Novel potential Huntington's treatments

The two populations of glia found in the brain, oligodendrocytes and astrocytes, are dysfunctional in Huntington's disease and may be the primary cause of much of the neuronal pathology observed in the condition, according to research from the University of Rochester Medical Center.

Glia cells play a critical role in maintaining the health of neurons and facilitating the chemical signaling between nerve cells. In Huntington's, glia are unable to perform these functions, leading to a breakdown in communication between neurons and, over time, cell death.

The new study focuses on oligodendrocytes and identifies how the suppression of a specific transcription gene called Tcf7L2 triggers a series of changes that impair the function of oligodendrocyte progenitor cells (OPCs). These cells constantly resupply the brain with oligodendrocytes, which, in turn, refresh the myelin insulation that helps signals travel in the brain more crisply. In Huntington's, OPCs are not able to meet demand, leading to deficient myelination in the brain, which can be observed in Huntington's patients in the form of white matter atrophy. When the researchers overexpressed Tcf7l2 in mice with the Huntington's disease mutation, their OPCs recovered and restored the myelin that had been lost to the disease.

Ref:

Steve Goldman,A TCF7L2-reponsive suppression of both homeostatic and compensatory remyelination in Huntington disease mice,Cell Reports