Laminopathies: Involvement of structural nuclear proteins in the pathogenesis of an increasing number of human diseases

✍ Scribed by Nadir M. Maraldi; Stefano Squarzoni; Patrizia Sabatelli; Cristina Capanni; Elisabetta Mattioli; Andrea Ognibene; Giovanna Lattanzi

• Book ID: 102313508
• Publisher: John Wiley and Sons
• Year: 2005
• Tongue: English
• Weight: 195 KB
• Volume: 203
• Category: Article
• ISSN: 0021-9541
• DOI: 10.1002/jcp.20217

✦ Synopsis

Abstract

Just at the beginning of the millennium the neologism laminopathies has been introduced in the scientific vocabulary. An exponential increase of interest on the subject started concomitantly, so that a formerly quite neglected group of rare human diseases is now widely investigated. This review will cover the history of the identification of the molecular basis for fourteen (since now) hereditary diseases arising from defects in genes that encode nuclear envelope and nuclear lamina‐associated proteins and will also consider the hypotheses that can account for the role of structural nuclear proteins in the pathogenesis of diseases affecting a wide spectrum of tissues. © 2004 Wiley‐Liss, Inc.