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Fundamental research

25 - 04 - 2017

Mice to understand ALS

Amyotrophic lateral sclerosis is a degeneration of the motor neurons which leads to the weakening and then hardening of the muscles. The most severe form is due to a mutation of the gene coding for a regulatory neuronal protein called FUS. The "Delocalization" of this protein from the nucleus to the cytoplasm could cause the death of motoneurons.

In order to reinforce this hypothesis, two mouse models have been developed which show either an absence or an accumulation of the FUS protein in the cytoplasm. When the protein is present in the cytoplasm, mice have motor neuron deficiency.

These results confirm that the accumulation of FUS in the cytoplasm leads to the death of motor neurons and paves the way for novel treatments against ALS that would inhibit FUS activity in the cytoplasm

 

Thierry Battmann