S Squarzoni

Summary

Affiliation: Unit of Bologna
Country: Italy

Publications

  1. pmc The empowerment of translational research: lessons from laminopathies
    Sara Benedetti
    National Research Council of Italy, Institute of Molecular Genetics, IGM CNR, Unit of Bologna, Via Di Barbiano 1 10, 40136, Bologna, Italy
    Orphanet J Rare Dis 7:37. 2012
  2. pmc Expression of collagen VI α5 and α6 chains in human muscle and in Duchenne muscular dystrophy-related muscle fibrosis
    Patrizia Sabatelli
    CNR National Research Council of Italy, Institute of Molecular Genetics IOR, Bologna, Italy
    Matrix Biol 31:187-96. 2012
  3. ncbi request reprint Immunocytochemical detection of emerin within the nuclear matrix
    S Squarzoni
    Istituto Citomorfologia N P CNR, Bologna, Italy
    Neuromuscul Disord 8:338-44. 1998
  4. ncbi request reprint Emerin presence in platelets
    S Squarzoni
    Ist Citomorfologia N P CNR, IOR, Bologna, Italy
    Acta Neuropathol 100:291-8. 2000
  5. ncbi request reprint Emerin increase in regenerating muscle fibers
    S Squarzoni
    ITOI CNR, Unit of Bologna c o Istituti Ortopedici Rizzoli, Bologna, Italy
    Eur J Histochem 49:355-62. 2005
  6. pmc Prelamin A-mediated recruitment of SUN1 to the nuclear envelope directs nuclear positioning in human muscle
    E Mattioli
    Institute for Molecular Genetics, IGM CNR, Unit of Bologna, Bologna, Italy
    Cell Death Differ 18:1305-15. 2011
  7. ncbi request reprint Immunocytochemistry of nuclear domains and Emery-Dreifuss muscular dystrophy pathophysiology
    N M Maraldi
    Laboratory of Cell Biology and Electron Microscopy, IOR, Bologna, Italy
    Eur J Histochem 47:3-16. 2003
  8. pmc Autophagic degradation of farnesylated prelamin A as a therapeutic approach to lamin-linked progeria
    V Cenni
    National Research Council of Italy, Institute of Molecular Genetics, IGM CNR, Unit of Bologna IOR, Bologna, Italy
    Eur J Histochem 55:e36. 2011
  9. ncbi request reprint Staurosporine treatment and serum starvation promote the cleavage of emerin in cultured mouse myoblasts: involvement of a caspase-dependent mechanism
    M Columbaro
    Laboratory of Neuromuscular Pathology, IOR, Bologna, Italy
    FEBS Lett 509:423-9. 2001
  10. pmc Lamin A N-terminal phosphorylation is associated with myoblast activation: impairment in Emery-Dreifuss muscular dystrophy
    V Cenni
    ITOI, CNR, Unit of Bologna, c o IOR, Bologna, Italy
    J Med Genet 42:214-20. 2005

Collaborators

Detail Information

Publications21

  1. pmc The empowerment of translational research: lessons from laminopathies
    Sara Benedetti
    National Research Council of Italy, Institute of Molecular Genetics, IGM CNR, Unit of Bologna, Via Di Barbiano 1 10, 40136, Bologna, Italy
    Orphanet J Rare Dis 7:37. 2012
    ....
  2. pmc Expression of collagen VI α5 and α6 chains in human muscle and in Duchenne muscular dystrophy-related muscle fibrosis
    Patrizia Sabatelli
    CNR National Research Council of Italy, Institute of Molecular Genetics IOR, Bologna, Italy
    Matrix Biol 31:187-96. 2012
    ..While the α5 chain may have a specialized function in tissue areas subjected to tensile stress, the α6 chain appears implicated in ECM remodeling during muscle fibrosis...
  3. ncbi request reprint Immunocytochemical detection of emerin within the nuclear matrix
    S Squarzoni
    Istituto Citomorfologia N P CNR, Bologna, Italy
    Neuromuscul Disord 8:338-44. 1998
    ..This suggests a tight binding between emerin and the nuclear lamina independently from the interactions between the C-terminal hydrophobic domain of the protein and the inner nuclear membrane...
  4. ncbi request reprint Emerin presence in platelets
    S Squarzoni
    Ist Citomorfologia N P CNR, IOR, Bologna, Italy
    Acta Neuropathol 100:291-8. 2000
    ..We found also that emerin is present in the cytoplasm of megakaryocytes, while it is absent in circulating granulocytes...
  5. ncbi request reprint Emerin increase in regenerating muscle fibers
    S Squarzoni
    ITOI CNR, Unit of Bologna c o Istituti Ortopedici Rizzoli, Bologna, Italy
    Eur J Histochem 49:355-62. 2005
    ..The impairment of skeletal muscle physiological regeneration or reorganization could be a possible pathogenetic mechanism for Emery Dreifuss muscular dystrophy...
  6. pmc Prelamin A-mediated recruitment of SUN1 to the nuclear envelope directs nuclear positioning in human muscle
    E Mattioli
    Institute for Molecular Genetics, IGM CNR, Unit of Bologna, Bologna, Italy
    Cell Death Differ 18:1305-15. 2011
    ..These results demonstrate that the interplay between SUN1 and farnesylated prelamin A contributes to nuclear positioning in human myofibers and may be implicated in pathogenetic mechanisms...
  7. ncbi request reprint Immunocytochemistry of nuclear domains and Emery-Dreifuss muscular dystrophy pathophysiology
    N M Maraldi
    Laboratory of Cell Biology and Electron Microscopy, IOR, Bologna, Italy
    Eur J Histochem 47:3-16. 2003
    ..The possibility that Emery-Deifuss muscular dystrophy pathogenesis could involve alteration of the signaling pathway is considered...
  8. pmc Autophagic degradation of farnesylated prelamin A as a therapeutic approach to lamin-linked progeria
    V Cenni
    National Research Council of Italy, Institute of Molecular Genetics, IGM CNR, Unit of Bologna IOR, Bologna, Italy
    Eur J Histochem 55:e36. 2011
    ....
  9. ncbi request reprint Staurosporine treatment and serum starvation promote the cleavage of emerin in cultured mouse myoblasts: involvement of a caspase-dependent mechanism
    M Columbaro
    Laboratory of Neuromuscular Pathology, IOR, Bologna, Italy
    FEBS Lett 509:423-9. 2001
    ....
  10. pmc Lamin A N-terminal phosphorylation is associated with myoblast activation: impairment in Emery-Dreifuss muscular dystrophy
    V Cenni
    ITOI, CNR, Unit of Bologna, c o IOR, Bologna, Italy
    J Med Genet 42:214-20. 2005
    ..A molecular complex in muscle cells formed by lamin A/C, emerin, and nuclear actin has been identified. The stability of this protein complex appears to be related to phosphorylation mechanisms...
  11. ncbi request reprint Functional domains of the nucleus: implications for Emery-Dreifuss muscular dystrophy
    N M Maraldi
    Laboratory of Cell Biology and Electron Microscopy, Istituto Ortopedici Rizzoli, CNR, Via Di Barbiano 1 10, 40139, Bologna, Italy
    Neuromuscul Disord 12:815-23. 2002
    ....
  12. pmc Rescue of heterochromatin organization in Hutchinson-Gilford progeria by drug treatment
    M Columbaro
    Laboratory of Cell Biology, Istituti Ortopedici Rizzoli, Bologna, Italy
    Cell Mol Life Sci 62:2669-78. 2005
    ..These results suggest that morpho-functional defects of HGPS nuclei are directly related to progerin accumulation and can be rectified by drug treatment...
  13. ncbi request reprint Increase of neuronal nitric oxide synthase in rat skeletal muscle during ageing
    C Capanni
    Dipartimento di Biochimica G Moruzzi Università di Bologna, Bologna, Italy
    Biochem Biophys Res Commun 245:216-9. 1998
    ..We report that in aged rats the nNOS amount in skeletal muscle increases both in the soluble and microsomal fractions and that an additional intracytoplasmic localisation appears...
  14. pmc Critical evaluation of the use of cell cultures for inclusion in clinical trials of patients affected by collagen VI myopathies
    P Sabatelli
    CNR National Research Council of Italy, Institute of Molecular Genetics c o IOR, Bologna, Italy
    J Cell Physiol 227:2927-35. 2012
    ....
  15. ncbi request reprint Nuclear envelope proteins and chromatin arrangement: a pathogenic mechanism for laminopathies
    N M Maraldi
    Istituto per i Trapianti d Organo e l Immunocitologia ITOI C N R, Unit of Bologna, via di Barbiano 1 10 c o IOR 40136 Bologna, Italy
    Eur J Histochem 50:1-8. 2006
    ..Our results further show that the absence of emerin expression alters the distribution of pre-lamin A and of heterochromatin areas, suggesting a major involvement of emerin in pre-lamin A-mediated mechanisms of chromatin remodeling...
  16. ncbi request reprint Emerin expression at the early stages of myogenic differentiation
    G Lattanzi
    Istituto di Citomorfologia Normale e Patologica CNR, via di Barbiano 1 10 40136 Bologna, Italy
    Differentiation 66:208-17. 2000
    ..These data suggest a role for emerin during proliferation of activated satellite cells and at the early stages of differentiation...
  17. ncbi request reprint Intracellular detection of laminin alpha 2 chain in skin by electron microscopy immunocytochemistry: comparison between normal and laminin alpha 2 chain deficient subjects
    S Squarzoni
    Istituto di Citomorfologia N P CNR, Bologna, Italy
    Neuromuscul Disord 7:91-8. 1997
    ..These data suggest that the function of the laminin alpha 2 chain is different in the epidermis as compared to that in muscle and peripheral nerve, where it is localized in the basement membrane...
  18. ncbi request reprint Nuclear alterations in autosomal-dominant Emery-Dreifuss muscular dystrophy
    P Sabatelli
    Istituto di Citomorfologia, Consiglio Nazionale delle Ricerche, via Di Barbiano 1/10 c/o Istituti Ortopedici Rizzoli, 40136 Bologna, Italy
    Muscle Nerve 24:826-9. 2001
    ....
  19. ncbi request reprint Heart-specific localization of emerin: new insights into Emery-Dreifuss muscular dystrophy
    L Cartegni
    Institute of Genetics, Biochemistry and Evolution CNR, via Abbiategrasso 207, 27100 Pavia, Italy
    Hum Mol Genet 6:2257-64. 1997
    ..In heart its specific localization to desmosomes and fasciae adherentes could account for the characteristic conduction defects described in patients...
  20. doi request reprint Autosomal recessive myosclerosis myopathy is a collagen VI disorder
    L Merlini
    Department of Experimental and Diagnostic Medicine, University of Ferrara, Italy
    Neurology 71:1245-53. 2008
    ..To determine the clinical and molecular features of a new phenotype related to collagen VI myopathies...
  21. ncbi request reprint 98th ENMC International Workshop on Congenital Muscular Dystrophy (CMD), 7th Workshop of the International Consortium on CMD, 2nd Workshop of the MYO CLUSTER project GENRE. 26-28th October, 2001, Naarden, The Netherlands
    F Muntoni
    Department of Paediatrics, Hammersmith Hospital, London, UK
    Neuromuscul Disord 12:889-96. 2002