Spinal motor neuron protein supersaturation patterns are associated with inclusion body formation in ALS.

ABSTRACT: Amyotrophic lateral sclerosis (ALS) is a heterogeneous degenerative motor neuron disease linked to numerous genetic mutations in apparently unrelated proteins. These proteins, including SOD1, TDP-43, and FUS, are highly aggregation-prone and form a variety of intracellular inclusion bodies that are characteristic of different neuropathological subtypes of the disease. Contained within these inclusions are a variety of proteins that do not share obvious characteristics other than coaggregation. However, recent evidence from other neurodegenerative disorders suggests that disease-affected biochemical pathways can be characterized by the presence of proteins that are supersaturated, with cellular concentrations significantly greater than their solubilities. Here, we show that the proteins tha

SUBMITTER: Ciryam P 

PROVIDER: S-EPMC5441770 | biostudies-literature | 2017 May

REPOSITORIES: biostudies-literature