Hospital Universitario de Canarias, Instituto de Investigación Sanitaria de Canarias, ITB-ULL and CIBERNED

New models and mechanisms of TDP-43 proteinopathies

Host: E. Buratti

Hypertrophic cardiomyopathy (HCM) is the most common genetic cardiac disease, and is associated TDP-43 is the central protein involved in amyotrophic lateral sclerosis (ALS) and other TDP-43 proteinopathies, including around half of frontotemporal dementia cases (FTD) and is also thought to be involved in Alzheimer’s disease. Over the last few years, we have developed different mouse models of TDP-43 proteinopathies that we will review here, together with new models that we are currently working on, illuminating TDP-43 functions in vivo. In addition, I would present data on a new functional interaction between TDP-43 and USP10.

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