TDP-43 prevents retrotransposon activation in the Drosophila motor system through regulation of Dicer-2 activity.

ABSTRACT:

Background

Mutations in the small RNA-binding protein TDP-43 lead to the formation of insoluble cytoplasmic aggregates that have been associated with the onset and progression of amyotrophic lateral sclerosis (ALS), a neurodegenerative disorder affecting homeostasis of the motor system which is also characterized by aberrant expression of retrotransposable elements (RTEs). Although the TDP-43 function was shown to be required in the neurons and glia to maintain the organization of neuromuscular synapses and prevent denervation of the skeletal muscles, the molecular mechanisms involved in physiological dysregulation remain elusive. Here, we address this issue using a null mutation of the TDP-43 Drosophila homolog, TBPH.

Results

Using genome-wide gene expression profiles, we d

SUBMITTER: Romano G 

PROVIDER: S-EPMC7334854 | biostudies-literature | 2020 Jul

REPOSITORIES: biostudies-literature