Published in J Cell Sci on June 03, 2003
Lamin A/C deficiency causes defective nuclear mechanics and mechanotransduction. J Clin Invest (2004) 5.68
Reversal of the cellular phenotype in the premature aging disease Hutchinson-Gilford progeria syndrome. Nat Med (2005) 5.01
Homozygous and compound heterozygous mutations in ZMPSTE24 cause the laminopathy restrictive dermopathy. J Invest Dermatol (2005) 2.06
Mammalian SUN protein interaction networks at the inner nuclear membrane and their role in laminopathy disease processes. J Biol Chem (2009) 1.79
How do mutations in lamins A and C cause disease? J Clin Invest (2004) 1.47
Identification of differential protein interactors of lamin A and progerin. Nucleus (2010) 1.14
Muscular dystrophy-associated SUN1 and SUN2 variants disrupt nuclear-cytoskeletal connections and myonuclear organization. PLoS Genet (2014) 1.13
Nesprins, but not sun proteins, switch isoforms at the nuclear envelope during muscle development. Dev Dyn (2010) 1.13
Lamin A N-terminal phosphorylation is associated with myoblast activation: impairment in Emery-Dreifuss muscular dystrophy. J Med Genet (2005) 1.12
Dependence of diffusional mobility of integral inner nuclear membrane proteins on A-type lamins. Biochemistry (2006) 1.12
Molecular genetic analysis of the nested Drosophila melanogaster lamin C gene. Genetics (2005) 1.01
The apparent absence of lamin B1 and emerin in many tissue nuclei is due to epitope masking. J Mol Histol (2005) 0.97
Morphological analysis of 13 LMNA variants identified in a cohort of 324 unrelated patients with idiopathic or familial dilated cardiomyopathy. Circ Cardiovasc Genet (2009) 0.96
Altered protein dynamics of disease-associated lamin A mutants. BMC Cell Biol (2004) 0.92
A comparative study of Drosophila and human A-type lamins. PLoS One (2009) 0.91
Blocking farnesylation of the prelamin A variant in Hutchinson-Gilford progeria syndrome alters the distribution of A-type lamins. Nucleus (2012) 0.90
Expression and localization of nuclear proteins in autosomal-dominant Emery-Dreifuss muscular dystrophy with LMNA R377H mutation. BMC Cell Biol (2004) 0.88
Altering lamina assembly reveals lamina-dependent and -independent functions for A-type lamins. J Cell Sci (2015) 0.78
A new LMNA mutation causing limb girdle muscular dystrophy 1B. J Neurol (2005) 0.77
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
Lamin a truncation in Hutchinson-Gilford progeria. Science (2003) 8.01
Lamin A/C deficiency causes defective nuclear mechanics and mechanotransduction. J Clin Invest (2004) 5.68
Astrocytes promote myelination in response to electrical impulses. Neuron (2006) 4.23
Functional amounts of dystrophin produced by skipping the mutated exon in the mdx dystrophic mouse. Nat Med (2003) 4.03
Life at the edge: the nuclear envelope and human disease. Nat Rev Mol Cell Biol (2002) 3.84
Juxtaparanodal clustering of Shaker-like K+ channels in myelinated axons depends on Caspr2 and TAG-1. J Cell Biol (2003) 3.80
A human iPSC model of Hutchinson Gilford Progeria reveals vascular smooth muscle and mesenchymal stem cell defects. Cell Stem Cell (2010) 3.41
Accelerated ageing in mice deficient in Zmpste24 protease is linked to p53 signalling activation. Nature (2005) 3.31
A perinuclear actin cap regulates nuclear shape. Proc Natl Acad Sci U S A (2009) 3.12
Lamins A and C but not lamin B1 regulate nuclear mechanics. J Biol Chem (2006) 3.02
Defects in nuclear structure and function promote dilated cardiomyopathy in lamin A/C-deficient mice. J Clin Invest (2004) 2.67
Abnormal nuclear shape and impaired mechanotransduction in emerin-deficient cells. J Cell Biol (2005) 2.66
Nesprin 4 is an outer nuclear membrane protein that can induce kinesin-mediated cell polarization. Proc Natl Acad Sci U S A (2009) 2.59
A progeroid syndrome in mice is caused by defects in A-type lamins. Nature (2003) 2.57
Nuclear lamin A/C deficiency induces defects in cell mechanics, polarization, and migration. Biophys J (2007) 2.51
Heterozygosity for Lmna deficiency eliminates the progeria-like phenotypes in Zmpste24-deficient mice. Proc Natl Acad Sci U S A (2004) 2.39
LBR and lamin A/C sequentially tether peripheral heterochromatin and inversely regulate differentiation. Cell (2013) 2.38
Neuronal SMN expression corrects spinal muscular atrophy in severe SMA mice while muscle-specific SMN expression has no phenotypic effect. Hum Mol Genet (2008) 2.35
Prelamin A and lamin A appear to be dispensable in the nuclear lamina. J Clin Invest (2006) 2.23
Homozygous defects in LMNA, encoding lamin A/C nuclear-envelope proteins, cause autosomal recessive axonal neuropathy in human (Charcot-Marie-Tooth disorder type 2) and mouse. Am J Hum Genet (2002) 2.20
Accumulation of the inner nuclear envelope protein Sun1 is pathogenic in progeric and dystrophic laminopathies. Cell (2012) 2.18
The imprinted gene Magel2 regulates normal circadian output. Nat Genet (2007) 2.18
A-type lamins regulate retinoblastoma protein function by promoting subnuclear localization and preventing proteasomal degradation. Proc Natl Acad Sci U S A (2004) 2.17
Risk factors and comorbidities in primary biliary cirrhosis: a controlled interview-based study of 1032 patients. Hepatology (2005) 2.15
Foxa2 is essential for mouse endometrial gland development and fertility. Biol Reprod (2010) 2.12
Diseases of the nuclear envelope. Cold Spring Harb Perspect Biol (2010) 2.12
MAN1, an integral protein of the inner nuclear membrane, binds Smad2 and Smad3 and antagonizes transforming growth factor-beta signaling. Hum Mol Genet (2004) 2.10
Nuclear positioning. Cell (2013) 2.01
The laminopathies: nuclear structure meets disease. Curr Opin Genet Dev (2003) 2.00
Dysfunctional connections between the nucleus and the actin and microtubule networks in laminopathic models. Biophys J (2008) 1.92
Functional coupling between the extracellular matrix and nuclear lamina by Wnt signaling in progeria. Dev Cell (2010) 1.90
Activation of MAPK pathways links LMNA mutations to cardiomyopathy in Emery-Dreifuss muscular dystrophy. J Clin Invest (2007) 1.87
Loss of emerin at the nuclear envelope disrupts the Rb1/E2F and MyoD pathways during muscle regeneration. Hum Mol Genet (2006) 1.85
Novel roles for A-type lamins in telomere biology and the DNA damage response pathway. EMBO J (2009) 1.83
The nuclear envelope as a signaling node in development and disease. Dev Cell (2009) 1.81
A dual role for A-type lamins in DNA double-strand break repair. Cell Cycle (2011) 1.80
The nuclear lamins: flexibility in function. Nat Rev Mol Cell Biol (2012) 1.76
To knockout in 129 or in C57BL/6: that is the question. Trends Genet (2004) 1.76
Dynamics and molecular interactions of linker of nucleoskeleton and cytoskeleton (LINC) complex proteins. J Cell Sci (2009) 1.74
A-type lamins: guardians of the soma? Nat Cell Biol (2004) 1.68
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
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
Transmembrane protein Sun2 is involved in tethering mammalian meiotic telomeres to the nuclear envelope. Proc Natl Acad Sci U S A (2007) 1.63
Global gene expression profiling reveals similarities and differences among mouse pluripotent stem cells of different origins and strains. Dev Biol (2007) 1.61
Lamin A variants that cause striated muscle disease are defective in anchoring transmembrane actin-associated nuclear lines for nuclear movement. Proc Natl Acad Sci U S A (2010) 1.61
The laminopathies: the functional architecture of the nucleus and its contribution to disease. Annu Rev Genomics Hum Genet (2006) 1.61
Expression of an LMNA-N195K variant of A-type lamins results in cardiac conduction defects and death in mice. Hum Mol Genet (2005) 1.61
At least ten genes define the imprinted Dlk1-Dio3 cluster on mouse chromosome 12qF1. PLoS One (2009) 1.60
The lipid phosphatase LPP3 regulates extra-embryonic vasculogenesis and axis patterning. Development (2003) 1.57
Aging and nuclear organization: lamins and progeria. Curr Opin Cell Biol (2004) 1.57
Biliary apotopes and anti-mitochondrial antibodies activate innate immune responses in primary biliary cirrhosis. Hepatology (2010) 1.53
Osteoclast size is controlled by Fra-2 through LIF/LIF-receptor signalling and hypoxia. Nature (2008) 1.52
The carboxyl-terminal region common to lamins A and C contains a DNA binding domain. Biochemistry (2003) 1.50
Actin-myosin-based contraction is responsible for apoptotic nuclear disintegration. J Cell Biol (2005) 1.49
Indoprofen upregulates the survival motor neuron protein through a cyclooxygenase-independent mechanism. Chem Biol (2004) 1.45
Nuclear lamin A inhibits adipocyte differentiation: implications for Dunnigan-type familial partial lipodystrophy. Hum Mol Genet (2006) 1.45
Human heterochromatin protein 1 isoforms HP1(Hsalpha) and HP1(Hsbeta) interfere with hTERT-telomere interactions and correlate with changes in cell growth and response to ionizing radiation. Mol Cell Biol (2003) 1.44
Primary laminopathy fibroblasts display altered genome organization and apoptosis. Aging Cell (2007) 1.43
Inactivation of the mouse Magel2 gene results in growth abnormalities similar to Prader-Willi syndrome. Hum Mol Genet (2007) 1.42
Lamin A/C haploinsufficiency causes dilated cardiomyopathy and apoptosis-triggered cardiac conduction system disease. J Mol Cell Cardiol (2007) 1.42
Targeted disruption of the 2B4 gene in mice reveals an in vivo role of 2B4 (CD244) in the rejection of B16 melanoma cells. J Immunol (2005) 1.41
Long-term persistence of donor nuclei in a Duchenne muscular dystrophy patient receiving bone marrow transplantation. J Clin Invest (2002) 1.41
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
Lamin A/C-mediated neuromuscular junction defects in Emery-Dreifuss muscular dystrophy. J Cell Biol (2009) 1.36
Nuclear envelope defects cause stem cell dysfunction in premature-aging mice. J Cell Biol (2008) 1.36
The nuclear envelope and human disease. Physiology (Bethesda) (2004) 1.32
B-MYB is essential for normal cell cycle progression and chromosomal stability of embryonic stem cells. PLoS One (2008) 1.32
Loss of nucleoplasmic LAP2alpha-lamin A complexes causes erythroid and epidermal progenitor hyperproliferation. Nat Cell Biol (2008) 1.30
A role for complexes of survival of motor neurons (SMN) protein with gemins and profilin in neurite-like cytoplasmic extensions of cultured nerve cells. Exp Cell Res (2005) 1.29
Lamin B1 fluctuations have differential effects on cellular proliferation and senescence. J Cell Biol (2013) 1.28
Nuclear envelope proteins and neuromuscular diseases. Muscle Nerve (2003) 1.23
The LINC complex is essential for hearing. J Clin Invest (2013) 1.22
MSH2 ATPase domain mutation affects CTG*CAG repeat instability in transgenic mice. PLoS Genet (2009) 1.22
Paternal and maternal genomes confer opposite effects on proliferation, cell-cycle length, senescence, and tumor formation. Proc Natl Acad Sci U S A (2003) 1.22
Cell nuclei spin in the absence of lamin b1. J Biol Chem (2007) 1.22
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
TAN lines: a novel nuclear envelope structure involved in nuclear positioning. Nucleus (2011) 1.20
A direct interaction between the survival motor neuron protein and p53 and its relationship to spinal muscular atrophy. J Biol Chem (2001) 1.20
Histone modifications and lamin A regulate chromatin protein dynamics in early embryonic stem cell differentiation. Nat Commun (2012) 1.19
Temsirolimus activates autophagy and ameliorates cardiomyopathy caused by lamin A/C gene mutation. Sci Transl Med (2012) 1.19
Leukemia inhibitory factor regulates microvessel density by modulating oxygen-dependent VEGF expression in mice. J Clin Invest (2008) 1.18
Muscleblind-like proteins: similarities and differences in normal and myotonic dystrophy muscle. Am J Pathol (2008) 1.17
Cochlin, a secreted von Willebrand factor type a domain-containing factor, is regulated by leukemia inhibitory factor in the uterus at the time of embryo implantation. Endocrinology (2003) 1.17
Lsh controls silencing of the imprinted Cdkn1c gene. Development (2005) 1.16
Defective mRNA in myotonic dystrophy accumulates at the periphery of nuclear splicing speckles. Genes Cells (2007) 1.16
Correlation of initial autoantibody profile and clinical outcome in primary biliary cirrhosis. Hepatology (2006) 1.15
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
Matrix metalloproteinase-1 associates with intracellular organelles and confers resistance to lamin A/C degradation during apoptosis. Am J Pathol (2005) 1.14
Nesprins, but not sun proteins, switch isoforms at the nuclear envelope during muscle development. Dev Dyn (2010) 1.13
PBC screen: an IgG/IgA dual isotype ELISA detecting multiple mitochondrial and nuclear autoantibodies specific for primary biliary cirrhosis. J Autoimmun (2010) 1.13
A new pathway that regulates 53BP1 stability implicates cathepsin L and vitamin D in DNA repair. EMBO J (2011) 1.12
Emerin interacts in vitro with the splicing-associated factor, YT521-B. Eur J Biochem (2003) 1.12
Dependence of diffusional mobility of integral inner nuclear membrane proteins on A-type lamins. Biochemistry (2006) 1.12