There are more than 30,000 people in the United States that cope with Lou Gehrig’s disease, also known as amyotrophic lateral sclerosis (ALS). ALS destroys the nerves that control movement, and eventually, the ability to breathe. It is caused by alterations in a gene called C9ORF72. This gene is also responsible for cognitive impairment called FTD or Fronto-temporal Dementia that can cause personality changes.

Researchers at Mayo Clinic now have a model to attack the mutations in C9ORF72. This is a significant step in understanding how ALS progresses. Mice with the defective gene show the same symptoms as humans as they age. They get hyperactive and show movement problems. Their smaller than normal  brains also showed protein clusters that are seen in humans as well.

Finding these protein clusters was unexpected and may lead to the opportunity to better define ALS therapies.

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