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Science. 2014 Nov 7;346(6210):718-722. doi: 10.1126/science.1258026. Epub 2014 Oct 2.

Structure of the large ribosomal subunit from human mitochondria.

Author information

1
MRC Laboratory of Molecular Biology, Francis Crick Avenue, Cambridge CB2 0QH, United Kingdom.
#
Contributed equally

Abstract

Human mitochondrial ribosomes are highly divergent from all other known ribosomes and are specialized to exclusively translate membrane proteins. They are linked with hereditary mitochondrial diseases and are often the unintended targets of various clinically useful antibiotics. Using single-particle cryogenic electron microscopy, we have determined the structure of its large subunit to 3.4 angstrom resolution, revealing 48 proteins, 21 of which are specific to mitochondria. The structure unveils an adaptation of the exit tunnel for hydrophobic nascent peptides, extensive remodeling of the central protuberance, including recruitment of mitochondrial valine transfer RNA (tRNA(Val)) to play an integral structural role, and changes in the tRNA binding sites related to the unusual characteristics of mitochondrial tRNAs.

PMID:
25278503
PMCID:
PMC4246062
DOI:
10.1126/science.1258026
[Indexed for MEDLINE]
Free PMC Article

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