Amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig disease, causes nerve cells in the spinal cord and brain to die, eventually leading to paralysis and death. In the April issue of Nature Medicine, Linda Greensmith and colleagues describe a drug that slows the disease in a mouse model of ALS.
About 20% of hereditary cases of ALS are caused by a mutation that disrupts the heat shock response, a mechanism that protects cells from stress. The researchers found that treating mice with a similar mutation with arimoclomol, a drug that induces the expression of heat shock proteins, greatly improves muscle function in the mice and prolongs the survival of their nerve cells. The overall result was a 22% increase in the lifespan of the mice.
The authors suggest that arimoclomol and similar drugs could be used to treat ALS and related neurodegenerative disorders.
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