010) 0.91
Abnormal p38α mitogen-activated protein kinase signaling in dilated cardiomyopathy caused by lamin A/C gene mutation. Hum Mol Genet (2012) 0.90
Liver aging and pseudocapillarization in a Werner syndrome mouse model. J Gerontol A Biol Sci Med Sci (2013) 0.90
Dual specificity phosphatase 4 mediates cardiomyopathy caused by lamin A/C (LMNA) gene mutation. J Biol Chem (2012) 0.89
Emerinopathy and laminopathy clinical, pathological and molecular features of muscular dystrophy with nuclear envelopathy in Japan. Acta Myol (2007) 0.89
Lamina-associated polypeptide 2alpha loss impairs heart function and stress response in mice. Circ Res (2009) 0.89
Lamina-associated polypeptide (LAP)2α and nucleoplasmic lamins in adult stem cell regulation and disease. Semin Cell Dev Biol (2013) 0.88
Specific contribution of lamin A and lamin C in the development of laminopathies. Exp Cell Res (2008) 0.85
LMNA E82K mutation activates FAS and mitochondrial pathways of apoptosis in heart tissue specific transgenic mice. PLoS One (2010) 0.84
Defective skeletal muscle growth in lamin A/C-deficient mice is rescued by loss of Lap2α. Hum Mol Genet (2013) 0.84
Depletion of extracellular signal-regulated kinase 1 in mice with cardiomyopathy caused by lamin A/C gene mutation partially prevents pathology before isoenzyme activation. Hum Mol Genet (2013) 0.84
Muscle development, regeneration and laminopathies: how lamins or lamina-associated proteins can contribute to muscle development, regeneration and disease. Cell Mol Life Sci (2012) 0.83
Excess SMAD signaling contributes to heart and muscle dysfunction in muscular dystrophy. Hum Mol Genet (2014) 0.83
Linker of nucleoskeleton and cytoskeleton complex proteins in cardiac structure, function, and disease. Circ Res (2014) 0.83
Causes and consequences of nuclear envelope alterations in tumour progression. Eur J Cell Biol (2016) 0.83
Uncoordinated transcription and compromised muscle function in the lmna-null mouse model of Emery- Emery-Dreyfuss muscular dystrophy. PLoS One (2011) 0.82
Inhibition of extracellular signal-regulated kinase 1/2 signaling has beneficial effects on skeletal muscle in a mouse model of Emery-Dreifuss muscular dystrophy caused by lamin A/C gene mutation. Skelet Muscle (2013) 0.82
Phosphorylation of connexin43 on S279/282 may contribute to laminopathy-associated conduction defects. Exp Cell Res (2012) 0.82
Viscoelastic behavior of human lamin A proteins in the context of dilated cardiomyopathy. PLoS One (2013) 0.81
Cardiomyocyte-specific expression of lamin a improves cardiac function in Lmna-/- mice. PLoS One (2012) 0.81
Mitogen-activated protein kinase kinase 1/2 inhibition and angiotensin II converting inhibition in mice with cardiomyopathy caused by lamin A/C gene mutation. Biochem Biophys Res Commun (2014) 0.80
Nuclear envelope: positioning nuclei and organizing synapses. Curr Opin Cell Biol (2015) 0.80
Depletion of lamina-associated polypeptide 1 from cardiomyocytes causes cardiac dysfunction in mice. Nucleus (2014) 0.80
RNAi-based gene therapy for dominant Limb Girdle Muscular Dystrophies. Curr Gene Ther (2012) 0.79
Lamin A/C mutants disturb sumo1 localization and sumoylation in vitro and in vivo. PLoS One (2012) 0.79
ERK1/2 directly acts on CTGF/CCN2 expression to mediate myocardial fibrosis in cardiomyopathy caused by mutations in the lamin A/C gene. Hum Mol Genet (2016) 0.78
Mitogen-activated protein kinase inhibitor regulation of heart function and fibrosis in cardiomyopathy caused by lamin A/C gene mutation. Trends Cardiovasc Med (2010) 0.78
Gamma-sarcoglycan is required for the response of archvillin to mechanical stimulation in skeletal muscle. Hum Mol Genet (2015) 0.78
Myoblasts and embryonic stem cells differentially engraft in a mouse model of genetic dilated cardiomyopathy. Mol Ther (2013) 0.77
Do lamin A and lamin C have unique roles? Chromosoma (2014) 0.76
Targeting Mitogen-Activated Protein Kinase Signaling in Mouse Models of Cardiomyopathy Caused by Lamin A/C Gene Mutations. Methods Enzymol (2015) 0.76
Deleterious assembly of the lamin A/C mutant p.S143P causes ER stress in familial dilated cardiomyopathy. J Cell Sci (2016) 0.75
Current insights into LMNA cardiomyopathies: Existing models and missing LINCs. Nucleus (2017) 0.75
What Should the Cardiologist know about Lamin Disease? Arrhythm Electrophysiol Rev (2012) 0.75
Genetic Variations Leading to Familial Dilated Cardiomyopathy. Mol Cells (2016) 0.75
Inherited bradyarrhythmia: A diverse genetic background. J Arrhythm (2015) 0.75
Extracellular Matrix Remodeling and Transforming Growth Factor-β Signaling Abnormalities Induced by Lamin A/C Variants that Cause Lipodystrophy. J Lipid Res (2016) 0.75
Recent advances in animal and human pluripotent stem cell modeling of cardiac laminopathy. Stem Cell Res Ther (2016) 0.75
Macrocyclic MEK1/2 inhibitor with efficacy in a mouse model of cardiomyopathy caused by lamin A/C gene mutation. Bioorg Med Chem (2016) 0.75
Murine Electrophysiological Models of Cardiac Arrhythmogenesis. Physiol Rev (2017) 0.75
The effect of the lamin A and its mutants on nuclear structure, cell proliferation, protein stability, and mobility in embryonic cells. Chromosoma (2016) 0.75
Hypertrophic cardiomyopathy: distribution of disease genes, spectrum of mutations, and implications for a molecular diagnosis strategy. Circulation (2003) 5.85
Autologous skeletal myoblast transplantation for severe postinfarction left ventricular dysfunction. J Am Coll Cardiol (2003) 4.91
"Laminopathies": a wide spectrum of human diseases. Exp Cell Res (2007) 3.46
Mandibuloacral dysplasia is caused by a mutation in LMNA-encoding lamin A/C. Am J Hum Genet (2002) 2.90
Meta-analysis of clinical characteristics of 299 carriers of LMNA gene mutations: do lamin A/C mutations portend a high risk of sudden death? J Mol Med (Berl) (2004) 2.81
Mutations in dynamin 2 cause dominant centronuclear myopathy. Nat Genet (2005) 2.33
Cardiovascular involvement, an overlooked feature of Erdheim-Chester disease: report of 6 new cases and a literature review. Medicine (Baltimore) (2004) 2.27
Disruption of the nonneuronal tph1 gene demonstrates the importance of peripheral serotonin in cardiac function. Proc Natl Acad Sci U S A (2003) 2.19
Primary prevention of sudden death in patients with lamin A/C gene mutations. N Engl J Med (2006) 2.16
Loss of the LAT adaptor converts antigen-responsive T cells into pathogenic effectors that function independently of the T cell receptor. Immunity (2009) 2.13
The proline-rich sequence of CD3epsilon controls T cell antigen receptor expression on and signaling potency in preselection CD4+CD8+ thymocytes. Nat Immunol (2008) 2.12
Acute respiratory failure in the elderly: etiology, emergency diagnosis and prognosis. Crit Care (2006) 2.03
Induction of T helper type 2 immunity by a point mutation in the LAT adaptor. Science (2002) 2.01
Tuning of natural killer cell reactivity by NKp46 and Helios calibrates T cell responses. Science (2012) 1.98
Thymus-specific serine protease regulates positive selection of a subset of CD4+ thymocytes. Eur J Immunol (2009) 1.92
Activation of MAPK pathways links LMNA mutations to cardiomyopathy in Emery-Dreifuss muscular dystrophy. J Clin Invest (2007) 1.87
"Necklace" fibers, a new histological marker of late-onset MTM1-related centronuclear myopathy. Acta Neuropathol (2008) 1.83
Nonrecurrent MECP2 duplications mediated by genomic architecture-driven DNA breaks and break-induced replication repair. Genome Res (2008) 1.81
Th2 lymphoproliferative disorder of LatY136F mutant mice unfolds independently of TCR-MHC engagement and is insensitive to the action of Foxp3+ regulatory T cells. J Immunol (2008) 1.78
Non-T cell activation linker (NTAL): a transmembrane adaptor protein involved in immunoreceptor signaling. J Exp Med (2002) 1.74
Comparison of echocardiography and plasma B-type natriuretic peptide for monitoring the response to treatment in acute heart failure. Eur Heart J (2004) 1.71
BMP signaling controls muscle mass. Nat Genet (2013) 1.68
A genome-wide association study identifies two loci associated with heart failure due to dilated cardiomyopathy. Eur Heart J (2011) 1.67
The Ig-like structure of the C-terminal domain of lamin A/C, mutated in muscular dystrophies, cardiomyopathy, and partial lipodystrophy. Structure (2002) 1.64
Visualization of the earliest steps of gammadelta T cell development in the adult thymus. Nat Immunol (2006) 1.64
Inhibition of extracellular signal-regulated kinase signaling to prevent cardiomyopathy caused by mutation in the gene encoding A-type lamins. Hum Mol Genet (2008) 1.64
Skeletal myoblast transplantation in ischemic heart failure: long-term follow-up of the first phase I cohort of patients. Circulation (2006) 1.63
Variant CCG and GGC repeats within the CTG expansion dramatically modify mutational dynamics and likely contribute toward unusual symptoms in some myotonic dystrophy type 1 patients. Hum Mol Genet (2010) 1.61
Nuclear envelope alterations in fibroblasts from LGMD1B patients carrying nonsense Y259X heterozygous or homozygous mutation in lamin A/C gene. Exp Cell Res (2003) 1.60
Exercise training attenuates the hypermuscular phenotype and restores skeletal muscle function in the myostatin null mouse. Exp Physiol (2011) 1.56
ZFHX1B mutations in patients with Mowat-Wilson syndrome. Hum Mutat (2007) 1.55
Comparison of brain natriuretic peptide and probrain natriuretic peptide in the diagnosis of cardiogenic pulmonary edema in patients aged 65 and older. J Am Geriatr Soc (2005) 1.54
Early identification of mutation carriers in familial hypertrophic cardiomyopathy by combined echocardiography and tissue Doppler imaging. Eur Heart J (2010) 1.53
Familial hypertrophic cardiomyopathy: the same mutation, different prognosis. Comparison of two families with a long follow-up. Rev Port Cardiol (2003) 1.50
Identification of the genotypes causing hypertrophic cardiomyopathy in northern Sweden. J Mol Cell Cardiol (2003) 1.48
Negative regulation of mast cell signaling and function by the adaptor LAB/NTAL. J Exp Med (2004) 1.47
Mutations in KLHL40 are a frequent cause of severe autosomal-recessive nemaline myopathy. Am J Hum Genet (2013) 1.46
Mutations of the FHL1 gene cause Emery-Dreifuss muscular dystrophy. Am J Hum Genet (2009) 1.44
Intensive cardiovascular risk factors therapy and prevalence of silent myocardial ischaemia in patients with type 2 diabetes. Arch Cardiovasc Dis (2008) 1.44
Multiplicity and plasticity of natural killer cell signaling pathways. Blood (2005) 1.40
Homozygous nonsense mutation in the FOXE3 gene as a cause of congenital primary aphakia in humans. Am J Hum Genet (2006) 1.40
Neutrophil depletion impairs natural killer cell maturation, function, and homeostasis. J Exp Med (2012) 1.39
Asymmetric septal hypertrophy in heterozygous cMyBP-C null mice. Cardiovasc Res (2004) 1.39
Activation of MAPK in hearts of EMD null mice: similarities between mouse models of X-linked and autosomal dominant Emery Dreifuss muscular dystrophy. Hum Mol Genet (2007) 1.38
Sudden cardiac death: clinical evaluation of paediatric family members. Europace (2010) 1.38
Genetic association study identifies HSPB7 as a risk gene for idiopathic dilated cardiomyopathy. PLoS Genet (2010) 1.37
Dynamic recruitment of the adaptor protein LAT: LAT exists in two distinct intracellular pools and controls its own recruitment. J Cell Sci (2004) 1.37
Lamin A/C-mediated neuromuscular junction defects in Emery-Dreifuss muscular dystrophy. J Cell Biol (2009) 1.36
Spectrum of cardiac lesions in Behçet disease: a series of 52 patients and review of the literature. Medicine (Baltimore) (2012) 1.33
ColVI myopathies: where do we stand, where do we go? Skelet Muscle (2011) 1.31
Protection, promotion and support of breast-feeding in Europe: progress from 2002 to 2007. Public Health Nutr (2009) 1.30
Clinical evaluation of nine free thyroxine assays: persistent problems in particular populations. Clin Chem Lab Med (2003) 1.29
LAT regulates gammadelta T cell homeostasis and differentiation. Nat Immunol (2003) 1.29
Clinical evaluation of a reverse hybridization assay for the molecular detection of twelve MEFV gene mutations. Clin Chem (2003) 1.27
Autophagic degradation of nuclear components in mammalian cells. Autophagy (2009) 1.27
Long-term efficacy of myoblast transplantation on regional structure and function after myocardial infarction. Circulation (2002) 1.27
Nonsense-mediated mRNA decay and ubiquitin-proteasome system regulate cardiac myosin-binding protein C mutant levels in cardiomyopathic mice. Circ Res (2009) 1.26
The function of Myostatin and strategies of Myostatin blockade-new hope for therapies aimed at promoting growth of skeletal muscle. Neuromuscul Disord (2005) 1.21
Evaluating the efficiency of isotope transmission for improved panel design and a comparison of the detection sensitivities of mass cytometer instruments. Cytometry A (2015) 1.21
Early onset collagen VI myopathies: Genetic and clinical correlations. Ann Neurol (2010) 1.21
Mitogen-activated protein kinase inhibitors improve heart function and prevent fibrosis in cardiomyopathy caused by mutation in lamin A/C gene. Circulation (2010) 1.21
Mutations in the ANKRD1 gene encoding CARP are responsible for human dilated cardiomyopathy. Eur Heart J (2009) 1.19
Impaired accumulation of antigen-specific CD8 lymphocytes in chemokine CCL25-deficient intestinal epithelium and lamina propria. J Immunol (2007) 1.19
A hypomorphic allele of ZAP-70 reveals a distinct thymic threshold for autoimmune disease versus autoimmune reactivity. J Exp Med (2009) 1.17
De novo LMNA mutations cause a new form of congenital muscular dystrophy. Ann Neurol (2008) 1.17
Clinical and genetic heterogeneity in laminopathies. Biochem Soc Trans (2011) 1.16
Evidence for FHL1 as a novel disease gene for isolated hypertrophic cardiomyopathy. Hum Mol Genet (2012) 1.16
Treatment with selumetinib preserves cardiac function and improves survival in cardiomyopathy caused by mutation in the lamin A/C gene. Cardiovasc Res (2011) 1.15
The E148Q MEFV allele is not implicated in the development of familial Mediterranean fever. Hum Mutat (2003) 1.11
Apoptosis in mitochondrial myopathies is linked to mitochondrial proliferation. Brain (2006) 1.11
Improvement in the management of chronic heart failure since the publication of the updated guidelines of the European Society of Cardiology. The Impact-Reco Programme. Eur J Heart Fail (2009) 1.11
A proton leak current through the cardiac sodium channel is linked to mixed arrhythmia and the dilated cardiomyopathy phenotype. PLoS One (2012) 1.07
Recent advances in understanding serotonin regulation of cardiovascular function. Trends Mol Med (2004) 1.05
Clinical relevance of atrial fibrillation/flutter, stroke, pacemaker implant, and heart failure in Emery-Dreifuss muscular dystrophy: a long-term longitudinal study. Stroke (2003) 1.05
Current aspects of the spectrum of acute heart failure syndromes in a real-life setting: the OFICA study. Eur J Heart Fail (2012) 1.04
CD93 is required for maintenance of antibody secretion and persistence of plasma cells in the bone marrow niche. Proc Natl Acad Sci U S A (2009) 1.04
Novel deletions in MYH7 and MYBPC3 identified in Indian families with familial hypertrophic cardiomyopathy. J Mol Cell Cardiol (2003) 1.04
The tetrapeptide AcSDKP, an inhibitor of primitive hematopoietic cell proliferation, induces angiogenesis in vitro and in vivo. Blood (2002) 1.04
Distinctive serum miRNA profile in mouse models of striated muscular pathologies. PLoS One (2013) 1.03
Influence of mobilized stem cells on myocardial infarct repair in a nonhuman primate model. Blood (2003) 1.01
Extreme variability of phenotype in patients with an identical missense mutation in the lamin A/C gene: from congenital onset with severe phenotype to milder classic Emery-Dreifuss variant. Arch Neurol (2004) 1.01
The lymphoid lineage-specific actin-uncapping protein Rltpr is essential for costimulation via CD28 and the development of regulatory T cells. Nat Immunol (2013) 1.00
'State-of-the-heart' of cardiac laminopathies. Curr Opin Cardiol (2013) 1.00
Roles of the C-terminal tyrosine residues of LAT in GPVI-induced platelet activation: insights into the mechanism of PLC gamma 2 activation. Blood (2007) 1.00
Emery-Dreifuss muscular dystrophy. Eur J Hum Genet (2002) 0.99
The type 1 cysteinyl leukotriene receptor triggers calcium influx and chemotaxis in mouse alpha beta- and gamma delta effector T cells. J Immunol (2005) 0.99
The Th2 lymphoproliferation developing in LatY136F mutant mice triggers polyclonal B cell activation and systemic autoimmunity. J Immunol (2006) 0.99
Comparative value of tissue Doppler imaging and m-mode color Doppler mitral flow propagation velocity for the evaluation of left ventricular filling pressure. Chest (2005) 0.99
Skeletal muscle biopsy analysis in reducing body myopathy and other FHL1-related disorders. J Neuropathol Exp Neurol (2013) 0.99