MeCP2 dysfunction prevents proper BMP signaling and neural progenitor expansion in brain organoid.

ABSTRACT:

Objectives

Sporadic mutations in MeCP2 are a hallmark of Rett syndrome (RTT). Many RTT brain organoid models have exhibited pathogenic phenotypes such as decreased spine density and small size of soma with altered electrophysiological signals. However, previous models are mainly focused on the phenotypes observed in the late phase and rarely provide clues for the defect of neural progenitors which generate different types of neurons and glial cells.

Methods

We newly established the RTT brain organoid model derived from MeCP2-truncated iPS cells which were genetically engineered by CRISPR/Cas9 technology. By immunofluorescence imaging, we studied the development of NPC pool and its fate specification into glutamatergic neurons or astrocytes in RTT organoids. By total RNA sequ

SUBMITTER: Hong H 

PROVIDER: S-EPMC10351674 | biostudies-literature | 2023 Jul

REPOSITORIES: biostudies-literature