Riluzole partially restores RNA polymerase III complex assembly in cells expressing the leukodystrophy-causative variant POLR3B R103H

Riluzole partially restores RNA polymerase III complex assembly in cells expressing the leukodystrophy-causative variant POLR3B R103H

Abstract The mechanism of assembly of RNA polymerase III (Pol III), the 17-subunit enzyme that synthesizes tRNAs, 5 S rRNA, and other small-nuclear (sn) RNAs in eukaryotes, is not clearly understood. The recent discovery of the HSP90 co-chaperone PAQosome (Particle for Arrangement of Quaternary stru...

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Bibliographic Details
Main Authors: Maxime Pinard, Samaneh Dastpeyman, Christian Poitras, Geneviève Bernard, Marie-Soleil Gauthier, Benoit Coulombe
Format: Article
Language:English
Published: BMC 2022-11-01
Series:Molecular Brain
Subjects:
RNA polymerase III
Protein complex assembly
POLR3-related leukodystrophy
4H leukodystrophy
Riluzole
Drug repurposing
Online Access:https://doi.org/10.1186/s13041-022-00974-z

Internet

https://doi.org/10.1186/s13041-022-00974-z

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