Astrocyte-forebrain co-cultures: an in vitro model for studying the role of astrocytes in childhood white matter disorders

Extract

Childhood white matter disorders are neurological disorders involving abnormalities in the white matter of the brain. affected children experience motor and cognitive disabilities and often die young. The disorders are most often genetic, are rare, but overall affect around 1:1000-2000 children, indicating the need for a generally applicable in vitro model. It is clear that disturbances in deposition or loss of myelin occur. However, increasing evidence indicates that axonal damage may play an even more central role. It is thought that glial cells, specifically astrocytes and oligodendrocytes, give less axonal trophic support. These cells are selectively affected in Vanishing white matter disease (VWM) and Megalencephalic leukoencephalopathy with subcortical cysts (MLC). We aim to asses if and how astrocyte (dys)function influences axonal integrity and function. …