Related Experiment Video
Updated: Dec 24, 2025

07:14
Optogenetic Phase Transition of TDP-43 in Spinal Motor Neurons of Zebrafish Larvae
Published on: February 25, 2022
6.4K
The Sense of Targeting Nonsense-Mediated Decay in C9-ALS/FTD
1Department of Biochemistry and Biophysics, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, USA.
Neuron
|April 10, 2020
Abstract:
Defective nucleocytoplasmic transport contributes to C9-ALS/FTD, but an inventory of proteins that become redistributed has remained elusive. In this issue of Neuron, Ortega et al. (2020) catalog these redistributed proteins and pinpoint nonsense-mediated decay as a therapeutic target for C9-ALS/FTD.
Related Concept Videos
Nonsense-mediated mRNA Decay
11.5K
The Upf proteins that carry out nonsense-mediated decay (NMD) are found in all eukaryotic organisms, including humans. Each protein has an individual role, but they need to work in collaboration. Upf1 is an ATP-dependent RNA helicase that unwinds the RNA helix. Because Upf1 can unwind any RNA, Upf2 and Upf3 are required to help Upf1 discriminate between nonsense and normal mRNAs.
Usually, Upf3 binds to an Exon Junction Complex (EJC) at mRNA splice sites. If a ribosome fully translates the mRNA,...
Usually, Upf3 binds to an Exon Junction Complex (EJC) at mRNA splice sites. If a ribosome fully translates the mRNA,...
11.5K
Nonsense-mediated mRNA Decay
3.2K
3.2K
Nuclear Export of mRNA
8.5K
Before mRNAs are exported to the cytoplasm, it is crucial to check each mRNA for structural and functional integrity. Eukaryotic cells use several different mechanisms, collectively known as mRNA surveillance, to look for irregularities in mRNAs. Irregular or aberrant mRNA are rapidly degraded by various enzymes. If a defective mRNA escapes the surveillance, it would be translated into a protein which would either be non-functional or not function properly. One of the primary irregularities in...
8.5K

