iPSC disease modeling of laminopathies

Guang Hui Liu*, Jiping Yang, Zhichao Ding, Alejandro Ocampo, Jing Qu, Keiichiro Suzuki, Juan Carlos Izpisua Belmonte

*Corresponding author for this work

Research output: Chapter in Book/Report/Conference proceedingChapterpeer-review

1 Scopus citations

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.

Original languageEnglish (US)
Title of host publicationHuman iPS Cells in Disease Modelling
PublisherSpringer Japan
Pages53-67
Number of pages15
ISBN (Electronic)9784431559665
ISBN (Print)9784431559641
DOIs
StatePublished - Jan 1 2016

Bibliographical note

Publisher Copyright:
© Springer Japan 2016.

Keywords

  • Disease model
  • IPSC
  • Laminopathy

ASJC Scopus subject areas

  • General Biochemistry, Genetics and Molecular Biology

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