The human RNA-binding protein RBFA promotes the maturation of the mitochondrial ribosome

The Biochemical Journal
Agata RozanskaRobert N Lightowlers

Abstract

Accurate assembly and maturation of human mitochondrial ribosomes is essential for synthesis of the 13 polypeptides encoded by the mitochondrial genome. This process requires the correct integration of 80 proteins, 1 mt (mitochondrial)-tRNA and 2 mt-rRNA species, the latter being post-transcriptionally modified at many sites. Here, we report that human ribosome-binding factor A (RBFA) is a mitochondrial RNA-binding protein that exerts crucial roles in mitoribosome biogenesis. Unlike its bacterial orthologue, RBFA associates mainly with helices 44 and 45 of the 12S rRNA in the mitoribosomal small subunit to promote dimethylation of two highly conserved consecutive adenines. Characterization of RBFA-depleted cells indicates that this dimethylation is not a prerequisite for assembly of the small ribosomal subunit. However, the RBFA-facilitated modification is necessary for completing mt-rRNA maturation and regulating association of the small and large subunits to form a functional monosome implicating RBFA in the quality control of mitoribosome formation.

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Citations

Jul 17, 2020·Biomolecular NMR Assignments·Andreas SchedlbauerSean R Connell
Oct 1, 2020·Cells·Ivan LaptevPetr Sergiev
Aug 8, 2018·Nucleic Acids Research·Elena LavdovskaiaRicarda Richter-Dennerlein
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Jan 10, 2021·Trends in Cell Biology·Priyanka MaitiRicarda Richter-Dennerlein
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May 1, 2021·International Journal of Molecular Sciences·Maria Isabel G Lopez SanchezJoanna Rorbach
May 1, 2021·Biochemical Society Transactions·Zhen LiaoAlexandre Smirnov
Jun 6, 2021·Science Advances·Andreas SchedlbauerSean R Connell
Aug 8, 2021·International Journal of Molecular Sciences·Marta Jedynak-SlyvkaRoman J Szczesny
Aug 3, 2021·Mitochondrion·Taru HilanderHongxia Zhao

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Methods Mentioned

BETA
GTPases
immunoprecipitation
CLIP
NMR
transfection
GTPase
immunoprecipitations
X-ray

Software Mentioned

ImageQuant
ClustalW
IonTorrent
Coot
GeneCards

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