Cytosolic N6AMT1-dependent translation supports mitochondrial RNA processing.
Details
Serval ID
serval:BIB_538A9FE626ED
Type
Article: article from journal or magazin.
Collection
Publications
Institution
Title
Cytosolic N6AMT1-dependent translation supports mitochondrial RNA processing.
Journal
Proceedings of the National Academy of Sciences of the United States of America
ISSN
1091-6490 (Electronic)
ISSN-L
0027-8424
Publication state
Published
Issued date
19/11/2024
Peer-reviewed
Oui
Volume
121
Number
47
Pages
e2414187121
Language
english
Notes
Publication types: Journal Article
Publication Status: ppublish
Publication Status: ppublish
Abstract
Mitochondrial biogenesis relies on both the nuclear and mitochondrial genomes, and imbalance in their expression can lead to inborn errors of metabolism, inflammation, and aging. Here, we investigate N6AMT1, a nucleo-cytosolic methyltransferase that exhibits genetic codependency with mitochondria. We determine transcriptional and translational profiles of N6AMT1 and report that it is required for the cytosolic translation of TRMT10C (MRPP1) and PRORP (MRPP3), two subunits of the mitochondrial RNAse P enzyme. In the absence of N6AMT1, or when its catalytic activity is abolished, RNA processing within mitochondria is impaired, leading to the accumulation of unprocessed and double-stranded RNA, thus preventing mitochondrial protein synthesis and oxidative phosphorylation, and leading to an immune response. Our work sheds light on the function of N6AMT1 in protein synthesis and highlights a cytosolic program required for proper mitochondrial biogenesis.
Keywords
Cytosol/metabolism, Mitochondria/metabolism, Mitochondria/genetics, Humans, Protein Biosynthesis, RNA, Mitochondrial/metabolism, RNA, Mitochondrial/genetics, Methyltransferases/metabolism, Methyltransferases/genetics, Mitochondrial Proteins/metabolism, Mitochondrial Proteins/genetics, Ribonuclease P/metabolism, Ribonuclease P/genetics, RNA Processing, Post-Transcriptional, Animals, Organelle Biogenesis, Mice, Oxidative Phosphorylation, OXPHOS, RNA processing, mitochondria, mitochondrial RNA granules, translation
Pubmed
Open Access
Yes
Create date
02/12/2024 9:31
Last modification date
03/12/2024 7:08