Modeling human congenital disorders with neural crest developmental defects using patient-derived induced pluripotent stem cells

Research output: Contribution to journalReview articlepeer-review

Abstract

The neural crest is said to be the fourth germ layer in addition to the ectoderm, mesoderm and endoderm because of its ability to differentiate into a variety of cells that contribute to the various tissues of the vertebrate body. Neural crest cells (NCCs) can be divided into three functional groups: cranial NCCs, cardiac NCCs and trunk NCCs. Defects related to NCCs can contribute to a broad spectrum of syndromes known as neurocristopathies. Studies on the neural crest have been carried out using animal models such as Xenopus, chicks, and mice. However, the precise control of human NCC development has not been elucidated in detail due to species differences. Using induced pluripotent stem cell (iPSC) technology, we developed an in vitro disease model of neurocristopathy by inducing the differentiation of patient-derived iPSCs into NCCs and/or neural crest derivatives. It is now possible to address complicated questions regarding the pathogenetic mechanisms of neurocristopathies by characterizing cellular biological features and transcriptomes and by transplanting patient-derived NCCs in vivo. Here, we provide some examples that elucidate the pathophysiology of neurocristopathies using disease modeling via iPSCs.

Original languageEnglish
Pages (from-to)275-280
Number of pages6
JournalRegenerative Therapy
Volume18
DOIs
Publication statusPublished - 2021 Dec

Keywords

  • Disease modeling
  • iPS cells derived neural crest cells
  • Neurocristopathy

ASJC Scopus subject areas

  • Biomaterials
  • Biomedical Engineering
  • Developmental Biology

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