The human RNA-binding protein RBFA promotes the maturation of the mitochondrial ribosome.
Biochem J
; 474(13): 2145-2158, 2017 06 13.
Article
em En
| MEDLINE
| ID: mdl-28512204
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.
Palavras-chave
Texto completo:
1
Bases de dados:
MEDLINE
Assunto principal:
Proteínas Ribossômicas
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RNA Ribossômico
/
Proteínas de Ligação a RNA
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Proteínas de Escherichia coli
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Proteínas Mitocondriais
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Ribossomos Mitocondriais
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Mitocôndrias
Limite:
Humans
Idioma:
En
Revista:
Biochem J
Ano de publicação:
2017
Tipo de documento:
Article
País de afiliação:
Reino Unido