Study on animal and patient models – The study, conducted on animal models and on patients, gives hope to identify a cure for ALS, which causes paralysis of the voluntary muscles to even involve the respiratory ones.
Research – The research was coordinated by Valentina Bonetto, from the Negri Irccs Pharmacological Research Institute, with the research group of the Regional Expert Center for ALS of the Molinette Hospital of the City of Health of Turin and the Department of Neuroscience of the University of Turin, led by Andrea Calvo.
Anomalies in a protein – Previous studies had already clarified that the vast majority of ALS patients have abnormalities in the TDP-43 protein, which plays an important role in cellular processes and which, if mutated, causes disease.
The role of the enzyme – The new research has shown that the enzyme cyclophylline A (PPIA) is essential for the proper functioning of TDP-43. In fact, its absence induces a neurodegenerative disease similar to ALS in the animal model and causes the abnormal accumulation of the TPD-43 protein.
The news comes immediately after the announcement, by the Italian Research Foundation for ALS, that Bonetto’s project on PPIA is among those selected by the AriSla 202 call and has also received a contribution from the “Io corro con Giovanni” Association. (founded by friends of Giovanni Longoni, who died of SLA in 2015). The research was mainly funded by the Regional Foundation for Biomedical Research of the Lombardy Region and the Ministry of Health.