YbeY is Required for Ribosome Small Subunit Assembly and TRNA Processing in Human Mitochondria

Overview

Journal Nucleic Acids Res

Publisher Oxford University Press

Specialty Biochemistry

Date 2021 May 26

PMID 34037799

Citations 8

Authors

Aaron R DSouza

Lindsey Van Haute

Christopher A Powell

Christian D Mutti

Petra Palenikova

Pedro Rebelo-Guiomar

Joanna Rorbach

Michal Minczuk

Affiliations

Soon will be listed here.

Abstract

Mitochondria contain their own translation apparatus which enables them to produce the polypeptides encoded in their genome. The mitochondrially-encoded RNA components of the mitochondrial ribosome require various post-transcriptional processing steps. Additional protein factors are required to facilitate the biogenesis of the functional mitoribosome. We have characterized a mitochondrially-localized protein, YbeY, which interacts with the assembling mitoribosome through the small subunit. Loss of YbeY leads to a severe reduction in mitochondrial translation and a loss of cell viability, associated with less accurate mitochondrial tRNASer(AGY) processing from the primary transcript and a defect in the maturation of the mitoribosomal small subunit. Our results suggest that YbeY performs a dual, likely independent, function in mitochondria being involved in precursor RNA processing and mitoribosome biogenesis. Issue Section: Nucleic Acid Enzymes.

Citing Articles

Molecular basis of human nuclear and mitochondrial tRNA 3' processing.

Bhatta A, Kuhle B, Yu R, Spanaus L, Ditter K, Bohnsack K Nat Struct Mol Biol. 2025; .

PMID: 39747487 DOI: 10.1038/s41594-024-01445-w.

Mitochondrial RNA maturation.

Chrzanowska-Lightowlers Z, Lightowlers R RNA Biol. 2024; 21(1):28-39.

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The catalytic activity of methyltransferase METTL15 is dispensable for its role in mitochondrial ribosome biogenesis.

Mutti C, Van Haute L, Minczuk M RNA Biol. 2024; 21(1):23-30.

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Molecular pathways in mitochondrial disorders due to a defective mitochondrial protein synthesis.

Antolinez-Fernandez A, Esteban-Ramos P, Fernandez-Moreno M, Clemente P Front Cell Dev Biol. 2024; 12:1410245.

PMID: 38855161 PMC: 11157125. DOI: 10.3389/fcell.2024.1410245.

The molecular machinery for maturation of primary mtDNA transcripts.

Vuckovic A, Freyer C, Wredenberg A, Hillen H Hum Mol Genet. 2024; 33(R1):R19-R25.

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