Cortical neurons derived from human pluripotent stem cells lacking FMRP display altered spontaneous firing patterns.

ABSTRACT:

Background

Fragile X syndrome (FXS), a neurodevelopmental disorder, is a leading monogenetic cause of intellectual disability and autism spectrum disorder. Notwithstanding the extensive studies using rodent and other pre-clinical models of FXS, which have provided detailed mechanistic insights into the pathophysiology of this disorder, it is only relatively recently that human stem cell-derived neurons have been employed as a model system to further our understanding of the pathophysiological events that may underlie FXS. Our study assesses the physiological properties of human pluripotent stem cell-derived cortical neurons lacking fragile X mental retardation protein (FMRP).

Methods

Electrophysiological whole-cell voltage- and current-clamp recordings were performed on two

SUBMITTER: Das Sharma S 

PROVIDER: S-EPMC7304215 | biostudies-literature | 2020 Jun

REPOSITORIES: biostudies-literature