The conserved interaction of C7orf30 with MRPL14 promotes biogenesis of the mitochondrial large ribosomal subunit and mitochondrial translation

Molecular Biology of the Cell

Volumn 24, Issue 3, 2013, Pages 184-193

  Fung, Stephen ^a   Nishimura, Tamiko ^a   Sasarman, Florin ^a   Shoubridge, Eric A ^a  

^a MCGILL UNIVERSITY   (Canada)

Author keywords

[No Author keywords available]

Indexed keywords

CELL PROTEIN; PROTEIN C7ORF30; PROTEIN MRPL14; UNCLASSIFIED DRUG;

ARTICLE; BIOGENESIS; CONTROLLED STUDY; HUMAN; HUMAN CELL; IMMUNOPRECIPITATION; MASS SPECTROMETRY; MITOCHONDRION; PRIORITY JOURNAL; PROTEIN ASSEMBLY; PROTEIN FUNCTION; PROTEIN PROTEIN INTERACTION; RIBOSOME;

GENE KNOCKDOWN TECHNIQUES; HEK293 CELLS; HUMANS; MITOCHONDRIA; MITOCHONDRIAL PROTEINS; PROTEIN BINDING; PROTEIN BIOSYNTHESIS; RIBOSOMAL PROTEINS; RIBOSOME SUBUNITS, LARGE; RNA, SMALL INTERFERING;

EID: 84873332767     PISSN: 10591524     EISSN: 19394586     Source Type: Journal    
DOI: 10.1091/mbc.E12-09-0651     Document Type: Article

References (29)

1. Antonicka H, Sasarman F, Kennaway NG, Shoubridge EA (2006). The molecular basis for tissue specificity of the oxidative phosphorylation deficiencies in patients with mutations in the mitochondrial translation factor EFG1. Hum Mol Genet 15, 1835-1846.
2. Baer RJ, Dubin DT (1981). Methylated regions of hamster mitochondrial ribosomal RNA: structural and functional correlates. Nucleic Acids Res 9, 323-337.
3. Barrientos A, Korr D, Barwell KJ, Sjulsen C, Gajewski CD, Manfredi G, Ackerman S, Tzagoloff A (2003). MTG1 codes for a conserved protein required for mitochondrial translation. Mol Biol Cell 14, 2292-2302.
4. Battersby BJ, Loredo-Osti JC, Shoubridge EA (2003). Nuclear genetic control of mitochondrial DNA segregation. Nat Genet 33, 183-186.
5. Camara Y et al. (2011). MTERF4 regulates translation by targeting the methyltransferase NSUN4 to the mammalian mitochondrial ribosome. Cell Metab 13, 527-539.
6. Christian BE, Spremulli LL (2011). Mechanism of protein biosynthesis in mammalian mitochondria. Biochim Biophys Acta 1819, 1035-1054.
7. Davies C, White SW, Ramakrishnan V (1996). The crystal structure of ribosomal protein L14 reveals an important organizational component of the translational apparatus. Structure 4, 55-66.
8. Galperin MY, Koonin EV (2004). "Conserved hypothetical" proteins: prioritization of targets for experimental study. Nucleic Acids Res 32, 5452-5462.
9. Gao H et al. (2003). Study of the structural dynamics of the E. coli 70S ribosome using real-space refinement. Cell 113, 789-801.
10. Gruschke S, Kehrein K, Rompler K, Grone K, Israel L, Imhof A, Herrmann JM, Ott M (2011). Cbp3-Cbp6 interacts with the yeast mitochondrial ribosomal tunnel exit and promotes cytochrome b synthesis and assembly. J Cell Biol 193, 1101-1114.
11. Hage AE, Tollervey D (2004). A surfeit of factors: why is ribosome assembly so much more complicated in eukaryotes than bacteria. RNA Biol 1, 10-15.
12. Han CD, Coe EH Jr., Martienssen RA (1992). Molecular cloning and characterization of iojap (ij), a pattern striping gene of maize. EMBO J 11, 4037-4046.
13. Hauser R et al. (2012). RsfA (YbeB) proteins are conserved ribosomal silencing factors. PLoS Genet 8, e1002815.
14. Jenkins MT (1924). Heritable characters of maize. XX iojap-striping, a chlorophyl defect. J. Heredity 15, 467-472.
15. Jiang M, Sullivan SM, Walker AK, Strahler JR, Andrews PC, Maddock JR (2007). Identification of novel Escherichia coli ribosome-associated proteins using isobaric tags and multidimensional protein identification techniques. J Bacteriol 189, 3434-3444.
16. Klement P, Nijtmans LG, Van den Bogert C, Houstek J (1995). Analysis of oxidative phosphorylation complexes in cultured human fibroblasts and amniocytes by blue-native-electrophoresis using mitoplasts isolated with the help of digitonin. Anal Biochem 231, 218-224.
17. Leary SC, Sasarman F (2009). Oxidative phosphorylation: synthesis of mitochondrially encoded proteins and assembly of individual structural subunits into functional holoenzyme complexes. Methods Mol Biol 554, 143-162.
18. Nolden M, Ehses S, Koppen M, Bernacchia A, Rugarli EI, Langer T (2005). The m-AAA protease defective in hereditary spastic paraplegia controls ribosome assembly in mitochondria. Cell 123, 277-289.
19. Rorbach J, Gammage PA, Minczuk M (2012). C7orf30 is necessary for biogenesis of the large subunit of the mitochondrial ribosome. Nucleic Acids Res 40, 4097-4109.
20. Sasarman F, Shoubridge EA (2012). Radioactive labeling of mitochondrial translation products in cultured cells. Methods Mol Biol 837, 207-217.
21. Shajani Z, Sykes MT, Williamson JR (2011). Assembly of bacterial ribosomes. Annu Rev Biochem 80, 501-526.
22. Sharma MR, Koc EC, Datta PP, Booth TM, Spremulli LL, Agrawal RK (2003). Structure of the mammalian mitochondrial ribosome reveals an expanded functional role for its component proteins. Cell 115, 97-108.
23. Sirum-Connolly K, Mason TL (1993). Functional requirement of a site-specific ribose methylation in ribosomal RNA. Science 262, 1886-1889.
24. Smeitink JA et al. (2006). Distinct clinical phenotypes associated with a mutation in the mitochondrial translation elongation factor EFTs. Am J Hum Genet 79, 869-877.
25. Walbot V, Coe EH (1979). Nuclear gene iojap conditions a programmed change to ribosome-less plastids in Zea mays. Proc Natl Acad Sci USA 76, 2760-2764.
26. Wanschers BF, Szklarczyk R, Pajak A, van den Brand MA, Gloerich J, Rodenburg RJ, Lightowlers RN, Nijtmans LG, Huynen MA (2012). C7orf30 specifically associates with the large subunit of the mitochondrial ribosome and is involved in translation. Nucleic Acids Res 40, 4040-4051.
27. Weraarpachai W et al. (2009). Mutation in TACO1, encoding a translational activator of COX I, results in cytochrome c oxidase deficiency and late-onset Leigh syndrome. Nat Genet 41, 833-837.
28. Weraarpachai W, Sasarman F, Nishimura T, Antonicka H, Aure K, Rotig A, Lombes A, Shoubridge EA (2012). Mutations in C12orf62, a factor that couples COX I synthesis with cytochrome c oxidase assembly, cause fatal neonatal lactic acidosis. Am J Hum Genet 90, 142-151.
29. Yusupov MM, Yusupova GZ, Baucom A, Lieberman K, Earnest TN, Cate JH, Noller HF (2001). Crystal structure of the ribosome at 5.5 A resolution. Science 292, 883-896.