Article
Hyperubiquitylation of DNA helicase RECQL4 by E3 ligase MITOL prevents mitochondrial entry and potentiates mitophagy.
The Journal of biological chemistry - 1 Sept 2023
Hussain Mansoor, Mohammed Aftab, Saifi Shabnam, Priya Swati, Sengupta Sagar
Abstract excerpt
Mutations in the DNA helicase RECQL4 lead to Rothmund-Thomson syndrome (RTS), a disorder characterized by mitochondrial dysfunctions, premature aging, and genomic instability. However, the mechanisms by which these mutations lead to pathology are unclear. Here we report that RECQL4 is ubiquitylated by a mitochondrial E3 ligase, MITOL, at two lysine residues (K1101, K1154) via K6 linkage. This ubiquitylation...
Read the complete abstract on PubMedTopics
Share this publication in a Topic to start or enrich a Post.
