Movement and Memory: TDP-43 Pathology in ALS-FTD

Abstract

Characterized by the progressive neurodegeneration of the motor system, amyotrophic lateral sclerosis (ALS) presents no features starkly reminiscent of dementia. However, significant clinical overlap between ALS and frontotemporal dementia (FTD), a type of non-Alzheimer’s dementia, has been identified in research, leading to a recognized spectrum of disease along which both diseases fall. This spectrum is typically unified by a common pattern of TAR DNA binding protein (TDP-43) proteinopathy or fused in sarcoma (FUS), meaning these proteins are commonly found misfolded as aggregates in diseased cells. The specific diagnosis of overlapping ALS and FTD, referred to as ALS-frontotemporal spectrum disorder (ALS-FTSD), presents a particularly intriguing, though under-investigated, area of research on a pathology that is expressed both behaviorally and physically.

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