Abstract:
:The human mitochondrial translation apparatus, which synthesizes the core subunits of the oxidative phosphorylation system, is of central interest as mutations in several genes encoding for mitoribosomal proteins or translation factors cause severe human diseases. Little is known, how this complex machinery assembles from nuclear-encoded protein components and mitochondrial-encoded RNAs, and which ancillary factors are required to form a functional mitoribosome. We have characterized the human Obg protein GTPBP10, which associates specifically with the mitoribosomal large subunit at a late maturation state. Defining its interactome, we have shown that GTPBP10 is in a complex with other mtLSU biogenesis factors including mitochondrial RNA granule components, the 16S rRNA module and late mtLSU assembly factors such as MALSU1, SMCR7L, MTERF4 and NSUN4. GTPBP10 deficiency leads to a drastic reduction in 55S monosome formation resulting in defective mtDNA-expression and in a decrease in cell growth. Our results suggest that GTPBP10 is a ribosome biogenesis factor of the mtLSU required for late stages of maturation.
journal_name
Nucleic Acids Resjournal_title
Nucleic acids researchauthors
Lavdovskaia E,Kolander E,Steube E,Mai MM,Urlaub H,Richter-Dennerlein Rdoi
10.1093/nar/gky701subject
Has Abstractpub_date
2018-09-19 00:00:00pages
8471-8482issue
16eissn
0305-1048issn
1362-4962pii
5063820journal_volume
46pub_type
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