Involvement of prelamin A in laminopathies. (Articolo in rivista)

Type
Label
  • Involvement of prelamin A in laminopathies. (Articolo in rivista) (literal)
Anno
  • 2007-01-01T00:00:00+01:00 (literal)
Alternative label
  • Maraldi NM, Lattanzi G. (2007)
    Involvement of prelamin A in laminopathies.
    in Critical reviews in eukaryotic gene expression
    (literal)
Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#autori
  • Maraldi NM, Lattanzi G. (literal)
Pagina inizio
  • 317 (literal)
Pagina fine
  • 334 (literal)
Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#numeroVolume
  • 17 (literal)
Rivista
Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#note
  • REVIEW (literal)
Note
  • ISI Web of Science (WOS) (literal)
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  • Laboratory of Cell Biology, Istituto Ortopedico Rizzoli, via Di Barbiano 1/10, I-40136 Bologna, Italy; IGM-CNR, Unit of Bologna, c/o IOR, Bologna, Italy; Department of Scienze Anatomiche Umane e Fisiopatologia Apparato Locomotore, University of Bologna, Bologna, Italy. (literal)
Titolo
  • Involvement of prelamin A in laminopathies. (literal)
Abstract
  • The precursor protein of the nuclear lamina constituent lamin A is a 74-kDa protein called prelamin A which undergoes subsequent steps of posttranslational modification at its C-terminal CaaX residue. The unexpected finding that accumulation of unprocessable prelamin A is the molecular basis of the most severe laminopathies so far identified, including Hutchinson–Gilford progeria and restrictive dermopathy, has opened new perspectives in the study of the pathogenic mechanisms causing all lamin A/C–linked disorders, as well as new interest in the analysis of molecular mechanisms regulating prelamin A processing. However, complete knowledge of the cellular pathways affected downstream of prelamin A accumulation is still lacking, but it could give new insights both in normal and pathogenic mechanisms regulated by lamins. In this article, we review the involvement of defects of prelamin A processing in the pathogenesis of a group of laminopathies. In particular, we discuss the possibility that mutations leading to accumulation of particular forms of prelamin A result in specific nuclear abnormalities and impairment of nuclear functions leading to cell senescence or altered metabolism. (literal)
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