Amyotrophic Lateral Sclerosis (ALS), also known as Lou Gehrig’s disease, affects more than 30,000 Americans. One such treatment is already under development for testing in humans after the current study showed it stopped nerve cell damage in mice with ALS.
The HMS study reveals that the aberrant behavior of an enzyme called RIPK1 damages neuronal axons by disrupting the production of myelin, the soft, gel-like substance that envelopes axons to insulate them from injury.
“Our study not only elucidates the mechanism of axonal injury and death but also identifies a possible protective strategy to counter it by inhibiting the activity of RIPK1,” said the study’s senior investigator, Junying Yuan, the Elizabeth D. Hay Professor of Cell Biology at HMS.
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