Disulfide cross-linked multimers of TDP-43 and spinal motoneuron loss in a TDP-43^A315T ALS/FTD mouse model.

ABSTRACT: Tar DNA binding protein 43 (TDP-43) is the principal component of ubiquitinated protein inclusions present in nervous tissue of most cases of both amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). Previous studies described a TDP-43^A315T transgenic mouse model that develops progressive motor dysfunction in the absence of protein aggregation or significant motoneuron loss, questioning its validity to study ALS. Here we have further characterized the course of the disease in TDP-43^A315T mice using a battery of tests and biochemical approaches. We confirmed that TDP-43 mutant mice develop impaired motor performance, accompanied by progressive body weight loss. Significant differences were observed in life span between genders, where females survived

SUBMITTER: Bargsted L 

PROVIDER: S-EPMC5660202 | biostudies-literature | 2017 Oct

REPOSITORIES: biostudies-literature