Laminopathies: multiple disorders arising from defects in nuclear architecture

Parnaik, Veena K. ; Manju, Kaliyaperumal (2006) Laminopathies: multiple disorders arising from defects in nuclear architecture Journal of Biosciences, 31 (3). pp. 405-421. ISSN 0250-5991

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Official URL: http://www.ias.ac.in/jbiosci/sep2006/405.pdf

Related URL: http://dx.doi.org/10.1007/BF02704113

Abstract

Lamins are the major structural proteins of the nucleus in an animal cell. In addition to being essential for nuclear integrity and assembly, lamins are involved in the organization of nuclear processes such as DNA replication, transcription and repair. Mutations in the human lamin A gene lead to highly debilitating genetic disorders that primarily affect muscle, adipose, bone or neuronal tissues and also cause premature ageing syndromes. Mutant lamins alter nuclear integrity and hinder signalling pathways involved in muscle differentiation and adipocyte differentiation, suggesting tissue-specific roles for lamins. Furthermore, cells expressing mutant lamins are impaired in their response to DNA damaging agents. Recent reports indicate that certain lamin mutations act in a dominant negative manner to cause nuclear defects and cellular toxicity, and suggest a possible role for aberrant lamins in normal ageing processes.

Item Type:Article
Source:Copyright of this article belongs to Indian Academy of Sciences.
Keywords:Adipocyte Differentiation; DNA Repair; Lamins; Muscle Differentiation; Nuclear Lamina
ID Code:34721
Deposited On:11 Apr 2011 14:08
Last Modified:17 May 2016 17:36

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