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YbeY is required for ribosome small subunit assembly and tRNA processing in human mitochondria.

Published version
Peer-reviewed

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Type

Article

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Authors

D'Souza, Aaron R 
Van Haute, Lindsey 
Powell, Christopher A 
Mutti, Christian D 
Páleníková, Petra 

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.

Description

Keywords

Mitochondria, Humans, Ribonucleases, Mitochondrial Proteins, RNA, Transfer, Immunohistochemistry, Sequence Alignment, Cell Survival, Protein Biosynthesis, RNA Processing, Post-Transcriptional, Amino Acid Sequence, Mass Spectrometry, Ribosome Subunits, Small, Gene Knockout Techniques, HEK293 Cells, Mitochondrial Ribosomes

Journal Title

Nucleic acids research

Conference Name

Journal ISSN

0305-1048

Volume Title

49

Publisher

Sponsorship
Medical Research Council (MC_UU_00015/4)