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Establishment of a Gorlin syndrome model from induced neural progenitor cells exhibiting constitutive GLI1 expression and high sensitivity to inhibition by smoothened (SMO)

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The hedgehog signaling pathway is a vital factor for embryonic development and stem cell maintenance. Dysregulation of its function results in tumor initiation and progression. The aim of this research… Click to show full abstract

The hedgehog signaling pathway is a vital factor for embryonic development and stem cell maintenance. Dysregulation of its function results in tumor initiation and progression. The aim of this research was to establish a disease model of hedgehog-related tumorigenesis with Gorlin syndrome-derived induced pluripotent stem cells (GS-iPSCs). Induced neural progenitor cells from GS-iPSCs (GS-NPCs) show constitutive high GLI1 expression and higher sensitivity to smoothened (SMO) inhibition compared with wild-type induced neural progenitor cells (WT-NPCs). The differentiation process from iPSCs to NPCs may have similarity in gene expression to Hedgehog signal-related carcinogenesis. Therefore, GS-NPCs may be useful for screening compounds to find effective drugs to control Hedgehog signaling activity. The hedgehog pathway is pivotal in basal cell carcinoma and medulloblastoma development. Gorlin syndrome-derived, induced pluripotent stem cells (iPSCs) carrying a heterozygous mutation in PTCH1, a hedgehog target gene, exhibited high GLI1 expression and enhanced sensitivity to a hedgehog pathway inhibitor after neural differentiation. These iPSCs can serve as a model for hedgehog pathway-related tumors.

Keywords: neural progenitor; progenitor cells; hedgehog; induced neural; expression; gorlin syndrome

Journal Title: Laboratory Investigation
Year Published: 2019

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