Possible Key to Huntington’s Disease Found

A breakdown of the myelin sheaths around the earliest forming tissue in the brain may be the cause of Huntington’s disease, say California researchers.

HD is a rare, inherited neurological disorder in which essential neurons die and patients lose control of their movement, behavior and thinking, in that order.

It is caused by a mutation in a gene called Htt, but what the gene actually did to create the disease was unknown.

The University of California, Los Angeles team, led by George Bartzokis, thinks that Htt’s effect on the protein that nourishes the protective myelin sheaths around neural pathways in the brain may be the culprit. They said it is possible the gene interferes with the protein’s delivery system, which means the myelin sheaths start dying, exposing the neurons and in turn, causing their death.

Myelin breakdown can be detected on magnetic resonance imaging before symptoms of the disease appear, the research team said.

Since HD takes decades to develop, if the researchers are correct, it may be possible to develop medications that could be administered in HD’s very early stages and alter the course of the disease.

The report appears in the current online issue of the journal Neurochemical Research.