Mice study points to glia cells as potential therapy target for Huntington’s disease

By The Science Advisory Board staff writers

August 31, 2022 -- A mice study by University of Rochester Medical Center (URMC) researchers has reinforced the potential of targeting glia cells -- important support cells found in the brain -- for the treatment of Huntington’s disease, and possibly outright replacement.

Glia cells, such as oligodendrocytes, play a critical role in maintaining the health of neurons and facilitating the chemical signaling between nerve cells. However, in Huntington's disease, glia cells are unable to perform these functions which leads to a breakdown in communication between neurons and ultimately cell death.

Using RNA sequencing and proteomic analysis, the URMC researchers investigated the role of oligodendrocyte progenitor cell (OPC) dysfunction. In their mice study, published August 30 in the journal Cell Reports, they identified how the suppression of a specific transcription gene called Tcf7L2 triggers a series of changes that impair the function of OPCs.

When the researchers overexpressed Tcf7L2 in mice with the Huntington's disease mutation, the OPCs recovered and restored the myelin -- the insulating layer or sheath that forms around nerves -- that had been lost to the disease. The researchers contend that their findings put new therapies within reach, arguing that replacing or "fixing" defective glia cells may prove a far easier proposition than replenishing lost neurons.

"While the loss of neurons gives rise to the symptoms and the ultimate fatal nature of the disease, reversing glial dysfunction may give us an opportunity to intervene early in the course of the disease, keeping neurons healthy for longer and slowing disease progression," Abdellatif Benraiss, PhD, first author of the study and a research associate professor in URCM's Department of Neurology, said in a statement.

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