Multivalent interactions driving RNP dynamics in development and disease

Title Multivalent interactions driving RNP dynamics in development and disease
Acronym RNPdynamics
Website https://www.ucl.ac.uk/ion/research/departments/neuromuscular-diseases/research-groups/rna-networks-group
Start date 2019-09-22
End date 2024-09-21
Sponsor European Research Council - Advanced Grant (ERC-AdG)
Institution University College London

Associated cell lines

Project Description

Ribonucleoprotein complexes (RNPs) play many key regulatory roles in development. Moreover, mutations causing cancer or neurodegenerative diseases, such as amyotrophic lateral sclerosis (ALS), often occur in RNA-binding proteins (RBPs). These mutations are concentrated in the intrinsically disordered regions (IDRs), which play a central role in the control of multivalent RNP assembly and disassembly. Transcriptomic insights into the physiological functions of such multivalent RNP assembly are needed to understand their regulation, or deregulation through disease-causing mutations. Here, we will use human embryonic stem cells to study the mechanisms by which the dynamic multivalent interactions drive RNP remodelling, how such RNP dynamics contributes to neuronal differentiation, and how we could fine-tune the RNP assembly of ALS-mutant RBPs, and thereby ameliorate their toxicity.