The brain's support cells, the so-called glial cells, play a main role in the development of the genetic brain disorder Huntington's disease, for which there is currently no treatment. In a new study, an international group of researchers from the Faculty of Health and Medical Sciences at the University of Copenhagen, among others, has now mapped important, hitherto unknown mechanisms in glial cells in a brain suffering from Huntington's disease. The new research results have been published in the prestigious journal Cell Stem Cell.

'In the study we show that glial cell maturation is severely impaired in patients with Huntington's disease, and this is a major contributor to the abnormalities we see in the brain. This leads to behavioral changes as well as to changes in motor function. The failure of glial maturation causes many of these symptoms, because diseased glial cells cannot support normal neuronal and synaptic function; this means that the communication between neurons is impaired, says the last author of the study. 

They have been researching glial cells and degenerative diseases in the brain, including Huntington's disease, for a number of years, and in this study they set out to determine what happens to glial cells at the molecular level in the Huntington's disease brain. To do so the researchers studied mice into which they had transplanted human glial progenitor cells containing the Huntington gene. These glial progenitor cells are precursors to mature glial cells, and were derived from pluripotent stem cells, using methods that the researchers developed for producing glial cells from stem cells.

Potential of Glial Cell Therapy

'This failure of glial cell maturation appears to be a common element of diseases that involve behavioral abnormalities and psychotic thinking. The unsuccessful glial cell maturation we saw in Huntington's disease is very similar to what we saw in one of our previous studies, where we studied the role of glial cells in schizophrenia. At the same time, our study stresses the potential of glial cell therapy as a possible treatment for Huntington's disease and other similar neurodegenerative diseases', Goldman explains.

Goldman and his colleagues have previously worked with glial cell transplantation, as described in a study from 2016. Here the researchers had transplanted healthy glial cells to mice suffering from Huntington's disease. This prolonged the life expectancy of the mice and alleviated the symptoms of the disease. And one of the next steps for Goldman and his research group is to conduct clinical trials involving transplantation of healthy glial cells to patients with Huntington's disease. They hope to be able to launch these trials within the next couple of years.