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Mutations causing amyotrophic lateral sclerosis (ALS) often affect the condensation properties of RNA-binding proteins (RBPs). However, the role of RBP condensation in the specificity and function of protein-RNA complexes remains unclear. We created a series of TDP-43 C-terminal domain (CTD) variants that exhibited a gradient of low to high condensation propensity, as observed in vitro and by nuclear mobility and foci formation. Notably, a capacity for condensation was required for efficient TDP-43 assembly on subsets of RNA-binding regions, which contain unusually long clusters of motifs of characteristic types and density. These "binding-region condensates" are promoted by homomeric CTD-driven interactions and required for efficient regulation of a subset of bound transcripts, including autoregulation of TDP-43 mRNA. We establish that RBP condensation can occur in a binding-region-specific manner to selectively modulate transcriptome-wide RNA regulation, which has implications for remodeling RNA networks in the context of signaling, disease, and evolution.

Original publication

DOI

10.1016/j.cell.2021.07.018

Type

Journal article

Journal

Cell

Publication Date

09/2021

Volume

184

Pages

4680 - 4696.e22

Addresses

The Francis Crick Institute, 1 Midland Road, London NW1 1AT, UK; Department of Neuromuscular Diseases, UCL Queen Square Institute of Neurology, Queen Square, London WC1N 3BG, UK. Electronic address: martina.hallegger@crick.ac.uk.

Keywords

Hela Cells, Cell Nucleus, Humans, RNA-Binding Proteins, DNA-Binding Proteins, RNA, RNA, Messenger, 3' Untranslated Regions, Poly A, Sequence Deletion, Base Sequence, Protein Binding, Homeostasis, Mutation, Point Mutation, Phase Transition, Protein Multimerization, HEK293 Cells, Nucleotide Motifs