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Myelin Associated With Early Evolution of Huntington's Disease

A new study has found that a breakdown of myelin is associated with the early evolution of Huntington's disease. Last month, Dr. George Bartzokis, director of the UCLA Memory Disorders and Alzheimer's Disease Clinic, suggested in the journal Alzheimer's and Dementia that the collapse of a type of myelin that develops late in life promotes the enlargement of toxic amyloid plaques long associated with Alzheimer's disease. Myelin is the "insulation" that wraps around nerve axons in the brain.

Now, in a new report presently online in the journal Neurochemical Research, Bartzokis turns his concentration to Huntington's disease. Again, he suggests that a breakdown of myelin is the cause, but with a twist - it is the myelin that develops early in the formation of the brain that breaks down ahead of time and ultimately leads to the disease's symptoms.

Huntington's disease (HD) is an uncommon, inherited neurological disorder that eventually deprives individuals of their ability to be in charge of their movement, behaviour and thinking. It affects around 30,000 people in the U.S., with another 150,000 at risk.

While it is known that HD is caused by a mutation in a gene called Huntingtin (Htt), the correct mechanism by which the Htt gene causes or contributes to neuronal cell death and HD symptoms remains uncertain. Bartzokis' research suggests that it is Htt's influence on myelin that may prove to be the cause.

The earliest parts of the developing brain include systems of neurons that control movement and behaviour. These neurons have long axons, finger-like projections that serve as the main transmission lines of the nervous system, covered with thick myelin sheaths. The sheaths are nourished by an ongoing supply of a protein called brain-derived neurotrophic factor, which travels down a neuron's axon.

Bartzokis believes that the Htt gene gets in the way of this nourishment-delivery system, causing a
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