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Volumn 1797, Issue 6-7, 2010, Pages 1105-1112

Consequences of the pathogenic T9176C mutation of human mitochondrial DNA on yeast mitochondrial ATP synthase

Author keywords

ATP synthase; Leigh syndrome; Mitochondrial disease; Mitochondrial DNA; NARP syndrome; Yeast

Indexed keywords

ADENOSINE TRIPHOSPHATASE; ADENOSINE TRIPHOSPHATE; CYSTEINE; LEUCINE; MITOCHONDRIAL DNA; OLIGOMYCIN; PROLINE; PROTON TRANSPORTING ADENOSINE TRIPHOSPHATE SYNTHASE; THREONINE;

EID: 77953807216     PISSN: 00052728     EISSN: None     Source Type: Journal    
DOI: 10.1016/j.bbabio.2009.12.022     Document Type: Article
Times cited : (53)

References (40)
  • 1
    • 0033525924 scopus 로고    scopus 로고
    • Oxidative phosphorylation at the fin de siecle
    • Saraste M. Oxidative phosphorylation at the fin de siecle. Science 1999, 283:1488-1493.
    • (1999) Science , vol.283 , pp. 1488-1493
    • Saraste, M.1
  • 5
    • 0242657369 scopus 로고    scopus 로고
    • Mechanics of coupling proton movements to c-ring rotation in ATP synthase
    • Fillingame R.H., Angevine C.M., Dmitriev O.Y. Mechanics of coupling proton movements to c-ring rotation in ATP synthase. FEBS Lett. 2003, 555:29-34.
    • (2003) FEBS Lett. , vol.555 , pp. 29-34
    • Fillingame, R.H.1    Angevine, C.M.2    Dmitriev, O.Y.3
  • 7
    • 0029122341 scopus 로고
    • A novel mitochondrial ATPase 6 point mutation in familial bilateral striatal necrosis
    • Thyagarajan D., Shanske S., Vazquez-Memije M., De Vivo D., DiMauro S. A novel mitochondrial ATPase 6 point mutation in familial bilateral striatal necrosis. Ann. Neurol. 1995, 38:468-472.
    • (1995) Ann. Neurol. , vol.38 , pp. 468-472
    • Thyagarajan, D.1    Shanske, S.2    Vazquez-Memije, M.3    De Vivo, D.4    DiMauro, S.5
  • 8
    • 67650964198 scopus 로고    scopus 로고
    • Introducing the human Leigh syndrome mutation T9176G into Saccharomyces cerevisiae mitochondrial DNA leads to severe defects in the incorporation of Atp6p into the ATP synthase and in the mitochondrial morphology
    • Kucharczyk R., Salin B., di Rago J.P. Introducing the human Leigh syndrome mutation T9176G into Saccharomyces cerevisiae mitochondrial DNA leads to severe defects in the incorporation of Atp6p into the ATP synthase and in the mitochondrial morphology. Hum. Mol. Genet. 2009, 18:2889-2898.
    • (2009) Hum. Mol. Genet. , vol.18 , pp. 2889-2898
    • Kucharczyk, R.1    Salin, B.2    di Rago, J.P.3
  • 9
    • 36349036738 scopus 로고    scopus 로고
    • A yeast model of the neurogenic ataxia retinitis pigmentosa (NARP) T8993G mutation in the mitochondrial ATP synthase-6 gene
    • Rak M., Tetaud E., Duvezin-Caubet S., Ezkurdia N., Bietenhader M., Rytka J., di Rago J.P. A yeast model of the neurogenic ataxia retinitis pigmentosa (NARP) T8993G mutation in the mitochondrial ATP synthase-6 gene. J. Biol. Chem. 2007, 282:34039-34047.
    • (2007) J. Biol. Chem. , vol.282 , pp. 34039-34047
    • Rak, M.1    Tetaud, E.2    Duvezin-Caubet, S.3    Ezkurdia, N.4    Bietenhader, M.5    Rytka, J.6    di Rago, J.P.7
  • 10
    • 67349139921 scopus 로고    scopus 로고
    • Biochemical consequences in yeast of the human mitochondrial DNA 8993T>C mutation in the ATPase6 gene found in NARP/MILS patients
    • Kucharczyk R., Rak M., di Rago J.P. Biochemical consequences in yeast of the human mitochondrial DNA 8993T>C mutation in the ATPase6 gene found in NARP/MILS patients. Biochim. Biophys. Acta 2009, 1793:817-824.
    • (2009) Biochim. Biophys. Acta , vol.1793 , pp. 817-824
    • Kucharczyk, R.1    Rak, M.2    di Rago, J.P.3
  • 11
    • 34249722612 scopus 로고    scopus 로고
    • Yeast cells lacking the mitochondrial gene encoding the ATP synthase subunit 6 exhibit a selective loss of complex IV and unusual mitochondrial morphology
    • Rak M., Tetaud E., Godard F., Sagot I., Salin B., Duvezin-Caubet S., Slonimski P.P., Rytka J., di Rago J.P. Yeast cells lacking the mitochondrial gene encoding the ATP synthase subunit 6 exhibit a selective loss of complex IV and unusual mitochondrial morphology. J. Biol. Chem. 2007, 282:10853-10864.
    • (2007) J. Biol. Chem. , vol.282 , pp. 10853-10864
    • Rak, M.1    Tetaud, E.2    Godard, F.3    Sagot, I.4    Salin, B.5    Duvezin-Caubet, S.6    Slonimski, P.P.7    Rytka, J.8    di Rago, J.P.9
  • 12
    • 0035223057 scopus 로고    scopus 로고
    • Genetic transformation of Saccharomyces cerevisiae mitochondria
    • Bonnefoy N., Fox T.D. Genetic transformation of Saccharomyces cerevisiae mitochondria. Methods Cell Biol. 2001, 65:381-396.
    • (2001) Methods Cell Biol. , vol.65 , pp. 381-396
    • Bonnefoy, N.1    Fox, T.D.2
  • 13
    • 0034122608 scopus 로고    scopus 로고
    • Maintenance and integrity of the mitochondrial genome: a plethora of nuclear genes in the budding yeast
    • Contamine V., Picard M. Maintenance and integrity of the mitochondrial genome: a plethora of nuclear genes in the budding yeast. Microbiol. Mol. Biol. Rev. 2000, 64:281-315.
    • (2000) Microbiol. Mol. Biol. Rev. , vol.64 , pp. 281-315
    • Contamine, V.1    Picard, M.2
  • 14
    • 0028331160 scopus 로고
    • Level of ATP synthase activity required for yeast Saccharomyces cerevisiae to grow on glycerol media
    • Mukhopadhyay A., Uh M., Mueller D.M. Level of ATP synthase activity required for yeast Saccharomyces cerevisiae to grow on glycerol media. FEBS Lett. 1994, 343:160-164.
    • (1994) FEBS Lett. , vol.343 , pp. 160-164
    • Mukhopadhyay, A.1    Uh, M.2    Mueller, D.M.3
  • 15
    • 0022492812 scopus 로고
    • Rhodamine 123 as a probe of transmembrane potential in isolated rat-liver mitochondria: spectral and metabolic properties
    • Emaus R.K., Grunwald R., Lemasters J.J. Rhodamine 123 as a probe of transmembrane potential in isolated rat-liver mitochondria: spectral and metabolic properties. Biochim. Biophys. Acta 1986, 850:436-448.
    • (1986) Biochim. Biophys. Acta , vol.850 , pp. 436-448
    • Emaus, R.K.1    Grunwald, R.2    Lemasters, J.J.3
  • 16
    • 0038070088 scopus 로고    scopus 로고
    • Investigation of the role and mechanism of IF1 and STF1 proteins, twin inhibitory peptides which interact with the yeast mitochondrial ATP synthase
    • Venard R., Brethes D., Giraud M.F., Vaillier J., Velours J., Haraux F. Investigation of the role and mechanism of IF1 and STF1 proteins, twin inhibitory peptides which interact with the yeast mitochondrial ATP synthase. Biochemistry 2003, 42:7626-7636.
    • (2003) Biochemistry , vol.42 , pp. 7626-7636
    • Venard, R.1    Brethes, D.2    Giraud, M.F.3    Vaillier, J.4    Velours, J.5    Haraux, F.6
  • 17
    • 0023013496 scopus 로고
    • Amino acid substitutions in mitochondrial ATPase subunit 6 of Saccharomyces cerevisiae leading to oligomycin resistance
    • John U.P., Nagley P. Amino acid substitutions in mitochondrial ATPase subunit 6 of Saccharomyces cerevisiae leading to oligomycin resistance. FEBS Lett. 1986, 207:79-83.
    • (1986) FEBS Lett. , vol.207 , pp. 79-83
    • John, U.P.1    Nagley, P.2
  • 18
    • 0023685584 scopus 로고
    • DNA sequence analysis of the Olir2-76 and Ossr1-92 alleles of the Oli-2 region of the yeast Saccharomyces cerevisiae. Analysis of related amino-acid substitutions and protein-antibiotic interaction
    • Ray M.K., Connerton I.F., Griffiths D.E. DNA sequence analysis of the Olir2-76 and Ossr1-92 alleles of the Oli-2 region of the yeast Saccharomyces cerevisiae. Analysis of related amino-acid substitutions and protein-antibiotic interaction. Biochim. Biophys. Acta 1988, 951:213-219.
    • (1988) Biochim. Biophys. Acta , vol.951 , pp. 213-219
    • Ray, M.K.1    Connerton, I.F.2    Griffiths, D.E.3
  • 19
    • 0024287185 scopus 로고
    • NH2-terminal sequence of the isolated yeast ATP synthase subunit 6 reveals post-translational cleavage
    • Michon T., Galante M., Velours J. NH2-terminal sequence of the isolated yeast ATP synthase subunit 6 reveals post-translational cleavage. Eur. J. Biochem. 1988, 172:621-625.
    • (1988) Eur. J. Biochem. , vol.172 , pp. 621-625
    • Michon, T.1    Galante, M.2    Velours, J.3
  • 20
    • 37549038676 scopus 로고    scopus 로고
    • The leader peptide of yeast Atp6p is required for efficient interaction with the Atp9p ring of the mitochondrial ATPase
    • Zeng X., Kucharczyk R., di Rago J.P., Tzagoloff A. The leader peptide of yeast Atp6p is required for efficient interaction with the Atp9p ring of the mitochondrial ATPase. J. Biol. Chem. 2007, 282:36167-36176.
    • (2007) J. Biol. Chem. , vol.282 , pp. 36167-36176
    • Zeng, X.1    Kucharczyk, R.2    di Rago, J.P.3    Tzagoloff, A.4
  • 21
    • 0034104291 scopus 로고    scopus 로고
    • The assembly factor Atp11p binds to the beta-subunit of the mitochondrial F(1)-ATPase
    • Wang Z.G., Ackerman S.H. The assembly factor Atp11p binds to the beta-subunit of the mitochondrial F(1)-ATPase. J. Biol. Chem. 2000, 275:5767-5772.
    • (2000) J. Biol. Chem. , vol.275 , pp. 5767-5772
    • Wang, Z.G.1    Ackerman, S.H.2
  • 22
    • 0029966509 scopus 로고    scopus 로고
    • Identification of functional domains in Atp11p. Protein required for assembly of the mitochondrial F1-ATPase in yeast
    • Wang Z.G., Ackerman S.H. Identification of functional domains in Atp11p. Protein required for assembly of the mitochondrial F1-ATPase in yeast. J. Biol. Chem. 1996, 271:4887-4894.
    • (1996) J. Biol. Chem. , vol.271 , pp. 4887-4894
    • Wang, Z.G.1    Ackerman, S.H.2
  • 23
    • 57649119786 scopus 로고    scopus 로고
    • Structural interactions between transmembrane helices 4 and 5 of subunit a and the subunit c ring of Escherichia coli ATP synthase
    • Moore K.J., Fillingame R.H. Structural interactions between transmembrane helices 4 and 5 of subunit a and the subunit c ring of Escherichia coli ATP synthase. J. Biol. Chem. 2008, 283:31726-31735.
    • (2008) J. Biol. Chem. , vol.283 , pp. 31726-31735
    • Moore, K.J.1    Fillingame, R.H.2
  • 24
    • 33644686458 scopus 로고    scopus 로고
    • Function, structure, and biogenesis of mitochondrial ATP synthase
    • Ackerman S.H., Tzagoloff A. Function, structure, and biogenesis of mitochondrial ATP synthase. Prog. Nucleic Acid Res. Mol. Biol. 2005, 80:95-133.
    • (2005) Prog. Nucleic Acid Res. Mol. Biol. , vol.80 , pp. 95-133
    • Ackerman, S.H.1    Tzagoloff, A.2
  • 25
    • 33846813499 scopus 로고    scopus 로고
    • The metalloprotease encoded by ATP23 has a dual function in processing and assembly of subunit 6 of mitochondrial ATPase
    • Zeng X., Neupert W., Tzagoloff A. The metalloprotease encoded by ATP23 has a dual function in processing and assembly of subunit 6 of mitochondrial ATPase. Mol. Biol. Cell 2007, 18:617-626.
    • (2007) Mol. Biol. Cell , vol.18 , pp. 617-626
    • Zeng, X.1    Neupert, W.2    Tzagoloff, A.3
  • 26
    • 2442489927 scopus 로고    scopus 로고
    • Atp10p assists assembly of Atp6p into the F0 unit of the yeast mitochondrial ATPase
    • Tzagoloff A., Barrientos A., Neupert W., Herrmann J.M. Atp10p assists assembly of Atp6p into the F0 unit of the yeast mitochondrial ATPase. J. Biol. Chem. 2004, 279:19775-19780.
    • (2004) J. Biol. Chem. , vol.279 , pp. 19775-19780
    • Tzagoloff, A.1    Barrientos, A.2    Neupert, W.3    Herrmann, J.M.4
  • 27
    • 0035794147 scopus 로고    scopus 로고
    • Identification of a nuclear gene (FMC1) required for the assembly/stability of yeast mitochondrial F(1)-ATPase in heat stress conditions
    • Lefebvre-Legendre L., Vaillier J., Benabdelhak H., Velours J., Slonimski P.P., di Rago J.P. Identification of a nuclear gene (FMC1) required for the assembly/stability of yeast mitochondrial F(1)-ATPase in heat stress conditions. J. Biol. Chem. 2001, 276:6789-6796.
    • (2001) J. Biol. Chem. , vol.276 , pp. 6789-6796
    • Lefebvre-Legendre, L.1    Vaillier, J.2    Benabdelhak, H.3    Velours, J.4    Slonimski, P.P.5    di Rago, J.P.6
  • 28
    • 56349099660 scopus 로고    scopus 로고
    • Suppression mechanisms of COX assembly defects in yeast and human: insights into the COX assembly process
    • Barrientos A., Gouget K., Horn D., Soto I.C., Fontanesi F. Suppression mechanisms of COX assembly defects in yeast and human: insights into the COX assembly process. Biochim. Biophys. Acta 2009, 1793:97-107.
    • (2009) Biochim. Biophys. Acta , vol.1793 , pp. 97-107
    • Barrientos, A.1    Gouget, K.2    Horn, D.3    Soto, I.C.4    Fontanesi, F.5
  • 29
    • 0037341660 scopus 로고    scopus 로고
    • F1-catalysed ATP hydrolysis is required for mitochondrial biogenesis in Saccharomyces cerevisiae growing under conditions where it cannot respire
    • Lefebvre-Legendre L., Balguerie A., Duvezin-Caubet S., Giraud M.F., Slonimski P.P., Di Rago J.P. F1-catalysed ATP hydrolysis is required for mitochondrial biogenesis in Saccharomyces cerevisiae growing under conditions where it cannot respire. Mol. Microbiol. 2003, 47:1329-1339.
    • (2003) Mol. Microbiol. , vol.47 , pp. 1329-1339
    • Lefebvre-Legendre, L.1    Balguerie, A.2    Duvezin-Caubet, S.3    Giraud, M.F.4    Slonimski, P.P.5    Di Rago, J.P.6
  • 30
    • 0037726805 scopus 로고    scopus 로고
    • Intrinsic and extrinsic uncoupling of oxidative phosphorylation
    • Kadenbach B. Intrinsic and extrinsic uncoupling of oxidative phosphorylation. Biochim. Biophys. Acta 2003, 1604:77-94.
    • (2003) Biochim. Biophys. Acta , vol.1604 , pp. 77-94
    • Kadenbach, B.1
  • 31
    • 0033515548 scopus 로고    scopus 로고
    • Oligomycin induces a decrease in the cellular content of a pathogenic mutation in the human mitochondrial ATPase 6 gene
    • Manfredi G., Gupta N., Vazquez-Memije M.E., Sadlock J.E., Spinazzola A., De Vivo D.C., Schon E.A. Oligomycin induces a decrease in the cellular content of a pathogenic mutation in the human mitochondrial ATPase 6 gene. J. Biol. Chem. 1999, 274:9386-9391.
    • (1999) J. Biol. Chem. , vol.274 , pp. 9386-9391
    • Manfredi, G.1    Gupta, N.2    Vazquez-Memije, M.E.3    Sadlock, J.E.4    Spinazzola, A.5    De Vivo, D.C.6    Schon, E.A.7
  • 34
    • 33847702960 scopus 로고    scopus 로고
    • ATP6 homoplasmic mutations inhibit and destabilize the human F1F0-ATP synthase without preventing enzyme assembly and oligomerization
    • Cortes-Hernandez P., Vazquez-Memije M.E., Garcia J.J. ATP6 homoplasmic mutations inhibit and destabilize the human F1F0-ATP synthase without preventing enzyme assembly and oligomerization. J. Biol. Chem. 2007, 282:1051-1058.
    • (2007) J. Biol. Chem. , vol.282 , pp. 1051-1058
    • Cortes-Hernandez, P.1    Vazquez-Memije, M.E.2    Garcia, J.J.3
  • 35
    • 0029006067 scopus 로고
    • Altered properties of mitochondrial ATP-synthase in patients with a T->G mutation in the ATPase 6 (subunit a) gene at position 8993 of mtDNA
    • Houstek J., Klement P., Hermanska J., Houstkova H., Hansikova H., Van den Bogert C., Zeman J. Altered properties of mitochondrial ATP-synthase in patients with a T->G mutation in the ATPase 6 (subunit a) gene at position 8993 of mtDNA. Biochim. Biophys. Acta 1995, 1271:349-357.
    • (1995) Biochim. Biophys. Acta , vol.1271 , pp. 349-357
    • Houstek, J.1    Klement, P.2    Hermanska, J.3    Houstkova, H.4    Hansikova, H.5    Van den Bogert, C.6    Zeman, J.7
  • 36
    • 0035794142 scopus 로고    scopus 로고
    • Impaired ATP synthase assembly associated with a mutation in the human ATP synthase subunit 6 gene
    • Nijtmans L.G., Henderson N.S., Attardi G., Holt I.J. Impaired ATP synthase assembly associated with a mutation in the human ATP synthase subunit 6 gene. J. Biol. Chem. 2001, 276:6755-6762.
    • (2001) J. Biol. Chem. , vol.276 , pp. 6755-6762
    • Nijtmans, L.G.1    Henderson, N.S.2    Attardi, G.3    Holt, I.J.4
  • 39
    • 0029904234 scopus 로고    scopus 로고
    • Expression of a recoded nuclear gene inserted into yeast mitochondrial DNA is limited by mRNA-specific translational activation
    • Steele D.F., Butler C.A., Fox T.D. Expression of a recoded nuclear gene inserted into yeast mitochondrial DNA is limited by mRNA-specific translational activation. Proc. Natl. Acad. Sci. U. S. A. 1996, 93:5253-5257.
    • (1996) Proc. Natl. Acad. Sci. U. S. A. , vol.93 , pp. 5253-5257
    • Steele, D.F.1    Butler, C.A.2    Fox, T.D.3
  • 40
    • 0026726161 scopus 로고
    • Characterization of ATP11 and detection of the encoded protein in mitochondria of Saccharomyces cerevisiae
    • Ackerman S.H., Martin J., Tzagoloff A. Characterization of ATP11 and detection of the encoded protein in mitochondria of Saccharomyces cerevisiae. J. Biol. Chem. 1992, 267:7386-7394.
    • (1992) J. Biol. Chem. , vol.267 , pp. 7386-7394
    • Ackerman, S.H.1    Martin, J.2    Tzagoloff, A.3


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