Slanted Science

Work carried out at several North American research institutes, and headed by Dr Stuart Lipton,has come up with a novel strategy for treating the terrible neurodegenerative condition called Huntington’s Disease (or Huntington’s Chorea).

They used a mouse model of the disease and found that, in the mice at least, disease progress can be halted by a drug currently being used to treat patients with Alzheimer’s.

Huntington’s Disease (aka Huntington’s Chorea) is an horrific, neurodegenerative disease which gradually steals control of movement and cognitive function from its sufferers.

We know what causes it: patients inherit a defective version of a gene called Huntingtin. This version produces a protein molecule which is unable to fold itself into the 3-dimensional shape which it needs to perform its job. The mutant protein is toxic to neurons, and there is widespread death of these cells in various areas of the brain.

What’s been found out in this new research is a mechanism by which the toxicity of mutant huntingtin can be reduced. They’s then gone on to show that a previously-known drug can inhibit the disease’s progress.

The drug is called memantine and is currently used to treat several conditions, including Alzheimer’s Disease, under various brand names (Axura, Namenda, Ebixa, Abixa and Memox). Its general mode of action is to reduce neuronal excitement.

This study has shown that overstimulated neurons (in scientific terms, those which have extrasynaptic activity) are more sensitive to the toxic effects of mutant huntingtin. Furthermore, in the scientists’ laboratory model, memantine was able to reduce this extrasynaptic activity – without disrupting normal, essential synaptic activity – and thus protect neurons from death.

We were unable to find any published reports of this as yet, but the groups’s press release states that:

A small human clinical trial of Memantine for Huntington’s disease has also recently shown positive effects. Larger, international clinical trials are now being planned.

Don’t Hold Your Breath, But This May Lead To: as always, we urge caution when reading about this kind of result. Be optimistic by all means, but please don’t imagine that a miracle cure will emerge within a year or two.

If you are currently facing a Huntington’s diagnosis, this book may provide some assistance in learning more about its causes and available support: