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Hoffmann, R. A wiki for the life sciences where authorship matters. Nature Genetics (2008)
 

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Terms

 

Gene Review: 

MRRF  -  mitochondrial ribosome recycling factor

Homo sapiens

Synonyms: MRFF, MTRRF, RRF, Ribosome-recycling factor, mitochondrial, Ribosome-releasing factor, mitochondrial
 
 
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Disease relevance of MRRF

  • We found this genetic alteration in four atypical and sporadic cases of mitochondrial encephalomyopathy, characterized by RRF and partial COX deficiency [1].
  • We related these results to exercise lactate production as well as to the pathologic features of the underlying myopathy. h-mtTFA levels were significantly inversely related to blood lactate and the percent of RRF, borderline to cox negative fibers [2].
 

High impact information on MRRF

  • We show that the elongation factor EF-G and the ribosomal recycling factor RRF split the ribosome into subunits in the absence of initiation factor 3 (IF3) by a mechanism that requires both GTP and GTP hydrolysis [3].
  • All muscle biopsies performed showed lipid storage apart from RRF and respiratory chain complexes deficiency [4].
  • RRF did not influence the large error (10-fold increase) induced by streptomycin [5].
  • In polyphenylalanine synthesis programmed by polyuridylic acid, misincorporation of isoleucine, leucine or a mixture of amino acids was stimulated upto 17-fold when RRF was omitted from the in vitro system [5].
  • Within RRF, apoptosis was associated with higher mutation proportion, suggesting that it was provoked by severe respiratory defect in the same time as increased mitochondrial mass [6].
 

Biological context of MRRF

  • Recently, molecular mimicry among tRNA, elongation factors, releasing factor (RF), and ribosome recycling factor (RRF) has been demonstrated and greatly improved our understanding of the mechanism of translation [7].
 

Anatomical context of MRRF

 

Associations of MRRF with chemical compounds

  • RESULTS: The KP index < 2.0 group showed significantly lower indices for weekly Kt/Vurea, nPNA and %LBM (%) than those of the KP index > 2.0 group, while there were no significant differences between the groups in the serum albumin concentration, serum creatinine concentration, FFEFBM and RRF [9].
 

Analytical, diagnostic and therapeutic context of MRRF

  • By using cryo-electron microscopy, we have obtained two density maps, one of the RRF bound posttermination complex and one of the 50S subunit bound with both EF-G and RRF [10].
  • In situ hybridization demonstrated an overall signal weaker than controls, with a relatively higher signal in RRF [11].

References

  1. Multiple deletions of mitochondrial DNA in sporadic and atypical cases of encephalomyopathy. Checcarelli, N., Prelle, A., Moggio, M., Comi, G., Bresolin, N., Papadimitriou, A., Fagiolari, G., Bordoni, A., Scarlato, G. J. Neurol. Sci. (1994) [Pubmed]
  2. Abnormal levels of human mitochondrial transcription factor A in skeletal muscle in mitochondrial encephalomyopathies. Siciliano, G., Mancuso, M., Pasquali, L., Manca, M.L., Tessa, A., Iudice, A. Neurol. Sci. (2000) [Pubmed]
  3. Splitting of the posttermination ribosome into subunits by the concerted action of RRF and EF-G. Zavialov, A.V., Hauryliuk, V.V., Ehrenberg, M. Mol. Cell (2005) [Pubmed]
  4. Lipomatosis, proximal myopathy, and the mitochondrial 8344 mutation. A lipid storage myopathy? Muñoz-Málaga, A., Bautista, J., Salazar, J.A., Aguilera, I., Garcia, R., Chinchon, I., Segura, M.D., Campos, Y., Arenas, J. Muscle Nerve (2000) [Pubmed]
  5. Dual functions of ribosome recycling factor in protein biosynthesis: disassembling the termination complex and preventing translational errors. Janosi, L., Ricker, R., Kaji, A. Biochimie (1996) [Pubmed]
  6. Apoptosis in mitochondrial myopathies is linked to mitochondrial proliferation. Auré, K., Fayet, G., Leroy, J.P., Lacène, E., Romero, N.B., Lombès, A. Brain (2006) [Pubmed]
  7. Moonlighting functions of polypeptide elongation factor 1: from actin bundling to zinc finger protein R1-associated nuclear localization. Ejiri, S. Biosci. Biotechnol. Biochem. (2002) [Pubmed]
  8. Making sense of mimic in translation termination. Nakamura, Y., Ito, K. Trends Biochem. Sci. (2003) [Pubmed]
  9. KP index at the initiation of dialysis for patients with end-stage renal disease. Hwang, E.W., Ji, S.B., Kim, J.K., Hwang, S.D. Korean J. Intern. Med. (2004) [Pubmed]
  10. Mechanism for the disassembly of the posttermination complex inferred from cryo-EM studies. Gao, N., Zavialov, A.V., Li, W., Sengupta, J., Valle, M., Gursky, R.P., Ehrenberg, M., Frank, J. Mol. Cell (2005) [Pubmed]
  11. Partial depletion and multiple deletions of muscle mtDNA in familial MNGIE syndrome. Papadimitriou, A., Comi, G.P., Hadjigeorgiou, G.M., Bordoni, A., Sciacco, M., Napoli, L., Prelle, A., Moggio, M., Fagiolari, G., Bresolin, N., Salani, S., Anastasopoulos, I., Giassakis, G., Divari, R., Scarlato, G. Neurology (1998) [Pubmed]
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