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C7orf30 specifically associates with the large subunit of the mitochondrial ribosome and is involved in translation

Lookup NU author(s): Alexksandra Pajak, Professor Robert Lightowlers



In a comparative genomics study for mitochondrial ribosome-associated proteins, we identified C7orf30, the human homolog of the plant protein iojap. Gene order conservation among bacteria and the observation that iojap orthologs cannot be transferred between bacterial species predict this protein to be associated with the mitochondrial ribosome. Here, we show colocalization of C7orf30 with the large subunit of the mitochondrial ribosome using isokinetic sucrose gradient and 2D Blue Native polyacrylamide gel electrophoresis (BN-PAGE) analysis. We co-purified C7orf30 with proteins of the large subunit, and not with proteins of the small subunit, supporting interaction that is specific to the large mitoribosomal complex. Consistent with this physical association, a mitochondrial translation assay reveals negative effects of C7orf30 siRNA knock-down on mitochondrial gene expression. Based on our data we propose that C7orf30 is involved in ribosomal large subunit function. Sequencing the gene in 35 patients with impaired mitochondrial translation did not reveal disease-causing mutations in C7orf30.

Publication metadata

Author(s): Wanschers BFJ, Szklarczyk R, Pajak A, van den Brand MAM, Gloerich J, Rodenburg RJT, Lightowlers RN, Nijtmans LG, Huynen MA

Publication type: Article

Publication status: Published

Journal: Nucleic Acids Research

Year: 2012

Volume: 40

Issue: 9

Pages: 4040-4051

Print publication date: 11/01/2012

Date deposited: 12/10/2012

ISSN (print): 0305-1048

ISSN (electronic): 1362-4962

Publisher: Oxford University Press


DOI: 10.1093/nar/gkr1271


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Funder referenceFunder name
Radboud University Nijmegen Medical Centre
050-71-053Netherlands Organization for Scientific Research (NWO)
BB/F011520/1Biotechnology and Biological Sciences Research Council
CSBR09/013VCSBR (Centres for Systems Biology Research) initiative from NWO