iPSC Disease Modeling of Laminopathies

Nuclear lamins, the major components of the nuclear lamina, participate in maintaining the nuclear structure and are involved in essential cellular processes, including DNA replication and chromatin organization. To date, more than 15 human diseases classified as laminopathies have been linked to mutations in components of the nuclear lamina. With the development of induced pluripotent stem cell (iPSC) and targeted gene editing technologies, novel disease models of laminopathies have been generated. These models will improve our understanding of the pathological mechanism of laminopathies and may eventually lead to the discovery of new therapeutic approaches for the treatment of these devastating diseases.