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Human iPS Cells in Disease Modelling pp 53–67Cite as

iPSC Disease Modeling of Laminopathies

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Abstract

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.

Guang-Hui Liu and Jingping Yang contributed equally with all other contributors.

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Acknowledgments

This work was supported by the National Basic Research Program of China (973 Program, 2015CB964800; 2014CB910503; 2014CB964600), the Strategic Priority Research Program of the Chinese Academy of Sciences (XDA01020312), the Beijing Natural Science Foundation (7141005; 5142016), the National High Technology Research and Development Program of China (2015AA020307), the National Natural Science Foundation of China (NSFC: 81330008; 31222039; 31201111; 81371342; 81300261; 81300677; 81271266; 81471414; 81422017; 81401159; 31322039), the Program of Beijing Municipal Science and Technology Commission (Z151100003915072), the Key Research Program of the Chinese Academy of Sciences (KJZDEW-TZ-L05), the Thousand Young Talents program of China, National Laboratory of Biomacromolecules (012kf02;2013kf05;2013kf11;2014kf02;2015kf10), the State Key Laboratory of Drug Research (SIMM1302KF-17).

JCIB was supported by G. Harold and Leila Y. Mathers Charitable Foundation, The Leona M. and Harry B. Helmsley Charitable Trust.

The Glenn Foundation and Universidad Católica San Antonio de Murcia (UCAM).

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    Authors and Affiliations

    1. National Laboratory of Biomacromolecules, Institute of Biophysics, Chinese Academy of Sciences, Beijing, 100101, China

      Guang-Hui Liu, Jiping Yang & Zhichao Ding

    2. State Key Laboratory of Stem Cell and Reproductive Biology, Institute of Zoology, Chinese Academy of Sciences, Beijing, 100101, China

      Jiping Yang & Jing Qu

    3. Gene Expression Laboratory, Salk Institute for Biological Studies, 10010 N. Torrey Pines Rd, La Jolla, CA, 92037, USA

      Alejandro Ocampo, Keiichiro Suzuki & Juan Carlos Izpisua Belmonte

    4. Universidad Católica San Antonio de Murcia (UCAM), Campus de los Jerónimos, N° 135, Guadalupe, 30107, Murcia, Spain

      Keiichiro Suzuki

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    1. Guang-Hui Liu
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    Correspondence to Guang-Hui Liu or Juan Carlos Izpisua Belmonte .

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    1. Keio University School of Medicine, Department of Cardiology, Tokyo, Tokyo, Japan

      Keiichi Fukuda

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    Liu, GH. et al. (2016). iPSC Disease Modeling of Laminopathies. In: Fukuda, K. (eds) Human iPS Cells in Disease Modelling. Springer, Tokyo. https://doi.org/10.1007/978-4-431-55966-5_5

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