Three years: this is the average period of life expectancy when Charcot’s disease, or amyotrophic lateral sclerosis (ALS), declares itself, as France Assos Santé reminds us. We do not yet know how to cure it, but research continues to advance on the issue, as evidenced by a recently published study. in the review Neuron.
As a reminder, ALS is a neurodegenerative disease. She is born from adegeneration of motor neurons which are the cells that control our voluntary muscles and allow us to movewalk, talk, breathe”, explains France Assos Health. Little by little, the patients are affected by paralysis, until they can no longer breathe.
Block a protein responsible for neuronal dysfunction
Of the Harvard and Boston scientists studied the immune mechanism involved in the destruction of these neurons in connection with mobility: the body thinks there is a danger and attacks the cells via an inflammatory reaction (pyroptosis) using proteins called gasdermines. In the brains of people with Charcot, there is a certain amount of gasdermin E, which leads to cellular retraction, causing neurodegeneration.
So they created models of these motoneurons, mimicking the reaction produced by ALS and tried to block the action of this gasdermin E. They then realized that the alteration of the cells was not taking place. These results were then confirmed in mice, whose symptoms of ALS were slowed down.
Although they do not yet know how to precisely target this type of gasdermine in the brain, the scientists nevertheless pointed out that this is an interesting advance, which could perhaps one day give rise to a treatment for slow down the progression of Charcot’s disease, by slowing down neuronal dysfunction.
Sources: Harvard, Neuron, France Assos Health
