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Critical Reviews™ in Eukaryotic Gene Expression
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ISSN Imprimir: 1045-4403
ISSN En Línea: 2162-6502

Critical Reviews™ in Eukaryotic Gene Expression

DOI: 10.1615/CritRevEukarGeneExpr.v17.i4.50
pages 317-334

Involvement of Prelamin A in Laminopathies

Nadir M. Maraldi
ITOI-CNR, Unit of Bologna, c /o IOR, Via di Barbiano 1/10; Laboratory of Biologia Cellulare e Microscopia Elettronica, IOR; and Department of Scienze Anatomiche Umane e Fisiopatologia Apparato Locomotore, University of Bologna, Italy
Giovanna Lattanzi
ITOI-CNR, Unit of Bologna, c /o IOR, Via di Barbiano 1/10, Bologna, Italy

SINOPSIS

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.


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