Article
What can we learn on ALS pathophysiology from iPSC-derived motor neurons harbouring TARBDP mutations: a systematic review.
Translational neurodegeneration - 20 Sept 2026
Pasho Elena, Catanese Alberto, Kabashi Edor, Ciura Sorana
Abstract excerpt
The degenerating motor neurons of amyotrophic lateral sclerosis (ALS) patients are characterized by the accumulation of cytoplasmic aggregates, specifically enriched in ubiquitinated TDP-43. Expressed mainly in the nucleus and partially in the cytoplasm to execute its role in RNA metabolism, the exact mechanisms that bring TDP-43 to aggregate in disease have yet to be described. Unfolding these processes could...
Read the complete abstract on PubMedTopics
- Amyotrophic Lateral Sclerosis
- Humans
- Motor Neurons
- Induced Pluripotent Stem Cells
- DNA-Binding Proteins
- Mutation
