A protein slows the progression of Charcot's disease in mice

An enzyme similar to that used for treating severe sepsis has slowed in mice the progress of Charcot's disease (amyotrophic lateral sclerosis) according to a study.

This enzyme, called activated protein C or drotrecogin has slowed the death of nerve cells in mice which had been inoculated with the equivalent of a particularly aggressive form of the disease Charcot. Their lives were extended by about 25%, state the authors of this potentially promising research.

Drotrecogin also extended the time during which these animals were able to function normally despite the fact that they showed symptoms of the incurable and fatal desease.

This substance has also slowed the rate of muscle wasting that affects people with amyotrophic lateral sclerosis, also known as Lou Gehrig's disease in the United States, named after a famous player in baseball who died in 1941 .

Although researchers believe that further research should be conducted before this enzyme can be tested in humans suffering from the disease, they are encouraged by the fact that a very similar substance is already used successfully in humans for treat sepsis.

They hope to conduct clinical trials within five years.

"The success of this research is very gratifying and we are hopeful that some forms of drotrecogin prove to be a useful therapy against this disease," said Dr. Berislav Zlokovic, Faculty of Medicine, University of Rochester (New York). He is one of the authors of the research published in the online version of the Journal of Clinical Investigation.

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