Rogier MinNeuroscience Symposium
The Neuroscience Symposia are organized weekly by the Netherlands Institute for Neuroscience. The presentations are given by researchers from the institute or by guest speakers. The title and content of the symposium is usually made known in the week prior to the presentation.
Colloquium room – Netherlands Institute for Neuroscience
Dr. Rogier Min,
Glial (patho)physiology group, Dept. of Child Neurology (Amsterdam UMC, location VUmc) & Dept. of Integrative Neurophysiology (CNCR, VU).
4:00 pm – Causes and consequences of disturbed brain ion and water homeostasis in the white matter disease MLC
5:00 pm – Discussion and drinks
Electrical activity in the brain is the basis of all our thoughts and actions. It is caused by movement of positively and negatively charged ions between intracellular and extracellular compartments, and crucially depends on maintaining a correct distribution of these ions. Therefore, disrupted ion and water homeostasis has severe consequences. Despite this fact, much is unclear about how homeostasis is achieved. Astrocytes play a crucial role in controlling the flow of water and ions in the brain. Astrocyte dysfunction leads to chronic white matter swelling in a group of neurological diseases, and MLC (Megalencephalic Leukoencephalopathy with subcortical Cysts) can be seen as a prototype for these diseases. Affected children develop a large head in their first year of life, mainly due to intramyelinic edema. They become ataxic and wheelchair bound, cognitive disabilities and epilepsy are common, and life expectancy is shortened. Most patients carry mutations in the gene MLC1, which encodes a membrane protein of unknown function that is mainly present in astrocyte endfeet. In this talk, I will discuss what we have learned about disrupted astrocyte physiology in MLC by studying patient cells and mouse models for the disease. Additionally, I will show what the consequences of this disruption are for neuronal network functioning.