Topic: radial glial progenitors

A de novo missense mutation in ceramide synthase 6 (CERS6) was identified by human exome sequencing in a patient with subcortical heterotopia. This is a rare disorder associated with epilepsy and intellectual disability, which may be caused by disturbances in cortical progenitors and /or migrating neurons. Ceramide synthases are important for the biogenesis of ceramides in the endoplasmic reticulum and mitochondria. These are then transferred to the Golgi apparatus for the formation of complex sphingolipids, and in this form further transported to other cell membranes. The major aim of this pr

The mammalian neocortex is populated by different neuronal and glia cell types. During development, excitatory projection neurons and certain glia lineages emerge from radial glial progenitors (RGP). Impairments in RGP progenitor behavior and lineage progression can affect neuron production and maturation, leading to cortical malformations such as micro- or macrocephaly. The Notch signaling pathway instructs stem cell self-renewal and differentiation in a variety of tissues, including the brain. The Notch1 receptor is expressed in RGPs during embryonic development and maintains the progenitor

In the developing cerebral cortex, radial glial progenitors (RGPs) produce all excitatory neurons and glia. Using Mosaic Analysis with Double Markers (MADM), we established a quantitative framework of cortical RGP lineage progression at single-cell resolution in vivo. The extent to which these quantitative aspects of RGP lineage progression are conserved in vitro is unclear. Here we established MADM in embryonic stem cells in order to contrast RGP lineage progression in vivo and in cortical organoids. We found that organoid RGP division behavior was less temporally stereotyped, with both persi