A chimeric mouse model to study human iPSC-derived neurons: the case of a truncating SHANK3 mutation.

ABSTRACT: Using human induced pluripotent stem cells (iPSC), recent studies have shown that the events underlying autism spectrum disorders (ASD) can occur during neonatal development. We previously analyzed the iPSC-derived pyramidal cortical neurons of a subset of patients with ASD carrying de novo heterozygous mutations in postsynaptic SHANK3 protein, in culture. We reported altered spinogenesis of those neurons. The transplantation of human iPSC-derived neuronal precursors into mouse brain represents a novel option for in vivo analysis of mutations affecting the human brain. In this study, we transplanted the neuronal precursor cells (NPC) into the cortex of newborn mice to analyze their integration and maturation at early stages of development and studied axonal projections of transplanted huma

SUBMITTER: Vitrac A 

PROVIDER: S-EPMC7414912 | biostudies-literature | 2020 Aug

REPOSITORIES: biostudies-literature