메뉴 건너뛰기




Volumn 14, Issue 1-2, 2004, Pages 7-11

Mitochondrial Membrane Potential and ATP Production in Primary Disorders of ATP Synthase

Author keywords

ATP Synthase; ATP6; Flow Cytometry; Membrane Potential; Mitochondrial diseases; MtDNA

Indexed keywords

ADENOSINE DIPHOSPHATE; ADENOSINE TRIPHOSPHATE; MITOCHONDRIAL DNA; PROTON TRANSPORTING ADENOSINE TRIPHOSPHATE SYNTHASE; REACTIVE OXYGEN METABOLITE;

EID: 10744232647     PISSN: 15376516     EISSN: 15376524     Source Type: Journal    
DOI: 10.1080/15376520490257347     Document Type: Conference Paper
Times cited : (18)

References (38)
  • 4
    • 0028810803 scopus 로고
    • Bilateral striatal necrosis with a novel point mutation in the mitochondrial ATPase 6 gene
    • De Meirleir, L., Seneca, S., Lissens, W., Schoentjes, E., and Desprechins, B. 1995. Bilateral striatal necrosis with a novel point mutation in the mitochondrial ATPase 6 gene. Pediatr. Neurol. 13:242-246.
    • (1995) Pediatr. Neurol. , vol.13 , pp. 242-246
    • De Meirleir, L.1    Seneca, S.2    Lissens, W.3    Schoentjes, E.4    Desprechins, B.5
  • 5
    • 0031965039 scopus 로고    scopus 로고
    • Fulminant Leigh syndrome and sudden unexpected death in a family with the T9176C mutation of the mitochondrial ATPase 6 gene
    • Dionisi-Vici, C., Seneca, S., Zeviani, M., Fariello, G., Rimoldi, M., Bertini, E., and De Meirleir, L. 1998. Fulminant Leigh syndrome and sudden unexpected death in a family with the T9176C mutation of the mitochondrial ATPase 6 gene. J. Inherit. Metab. Dis. 21:2-8.
    • (1998) J. Inherit. Metab. Dis. , vol.21 , pp. 2-8
    • Dionisi-Vici, C.1    Seneca, S.2    Zeviani, M.3    Fariello, G.4    Rimoldi, M.5    Bertini, E.6    De Meirleir, L.7
  • 6
    • 84874756802 scopus 로고    scopus 로고
    • Use of GUG as initiation codon for the human mitochondria-encoded ATP6 subunit
    • European Science Foundation, PD4/171
    • Dubot, A., Godinot, C., Vojtiskova, A., Pecina, P., Jesina, P., and Houstek, J. 2003. Use of GUG as initiation codon for the human mitochondria-encoded ATP6 subunit. In Functional Genomics and Disease, European Science Foundation, PD4/171, http://www.esffg2003.org.
    • (2003) Functional Genomics and Disease
    • Dubot, A.1    Godinot, C.2    Vojtiskova, A.3    Pecina, P.4    Jesina, P.5    Houstek, J.6
  • 7
    • 0033039069 scopus 로고    scopus 로고
    • Tetramethyl rhodamine methyl ester (TMRM) is suitable for cytofluorometric measurements of mitochondrial membrane potential in cells treated with digitonin
    • Floryk, D., and Houstek, J. 1999. Tetramethyl rhodamine methyl ester (TMRM) is suitable for cytofluorometric measurements of mitochondrial membrane potential in cells treated with digitonin. Biosci. Rep. 19:27-34.
    • (1999) Biosci. Rep. , vol.19 , pp. 27-34
    • Floryk, D.1    Houstek, J.2
  • 8
    • 0842321030 scopus 로고    scopus 로고
    • New mtDNA mutation 9204delTA in a family with mitochondrial encephalopathy and ATP-synthase defect
    • Fornuskova, D., Tesarova, M., Hansikova, H., and Zeman, J. 2003. New mtDNA mutation 9204delTA in a family with mitochondrial encephalopathy and ATP-synthase defect. Cas. Lek. Cesk. 142:313.
    • (2003) Cas. Lek. Cesk. , vol.142 , pp. 313
    • Fornuskova, D.1    Tesarova, M.2    Hansikova, H.3    Zeman, J.4
  • 10
    • 0025267548 scopus 로고
    • A new mitochondrial disease associated with mitochondrial DNA heteroplasmy
    • Holt, I. J., Harding, A. E., Petty, R. K. H., and Morgan-Hughes, J. A. 1990. A new mitochondrial disease associated with mitochondrial DNA heteroplasmy. Am. J. Hum. Genet. 46:428-433.
    • (1990) Am. J. Hum. Genet. , vol.46 , pp. 428-433
    • Holt, I.J.1    Harding, A.E.2    Petty, R.K.H.3    Morgan-Hughes, J.A.4
  • 12
    • 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., and Zeman, J. 1995. 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 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
  • 13
    • 0035902609 scopus 로고    scopus 로고
    • Energy-driven subunit rotation at the interface between subunit a and the c oligomer in the F(O) sector of Escherichia coli ATP synthase
    • Hutcheon, M. L., Duncan, T. M., Ngai, H., and Cross, R. L. 2001. Energy-driven subunit rotation at the interface between subunit a and the c oligomer in the F(O) sector of Escherichia coli ATP synthase. Proc. Natl. Acad. Sci. USA 98:8519-8524.
    • (2001) Proc. Natl. Acad. Sci. USA , vol.98 , pp. 8519-8524
    • Hutcheon, M.L.1    Duncan, T.M.2    Ngai, H.3    Cross, R.L.4
  • 14
    • 0025019341 scopus 로고
    • Organization of the mitochondrial genome of Atlantic cod, Gadus morhua
    • Johansen, S., Guddal, P. H., and Johansen, T. 1990. Organization of the mitochondrial genome of Atlantic cod, Gadus morhua. Nucleic Acids Res. 18:411-419.
    • (1990) Nucleic Acids Res. , vol.18 , pp. 411-419
    • Johansen, S.1    Guddal, P.H.2    Johansen, T.3
  • 15
    • 0037726805 scopus 로고    scopus 로고
    • Intrinsic and extrinsic uncoupling of oxidative phosphorylation
    • Kadenbach, B. 2003. Intrinsic and extrinsic uncoupling of oxidative phosphorylation. Biochim. Biophys. Acta 1604:77-94.
    • (2003) Biochim. Biophys. Acta , vol.1604 , pp. 77-94
    • Kadenbach, B.1
  • 17
    • 0030729851 scopus 로고    scopus 로고
    • High protonic potential actuates a mechanism of production of reactive oxygen species in mitochondria
    • Korshunov, S. S., Skulachev, V. P., and Starkov, A. A. 1997. High protonic potential actuates a mechanism of production of reactive oxygen species in mitochondria. FEBS Lett. 416:15-18.
    • (1997) FEBS Lett. , vol.416 , pp. 15-18
    • Korshunov, S.S.1    Skulachev, V.P.2    Starkov, A.A.3
  • 19
    • 0033381362 scopus 로고    scopus 로고
    • Cooperation of a "reactive oxygen cycle" with the Q cycle and the proton cycle in the respiratory chain: Superoxide generating and cycling mechanisms in mitochondria
    • Liu, S. S. 1999. Cooperation of a "reactive oxygen cycle" with the Q cycle and the proton cycle in the respiratory chain: superoxide generating and cycling mechanisms in mitochondria. J. Bioenerg. Biomembr. 31:367-376.
    • (1999) J. Bioenerg. Biomembr. , vol.31 , pp. 367-376
    • Liu, S.S.1
  • 20
    • 84874756804 scopus 로고    scopus 로고
    • Increased uncoupling in a patient with a quantitative defect of the F0F1-ATP-synthase
    • Mayr, J. A., Paul, J., Kurnik, P., Fotschl, U., Houstek, J., and Sperl, W. 2002. Increased uncoupling in a patient with a quantitative defect of the F0F1-ATP-synthase. J. Inherit. Metab. Dis. 25(suppl. 1):178.
    • (2002) J. Inherit. Metab. Dis. , vol.25 , Issue.SUPPL. 1 , pp. 178
    • Mayr, J.A.1    Paul, J.2    Kurnik, P.3    Fotschl, U.4    Houstek, J.5    Sperl, W.6
  • 21
    • 0032532674 scopus 로고    scopus 로고
    • Mitochondrial ATP synthesis in permeabilized cells: Assessment of the ATP/O values in situ
    • Ouhabi, R., Boue-Grabot, M., and Mazat, J. P. 1998. Mitochondrial ATP synthesis in permeabilized cells: assessment of the ATP/O values in situ. Anal. Biochem. 263:169-175.
    • (1998) Anal. Biochem. , vol.263 , pp. 169-175
    • Ouhabi, R.1    Boue-Grabot, M.2    Mazat, J.P.3
  • 22
    • 0027203797 scopus 로고
    • A gene for cytochrome c oxidase subunit II in duck mitochondrial DNA: Structural features and sequence evolution
    • Pan, Y. F., Lee, Y. W., Wei, Y. H., and Chiang, A. N. 1993. A gene for cytochrome c oxidase subunit II in duck mitochondrial DNA: structural features and sequence evolution. Biochem. Mol. Biol. Int. 30:479-489.
    • (1993) Biochem. Mol. Biol. Int. , vol.30 , pp. 479-489
    • Pan, Y.F.1    Lee, Y.W.2    Wei, Y.H.3    Chiang, A.N.4
  • 25
    • 0033584845 scopus 로고    scopus 로고
    • Threshold effect and tissue specificity: Implication for mitochondrial cytopathies
    • Rossignol, R., Malgat, M., Mazat, J. P., and Letellier, T. 1999. Threshold effect and tissue specificity: implication for mitochondrial cytopathies. J. Biol. Chem. 274:33426-33432.
    • (1999) J. Biol. Chem. , vol.274 , pp. 33426-33432
    • Rossignol, R.1    Malgat, M.2    Mazat, J.P.3    Letellier, T.4
  • 26
    • 0035782974 scopus 로고    scopus 로고
    • Pathogenesis of primary defects in mitochondrial ATP synthesis
    • Schon, E. A., Santra, S., Pallotti, F., and Girvin, M. E. 2001. Pathogenesis of primary defects in mitochondrial ATP synthesis. Semin. Cell Dev. Biol. 12:441-448.
    • (2001) Semin. Cell Dev. Biol. , vol.12 , pp. 441-448
    • Schon, E.A.1    Santra, S.2    Pallotti, F.3    Girvin, M.E.4
  • 30
    • 0033607504 scopus 로고    scopus 로고
    • Molecular architecture of the rotary motor in ATP synthase
    • Stock, D., Leslie, A. G., and Walker, J. E. 1999. Molecular architecture of the rotary motor in ATP synthase. Science 286:1700-1705.
    • (1999) Science , vol.286 , pp. 1700-1705
    • Stock, D.1    Leslie, A.G.2    Walker, J.E.3
  • 32
    • 0027244336 scopus 로고
    • The mitochondrial DNA mutation at 8993 associated with NARP slows the rate of ATP synthesis in isolated lymphoblast mitochondria
    • Tatuch, Y., and Robinson, B. H. 1993. The mitochondrial DNA mutation at 8993 associated with NARP slows the rate of ATP synthesis in isolated lymphoblast mitochondria. Biochem. Biophys. Res. Commun. 192:124-128.
    • (1993) Biochem. Biophys. Res. Commun. , vol.192 , pp. 124-128
    • Tatuch, Y.1    Robinson, B.H.2
  • 33
    • 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., and DiMauro, S. 1995. A novel mitochondrial ATPase 6 point mutation in familial bilateral striatal necrosis. Ann. Neurol. 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
  • 34
    • 0028868612 scopus 로고
    • Nuclear DNA origin of cytochrome c oxidase deficiency in Leigh's syndrome: Genetic evidence based on patient-derived rho degrees transformants
    • Tiranti, V., Munaro, M., Sandona, D., Lamantea, E., Rimoldi, M., DiDonato, S., Bisson, R., and Zeviani, M. 1995. Nuclear DNA origin of cytochrome c oxidase deficiency in Leigh's syndrome: Genetic evidence based on patient-derived rho degrees transformants. Hum. Mol. Genet. 4:2017-2023.
    • (1995) Hum. Mol. Genet. , vol.4 , pp. 2017-2023
    • Tiranti, V.1    Munaro, M.2    Sandona, D.3    Lamantea, E.4    Rimoldi, M.5    DiDonato, S.6    Bisson, R.7    Zeviani, M.8
  • 35
    • 84874756612 scopus 로고    scopus 로고
    • Severe complex V deficiency with fatal outcome in neonatal period
    • ed. G. T. N. Besley. Prague, Czech Republic: Kluwer Academic
    • Van Coster, R., Smet, J., and Eyskens, F. 2001. Severe complex V deficiency with fatal outcome in neonatal period. In Society for the Study of Inborn Errors of Metabolism, ed. G. T. N. Besley, 24, p. 83. Prague, Czech Republic: Kluwer Academic.
    • (2001) Society for the Study of Inborn Errors of Metabolism , vol.24 , pp. 83
    • Van Coster, R.1    Smet, J.2    Eyskens, F.3
  • 36
    • 0028301141 scopus 로고
    • The role of the stalk in the coupling mechanism of F1F0-ATPases
    • Walker, J. E., and Collinson, I. R. 1994. The role of the stalk in the coupling mechanism of F1F0-ATPases. FEBS Lett. 346:39-43.
    • (1994) FEBS Lett. , vol.346 , pp. 39-43
    • Walker, J.E.1    Collinson, I.R.2
  • 37
    • 0033525773 scopus 로고    scopus 로고
    • Mitochondrial diseases in man and mouse
    • Wallace, D. C. 1999. Mitochondrial diseases in man and mouse. Science 283:1482-1488.
    • (1999) Science , vol.283 , pp. 1482-1488
    • Wallace, D.C.1
  • 38
    • 0027238928 scopus 로고
    • Studies on mitochondrial oxidative phosphorylation in permeabilized human skin fibroblasts: Application to mitochondrial encephalomyopathies
    • Wanders, R. J., Ruiter, J. P., and Wijburg, F. A. 1993. Studies on mitochondrial oxidative phosphorylation in permeabilized human skin fibroblasts: application to mitochondrial encephalomyopathies. Biochim. Biophys. Acta 1181:219-222.
    • (1993) Biochim. Biophys. Acta , vol.1181 , pp. 219-222
    • Wanders, R.J.1    Ruiter, J.P.2    Wijburg, F.A.3


* 이 정보는 Elsevier사의 SCOPUS DB에서 KISTI가 분석하여 추출한 것입니다.