TDP-43 seeding mouse models are established by injecting brain extracts from patients affected by TDP-43 proteinopathy. TAR DNA binding protein (TDP-43) encoded by TARDBP gene, is a major protein involved in the pathogenesis of Amyothropic lateral sclerosis (ALS) and  frontotemporal dementia (FTD). Therefore, injection of brain extracts from these diseases in the brain of mice closely mimics the structural features of TDP-43 aggregates found in patients, and allows studying the pathologic spreading of TDP-43 in response to novel compounds as a therapeutic readout.  

As part of our specialised preclinical research services, InnoSer offers research services including efficacy studies in TDP-43 seeding mouse models, using either recombinant and/or patient-derived seeds. Accordingly, brain extracts from both ALS and FTD patients can be used, mimicking the unique disease pathophysiology, with the choice of the brain source extracts ultimately depending on your target disease of interest.