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Volumn 416, Issue 1, 2008, Pages 15-26

Disruption of a mitochondrial RNA-binding protein gene results in decreased cytochrome b expression and a marked reduction in ubiquinol-cytochrome c reductase activity in mouse heart mitochondria

Author keywords

Cytochrome b; Mitochondrion; Pentatricopeptide repeat (PPR) protein; Pentatricopeptide repeat domain protein 2 (PTCD2); RNA processing

Indexed keywords

CARDIOMYOCYTES; CATALYTIC CORE; COMPLEX ACTIVITY; CYTOCHROME B; CYTOCHROME C; ENCODED PROTEINS; HEART FAILURE; MITOCHONDRIAL DNA; MITOCHONDRIAL FUNCTION; MOUSE HEART; NADH DEHYDROGENASE; NORTHERN BLOTTING; PENTATRICOPEPTIDE REPEAT (PPR) PROTEIN; PENTATRICOPEPTIDE REPEAT DOMAIN PROTEIN 2 (PTCD2); PENTATRICOPEPTIDE REPEATS; REDUCTASE ACTIVITY; RNA METABOLISMS; RNA PROCESSING; RNA-BINDING PROTEIN; SKELETAL MUSCLE; UBIQUINOL;

EID: 56049093869     PISSN: 02646021     EISSN: 14708728     Source Type: Journal    
DOI: 10.1042/BJ20080847     Document Type: Article
Times cited : (67)

References (31)
  • 1
    • 0033965922 scopus 로고    scopus 로고
    • The PPR motif - a TPR-related motif prevalent in plant organellar proteins
    • Small, I. D. and Peeters, N. (2000) The PPR motif - a TPR-related motif prevalent in plant organellar proteins. Trends Biochem. Sci. 25, 46-47
    • (2000) Trends Biochem. Sci , vol.25 , pp. 46-47
    • Small, I.D.1    Peeters, N.2
  • 2
    • 0030671215 scopus 로고    scopus 로고
    • The Neurospora crassa cya-5 nuclear gene encodes a protein with a region of homology to the Saccharomyces cerevisiae PET309 protein and is required in a post-transcriptional step for the expression of the mitochondrially encoded COXI protein
    • Coffin, J. W., Dhillon, R., Ritzel, R. G. and Nargang, F. E. (1997) The Neurospora crassa cya-5 nuclear gene encodes a protein with a region of homology to the Saccharomyces cerevisiae PET309 protein and is required in a post-transcriptional step for the expression of the mitochondrially encoded COXI protein. Curr. Genet. 32, 273-280
    • (1997) Curr. Genet , vol.32 , pp. 273-280
    • Coffin, J.W.1    Dhillon, R.2    Ritzel, R.G.3    Nargang, F.E.4
  • 3
    • 0028984035 scopus 로고
    • The product of the nuclear gene PET309 is required for translation of mature mRNA and stability or production of intron-containing RNAs derived from the mitochondrial COX1 locus of Saccharomyces cerevisiae
    • Manthey, G. M. and McEwen, J. E. (1995) The product of the nuclear gene PET309 is required for translation of mature mRNA and stability or production of intron-containing RNAs derived from the mitochondrial COX1 locus of Saccharomyces cerevisiae. EMBO J. 14, 4031-4043
    • (1995) EMBO J , vol.14 , pp. 4031-4043
    • Manthey, G.M.1    McEwen, J.E.2
  • 5
    • 0033522445 scopus 로고    scopus 로고
    • Molecular cloning of the maize gene crp1 reveals similarity between regulators of mitochondrial and chloroplast gene expression
    • Fisk, D. G., Walker, M. B. and Barkan, A. (1999) Molecular cloning of the maize gene crp1 reveals similarity between regulators of mitochondrial and chloroplast gene expression. EMBO J. 18, 2621-2630
    • (1999) EMBO J , vol.18 , pp. 2621-2630
    • Fisk, D.G.1    Walker, M.B.2    Barkan, A.3
  • 8
    • 4344595430 scopus 로고    scopus 로고
    • The role of the LRPPRC (leucine-rich pentatricopeptide repeat cassette) gene in cytochrome oxidase assembly: Mutation causes lowered levels of COX (cytochrome c oxidase) I and COX III mRNA
    • Xu, F., Morin, C., Mitchell, G., Ackerley, C. and Robinson, B. H. (2004) The role of the LRPPRC (leucine-rich pentatricopeptide repeat cassette) gene in cytochrome oxidase assembly: mutation causes lowered levels of COX (cytochrome c oxidase) I and COX III mRNA. Biochem. J. 382, 331-336
    • (2004) Biochem. J , vol.382 , pp. 331-336
    • Xu, F.1    Morin, C.2    Mitchell, G.3    Ackerley, C.4    Robinson, B.H.5
  • 9
    • 0036310995 scopus 로고    scopus 로고
    • LRP130, a protein containing nine pentatricopeptide repeat motifs, interacts with a single-stranded cytosine-rich sequence of mouse hypervariable minisatellite Pc-1
    • Tsuchiya, N., Fukuda, H., Sugimura, T., Nagao, M. and Nakagama, H. (2002) LRP130, a protein containing nine pentatricopeptide repeat motifs, interacts with a single-stranded cytosine-rich sequence of mouse hypervariable minisatellite Pc-1. Eur. J. Biochem. 269, 2927-2933
    • (2002) Eur. J. Biochem , vol.269 , pp. 2927-2933
    • Tsuchiya, N.1    Fukuda, H.2    Sugimura, T.3    Nagao, M.4    Nakagama, H.5
  • 10
    • 0035699578 scopus 로고    scopus 로고
    • Sequence analysis of LRPPRC and its SEC1 domain interaction partners suggests roles in cytoskeletal organization, vesicular trafficking, nucleocytosolic shuttling, and chromosome activity
    • Liu, L. and McKeehan, W. L. (2002) Sequence analysis of LRPPRC and its SEC1 domain interaction partners suggests roles in cytoskeletal organization, vesicular trafficking, nucleocytosolic shuttling, and chromosome activity. Genomics 79, 124-136
    • (2002) Genomics , vol.79 , pp. 124-136
    • Liu, L.1    McKeehan, W.L.2
  • 11
    • 0037774459 scopus 로고    scopus 로고
    • LRP130, a pentatricopeptide motif protein with a noncanonical RNA-binding domain, is bound in vivo to mitochondrial and nuclear RNAs
    • Mili, S. and Pinol-Roma, S. (2003) LRP130, a pentatricopeptide motif protein with a noncanonical RNA-binding domain, is bound in vivo to mitochondrial and nuclear RNAs. Mol. Cell. Biol. 23, 4972-4982
    • (2003) Mol. Cell. Biol , vol.23 , pp. 4972-4982
    • Mili, S.1    Pinol-Roma, S.2
  • 12
    • 4444268726 scopus 로고    scopus 로고
    • Coupling the mitochondrial transcription machinery to human disease
    • Shadel, G. S. (2004) Coupling the mitochondrial transcription machinery to human disease. Trends Genet. 20, 513-519
    • (2004) Trends Genet , vol.20 , pp. 513-519
    • Shadel, G.S.1
  • 13
    • 34748924950 scopus 로고    scopus 로고
    • Pentatricopeptide repeat proteins in Trypanosoma brucei function in mitochondrial ribosomes
    • Pusnik, M., Small, I., Read, L. K., Fabbro, T. and Schneider, A. (2007) Pentatricopeptide repeat proteins in Trypanosoma brucei function in mitochondrial ribosomes. Mol. Cell. Biol. 27, 6876-6888
    • (2007) Mol. Cell. Biol , vol.27 , pp. 6876-6888
    • Pusnik, M.1    Small, I.2    Read, L.K.3    Fabbro, T.4    Schneider, A.5
  • 14
    • 34249938871 scopus 로고    scopus 로고
    • Conserved domain structure of pentatricopeptide repeat proteins involved in chloroplast RNA editing
    • Okuda, K., Myouga, F., Motohashi, R., Shinozaki, K. and Shikanai, T. (2007) Conserved domain structure of pentatricopeptide repeat proteins involved in chloroplast RNA editing. Proc. Natl. Acad. Sci. U.S.A. 104, 8178-8183
    • (2007) Proc. Natl. Acad. Sci. U.S.A , vol.104 , pp. 8178-8183
    • Okuda, K.1    Myouga, F.2    Motohashi, R.3    Shinozaki, K.4    Shikanai, T.5
  • 15
    • 33745473351 scopus 로고    scopus 로고
    • Defective mitochondrial gene expression results in reactive oxygen species-mediated inhibition of respiration and reduction of yeast life span
    • Bonawitz, N. D., Rodeheffer, M. S. and Shadel, G. S. (2006) Defective mitochondrial gene expression results in reactive oxygen species-mediated inhibition of respiration and reduction of yeast life span. Mol. Cell. Biol. 26, 4818-4829
    • (2006) Mol. Cell. Biol , vol.26 , pp. 4818-4829
    • Bonawitz, N.D.1    Rodeheffer, M.S.2    Shadel, G.S.3
  • 17
    • 0025123850 scopus 로고
    • The use of skin fibroblast cultures in the detection of respiratory chain defects in patients with lacticacidemia
    • Robinson, B. H., Glerum, D. M., Chow, W., Petrova-Benedict, R., Lightowlers, R. and Capaldi, R. (1990) The use of skin fibroblast cultures in the detection of respiratory chain defects in patients with lacticacidemia. Pediatr. Res. 28, 549-555
    • (1990) Pediatr. Res , vol.28 , pp. 549-555
    • Robinson, B.H.1    Glerum, D.M.2    Chow, W.3    Petrova-Benedict, R.4    Lightowlers, R.5    Capaldi, R.6
  • 20
    • 0026409298 scopus 로고
    • Blue native electrophoresis for isolation of membrane protein complexes in enzymatically active form
    • Schagger, H. and von Jagow, G. (1991) Blue native electrophoresis for isolation of membrane protein complexes in enzymatically active form. Anal. Biochem. 199, 223-231
    • (1991) Anal. Biochem , vol.199 , pp. 223-231
    • Schagger, H.1    von Jagow, G.2
  • 21
    • 0029013434 scopus 로고
    • Quantification of oxidative phosphorylation enzymes after blue native electrophoresis and two-dimensional resolution: Normal complex I protein amounts in Parkinson's disease conflict with reduced catalytic activities
    • Schagger, H. (1995) Quantification of oxidative phosphorylation enzymes after blue native electrophoresis and two-dimensional resolution: normal complex I protein amounts in Parkinson's disease conflict with reduced catalytic activities. Electrophoresis 16, 763-770
    • (1995) Electrophoresis , vol.16 , pp. 763-770
    • Schagger, H.1
  • 22
    • 0029915525 scopus 로고    scopus 로고
    • Computational method to predict mitochondrially imported proteins and their targeting sequences
    • Claros, M. G. and Vincens, P. (1996) Computational method to predict mitochondrially imported proteins and their targeting sequences. Eur. J. Biochem. 241, 779-786
    • (1996) Eur. J. Biochem , vol.241 , pp. 779-786
    • Claros, M.G.1    Vincens, P.2
  • 25
    • 30044447187 scopus 로고    scopus 로고
    • Oxygen tension regulates mitochondrial DNA-encoded complex I gene expression
    • Piruat, J. I. and Lopez-Barneo, J. (2005) Oxygen tension regulates mitochondrial DNA-encoded complex I gene expression. J. Biol. Chem. 280, 42676-42684
    • (2005) J. Biol. Chem , vol.280 , pp. 42676-42684
    • Piruat, J.I.1    Lopez-Barneo, J.2
  • 28
    • 0017226321 scopus 로고
    • Infantile cardiomyopathy with histiocytoid change in cardiac muscle cells. Report of six patients
    • Ferrans, V. J., McAllister, Jr, H. A. and Haese, W. H. (1976) Infantile cardiomyopathy with histiocytoid change in cardiac muscle cells. Report of six patients. Circulation 53, 708-719
    • (1976) Circulation , vol.53 , pp. 708-719
    • Ferrans, V.J.1    McAllister Jr, H.A.2    Haese, W.H.3
  • 29
    • 0021186586 scopus 로고
    • Histiocytoid cardiomyopathy of infancy: Deficiency of reducible cytochrome b in heart mitochondria
    • Papadimitriou, A., Neustein, H. B., Dimauro, S., Stanton, R. and Bresolin, N. (1984) Histiocytoid cardiomyopathy of infancy: deficiency of reducible cytochrome b in heart mitochondria. Pediatr. Res. 18, 1023-1028
    • (1984) Pediatr. Res , vol.18 , pp. 1023-1028
    • Papadimitriou, A.1    Neustein, H.B.2    Dimauro, S.3    Stanton, R.4    Bresolin, N.5
  • 30
    • 0026249415 scopus 로고
    • Right ventricular dysplasia associated with sudden death in young adults
    • Goodin, J. C., Farb, A., Smialek, J. E., Field, F. and Virmani, R. (1991) Right ventricular dysplasia associated with sudden death in young adults. Mod. Pathol. 4, 702-706
    • (1991) Mod. Pathol , vol.4 , pp. 702-706
    • Goodin, J.C.1    Farb, A.2    Smialek, J.E.3    Field, F.4    Virmani, R.5
  • 31
    • 0033888963 scopus 로고    scopus 로고
    • A missense mutation in the mitochondrial cytochrome b gene in a revisited case with histiocytoid cardiomyopathy
    • Andreu, A. L., Checcarelli, N., Iwata, S., Shanske, S. and DiMauro, S. (2000) A missense mutation in the mitochondrial cytochrome b gene in a revisited case with histiocytoid cardiomyopathy. Pediatr. Res. 48, 311-314
    • (2000) Pediatr. Res , vol.48 , pp. 311-314
    • Andreu, A.L.1    Checcarelli, N.2    Iwata, S.3    Shanske, S.4    DiMauro, S.5


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