[HTML][HTML] TDP-43 proteinopathy and ALS: insights into disease mechanisms and therapeutic targets

EL Scotter, HJ Chen, CE Shaw - Neurotherapeutics, 2015 - Elsevier
Therapeutic options for patients with amyotrophic lateral sclerosis (ALS) are currently
limited. However, recent studies show that almost all cases of ALS, as well as tau-negative
frontotemporal dementia (FTD), share a common neuropathology characterized by the
deposition of TAR-DNA binding protein (TDP)-43-positive protein inclusions, offering an
attractive target for the design and testing of novel therapeutics. Here we demonstrate how
diverse environmental stressors linked to stress granule formation, as well as mutations in …