Acta Neuropathologica

, Volume 100, Issue 3, pp 291–298

Emerin presence in platelets

  • S. Squarzoni
  • P. Sabatelli
  • C. Capanni
  • S. Petrini
  • A. Ognibene
  • D. Toniolo
  • F. Cobianchi
  • G. Zauli
  • A. Bassini
  • A. Baracca
  • C. Guarnieri
  • L. Merlini
  • N. M. Maraldi
Regular Paper

DOI: 10.1007/s004019900169

Cite this article as:
Squarzoni, S., Sabatelli, P., Capanni, C. et al. Acta Neuropathol (2000) 100: 291. doi:10.1007/s004019900169

Abstract

Emerin is an almost ubiquitous protein which is abnormal in X-linked Emery-Dreifuss muscular dystrophy (EMD), a syndrome characterized by muscle weakness, joint contractures and cardiac arrhythmia. Emerin is localized in the cells at the nuclear rim and its function is still unknown. In some models, emerin has also been described in the cytoplasm; however, its presence outside the nucleus is still matter of debate. We report the presence of emerin in circulating normal human platelets and its absence in platelets from X-linked EMD patients. Since platelets are cytoplasmic fragments derived from megakaryocytes, the presence of emerin in platelets confirms cytoplasmic localization of this protein, probably related to specific functions. We found also that emerin is present in the cytoplasm of megakaryocytes, while it is absent in circulating granulocytes.

Key words Emerin Platelets Cytoskeleton Emery-Dreifuss muscular dystrophy 

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Copyright information

© Springer-Verlag Berlin Heidelberg 2000

Authors and Affiliations

  • S. Squarzoni
    • 1
  • P. Sabatelli
    • 1
  • C. Capanni
    • 2
  • S. Petrini
    • 3
  • A. Ognibene
    • 4
  • D. Toniolo
    • 5
  • F. Cobianchi
    • 5
  • G. Zauli
    • 6
  • A. Bassini
    • 6
  • A. Baracca
    • 2
  • C. Guarnieri
    • 2
  • L. Merlini
    • 3
  • N. M. Maraldi
    • 1
  1. 1.Ist. Citomorfologia N.P. CNR c/o IOR, via Di Barbiano 1/10, 40136 Bologna, Italy e-mail: squarzoni@area.bo.cnr.it, Tel.: +39-051-584090, Fax: +39-051-583593IT
  2. 2.Dipartimento di Biochimica ‘G. Moruzzi’, Università di Bologna, Bologna, ItalyIT
  3. 3.Lab. Patologia Neuromuscolare IOR, Bologna, ItalyIT
  4. 4.Lab. Biologia Cellulare e Microscopia Elettronica IOR, Bologna, ItalyIT
  5. 5.Ist. Genetica Biochimica Evoluzionistica CNR, Pavia, ItalyIT
  6. 6.Sezione di Anatomia Umana, Dipartimento di Morfologia ed Embriologia, Università di Ferrara, Ferrara, ItalyIT

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