• Open Access

Mouse models of laminopathies

Authors


Correspondence

Kan Cao, Ph.D., Department of Cell Biology and Molecular Genetics, 2114 Bioscience Research Building, University of Maryland, College Park, MD 20742, USA. Tel. 301-405-3016; fax 301-405-0745; e-mail: kcao@umd.edu

Abstract

The A- and B-type lamins are nuclear intermediate filament proteins in eukaryotic cells with a broad range of functions, including the organization of nuclear architecture and interaction with proteins in many cellular functions. Over 180 disease-causing mutations, termed ‘laminopathies,’ have been mapped throughout LMNA, the gene for A-type lamins in humans. Laminopathies can range from muscular dystrophies, cardiomyopathy, to Hutchinson–Gilford progeria syndrome. A number of mouse lines carrying some of the same mutations as those resulting in human diseases have been established. These LMNA-related mouse models have provided valuable insights into the functions of lamin A biogenesis and the roles of individual A-type lamins during tissue development. This review groups these LMNA-related mouse models into three categories: null mutants, point mutants, and progeroid mutants. We compare their phenotypes and discuss their potential implications in laminopathies and aging.

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