Deficiency of an enzyme, cyclophylline A, induces amyotrophic lateral sclerosis (ALS).
A study by the Mario Negri Institute of Milan and the City of Health of Turin published in the authoritative scientific journal Brain reveals one of the mechanisms that causes the disease.
Conducted on animal models and on patients, the outcome of the study "could be an important first step towards the development of a therapy", say the researchers, for this serious neurodegenerative disease that leads to paralysis of voluntary muscles and even involves respiratory ones. .
The study was coordinated by Valentina Bonetto of the Mario Negri IRCCS Pharmacological Research Institute with the research group of the Regional Expert Center for ALS (CRESLA) of the Molinette Hospital of the City of Health of Turin and of the Department of Neuroscience of the University of Turin. , coordinated by Professor Andrea Calvo. Previous studies had already shown that the vast majority of ALS patients have abnormalities in the TDP-43 protein, which plays an important role in cellular processes and, if mutated, causes disease. The new research has shown that the cyclophylline A (PPIA) enzyme is critical for the proper functioning of TDP-43. In fact, the absence of cyclophylline A in the animal model induces a neurodegenerative disease similar to ALS and causes theabnormal accumulation of the TPD-43 protein. The news comes immediately after the announcement by the Italian Research Foundation for Amyotrophic Lateral Sclerosis (AriSLA) that Dr. Bonetto's project on PPIA (GATTALS) is among those selected by the AriSLA 2021 Call and has also received a contribution from Association 'Io Corro con Giovanni'. The research was mainly funded by the Regional Foundation for Biomedical Research of the Lombardy Region (TRANS-ALS Project) and the Ministry of Health.I'm running with Giovanni '. The research was mainly funded by the Regional Foundation for Biomedical Research of the Lombardy Region (TRANS-ALS Project) and the Ministry of Health.I'm running with Giovanni '. The research was mainly funded by the Regional Foundation for Biomedical Research of the Lombardy Region (TRANS-ALS Project) and the Ministry of Health.