Format

Send to

Choose Destination
Biochem J. 2017 Jun 13;474(13):2145-2158. doi: 10.1042/BCJ20170256.

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

Author information

1
Institute for Cell and Molecular Bioscience, Newcastle University, Medical School, Framlington Place, Newcastle upon Tyne NE2 4HH, U.K.
2
The Wellcome Centre for Mitochondrial Research, Institute of Neuroscience, Newcastle University, Medical School, Framlington Place, Newcastle upon Tyne NE2 4HH, U.K.
3
The Wellcome Centre for Mitochondrial Research, Institute of Neuroscience, Newcastle University, Medical School, Framlington Place, Newcastle upon Tyne NE2 4HH, U.K. zofia.chrzanowska-lightowlers@ncl.ac.uk robert.lightowlers@ncl.ac.uk.
4
Institute for Cell and Molecular Bioscience, Newcastle University, Medical School, Framlington Place, Newcastle upon Tyne NE2 4HH, U.K. zofia.chrzanowska-lightowlers@ncl.ac.uk robert.lightowlers@ncl.ac.uk.

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.

KEYWORDS:

mitochondrial gene expression; mitochondrial ribosomes; mitoribosomal maturation; ribosomal RNA maturation; ribosome assembly

PMID:
28512204
PMCID:
PMC5468982
DOI:
10.1042/BCJ20170256
[Indexed for MEDLINE]
Free PMC Article

Supplemental Content

Full text links

Icon for HighWire Icon for PubMed Central
Loading ...
Support Center