核纤层蛋 z-bo 2020-10-31 476

　　The LMNA gene encodes type A nuclear lamin (lamin), and its mutation leads to a series of complex and diverse genetic diseases. At present, people have discovered more than 900 LMNA gene mutations, these mutations cause a variety of phenotypes of laminopathy (laminopathies).

　　such as: mandibuloacral dysplasia (MAD), Emery-Dreifuss muscular dystrophy (EDMD), Hutchinson-Gilford progeria syndrome (HGPS), etc. In order to better understand the molecular mechanism of laminopathy and the screening of therapeutic drugs, researchers have constructed a series of mouse strains with LMNA gene mutations. These mouse models are designed to understand the function of laminin and its effects in individuals. The role in growth and development provides valuable research materials.

　　This article reviews the mouse models of laminopathy and discusses their significance in laminopathy and physiological aging.