Structure of the large ribosomal subunit from human mitochondria

Alan Brown, Alexey Amunts, Xiao Chen Bai, Yoichiro Sugimoto, Patricia C. Edwards, Garib Murshudov, Sjors H.W. Scheres, V. Ramakrishnan

Research output: Contribution to journalArticle

127 Citations (Scopus)

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 (tRNAVal) to play an integral structural role, and changes in the tRNA binding sites related to the unusual characteristics of mitochondrial tRNAs.

Original languageEnglish (US)
Pages (from-to)718-722
Number of pages5
JournalScience
Volume346
Issue number6210
DOIs
StatePublished - Nov 7 2014

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Large Ribosome Subunits
Transfer RNA
Mitochondria
RNA, Transfer, Val
Mitochondrial Diseases
Inborn Genetic Diseases
Valine
Ribosomes
Electron Microscopy
Membrane Proteins
Binding Sites
Anti-Bacterial Agents
Peptides
Proteins

ASJC Scopus subject areas

  • General

Cite this

Brown, A., Amunts, A., Bai, X. C., Sugimoto, Y., Edwards, P. C., Murshudov, G., ... Ramakrishnan, V. (2014). Structure of the large ribosomal subunit from human mitochondria. Science, 346(6210), 718-722. https://doi.org/10.1126/science.1258026

Structure of the large ribosomal subunit from human mitochondria. / Brown, Alan; Amunts, Alexey; Bai, Xiao Chen; Sugimoto, Yoichiro; Edwards, Patricia C.; Murshudov, Garib; Scheres, Sjors H.W.; Ramakrishnan, V.

In: Science, Vol. 346, No. 6210, 07.11.2014, p. 718-722.

Research output: Contribution to journalArticle

Brown, A, Amunts, A, Bai, XC, Sugimoto, Y, Edwards, PC, Murshudov, G, Scheres, SHW & Ramakrishnan, V 2014, 'Structure of the large ribosomal subunit from human mitochondria', Science, vol. 346, no. 6210, pp. 718-722. https://doi.org/10.1126/science.1258026
Brown A, Amunts A, Bai XC, Sugimoto Y, Edwards PC, Murshudov G et al. Structure of the large ribosomal subunit from human mitochondria. Science. 2014 Nov 7;346(6210):718-722. https://doi.org/10.1126/science.1258026
Brown, Alan ; Amunts, Alexey ; Bai, Xiao Chen ; Sugimoto, Yoichiro ; Edwards, Patricia C. ; Murshudov, Garib ; Scheres, Sjors H.W. ; Ramakrishnan, V. / Structure of the large ribosomal subunit from human mitochondria. In: Science. 2014 ; Vol. 346, No. 6210. pp. 718-722.
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