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Supercomplexes and subcomplexes of mitochondrial oxidative phosphorylation
Open accessAbstract
Dimerization or oligomerization of ATP synthase has been proposed to play an important role for mitochondrial cristae formation and to be involved in regulating ATP synthase activity. We found comparable oligomycin-sensitive ATPase activity for monomeric and oligomeric ATP synthase suggesting that oligomerization/monomerization dynamics are not directly involved in regulating ATP synthase activity. Binding of the natural IF1 inhibitor protein has been shown to induce dimerization of F1-subcomplexes. This suggested that binding of IF1 might also dimerize holo ATP synthase, and possibly link dimerization and inhibition. Analyzing mitochondria of human rho zero cells that contain mitochondria but lack mitochondrial DNA, we identified three subcomplexes of ATP synthase: (i) F1 catalytic domain, (ii) F1-domain with bound IF1, and (iii) F1-c subcomplex with bound IF1 and a ring of subunits c. Since both IF1 containing subcomplexes were present in monomeric state and exhibited considerably reduced ATPase activity as compared to the third subcomplex lacking IF1, we postulate that inhibition and induction of dimerization of F1-subcomplexes by IF1 are independent events. F1-subcomplexes were also found in mitochondria of patients with specific mitochondrial disorders, and turned out to be useful for the clinical differentiation between various types of mitochondrial biosynthesis disorders. Supramolecular associations of respiratory complexes, the "respirasomes", seem not to be the largest assemblies in the structural organization of the respiratory chain, as suggested by differential solubilization of mitochondria and electron microscopic analyses of whole mitochondria. We present a model for a higher supramolecular association of respirasomes into a "respiratory string".
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- Allen, 1989, An investigation of mitochondrial inner membranes by rapid-freeze deep-etch techniques, J. Cell Biol., 108, 2233, 10.1083/jcb.108.6.2233 · DOI · Paper page
- Allen, 1995, Membrane tubulation and proton pumps, Protoplasma, 189, 1, 10.1007/BF01280286 · DOI
- Frey, 2000, The internal structure of mitochondria, TIBS, 25, 319
- Arnold, 1998, Yeast mitochondrial F1FO-ATP synthase exists as a dimer: identification of three dimer-specific subunits, EMBO J., 17, 7170, 10.1093/emboj/17.24.7170 · DOI · Paper page
- Schägger, 2000, Supercomplexes in the respiratory chains of yeast and mammalian mitochondria, EMBO J., 19, 1777, 10.1093/emboj/19.8.1777 · DOI · Paper page
- Eubel, 2003, New insights into the respiratory chain of plant mitochondria: supercomplexes and a unique composition of complex II, Plant Physiol., 133, 274, 10.1104/pp.103.024620 · DOI · Paper page
- Paumard, 2002, The ATP synthase is involved in generating mitochondrial cristae morphology, EMBO J., 21, 221, 10.1093/emboj/21.3.221 · DOI · Paper page
- Van Lis, 2003, Identification of novel mitochondrial protein components of Chlamydomonas reinhardtii. A proteomic approach, Plant Physiol., 132, 318, 10.1104/pp.102.018325 · DOI
- Rexroth, 2004, Dimeric H+-ATP synthase in the chloroplast of Chlamydomonas reinhardtii, Biochim. Biophys. Acta, 1658, 202, 10.1016/j.bbabio.2004.05.014 · DOI
- Schägger, 1994, Analysis of molecular masses and oligomeric states of protein complexes by blue native electrophoresis and isolation of membrane protein complexes by two-dimensional native electrophoresis, Anal. Biochem., 217, 220, 10.1006/abio.1994.1112 · DOI · Paper page
- Arselin, 2004, The modulation in subunits e and g amounts of yeast ATP synthase modifies mitochondrial cristae morphology, J. Biol. Chem., 279, 40392, 10.1074/jbc.M404316200 · DOI
- Wittig, 2005, Advantages and limitations of clear-native PAGE, Proteomics, 5, 4338, 10.1002/pmic.200500081 · DOI · Paper page
- Pfeiffer, 2003, Cardiolipin stabilizes respiratory chain supercomplexes, J. Biol. Chem., 278, 52873, 10.1074/jbc.M308366200 · DOI · Paper page
- Gavin, 2003, FRET reveals changes in the F1-stator stalk interaction during activity of F1FO-ATP synthase, Biochim. Biophys. Acta, 1607, 167, 10.1016/j.bbabio.2003.09.013 · doi:10.1016/j.bbabio.2003.09.013
- Krause, 2005, Active oligomeric ATP synthases in mammalian mitochondria, Biochem. Biophys. Res. Commun., 329, 583, 10.1016/j.bbrc.2005.02.010 · DOI
- Zerbetto, 1997, Quantification of muscle mitochondrial oxidative phosphorylation enzymes via histochemical staining of blue native polyacrylamide gels, Electrophoresis, 18, 2059, 10.1002/elps.1150181131 · DOI · Paper page
- Dienhart, 2002, Formation of the yeast F1FO-ATP synthase dimeric complex does not require the ATPase inhibitor protein Inh1, J. Biol. Chem., 277, 39289, 10.1074/jbc.M205720200 · DOI
- Cabezon, 2000, Modulation of the oligomerization state of the bovine F1-ATPase inhibitor protein, IF1, by pH, J. Biol. Chem., 275, 25460, 10.1074/jbc.M003859200 · DOI · Paper page
- Cabezon, 2003, The structure of bovine F1-ATPase in complex with its regulatory protein IF1, Nat. Struct. Biol., 10, 744, 10.1038/nsb966 · DOI · Paper page
- Tomasetig, 2002, Dimerization of F0F1ATP synthase from bovine heart is independent from the binding of the inhibitor protein IF1, Biochim. Biophys. Acta, 1556, 133, 10.1016/S0005-2728(02)00344-4 · DOI
- Carrozzo, 2006, Subcomplexes of human ATP synthase mark mitochondrial biosynthesis disorders, Ann. Neurol., 59, 265, 10.1002/ana.20729 · DOI
- Gavin, 2005, Yeast F1FO-ATP synthase complex interactions in vivo can occur in the absence of the dimer specific subunit e, J. Bioenerg. Biomembranes, 37, 55, 10.1007/s10863-005-4128-8 · DOI
- Spannagel, 1998, Evidence of a subunit 4 (subunit b) dimer in favor of the proximity of ATP synthase complexes in yeast inner mitochondrial membrane, Biochim. Biophys. Acta, 1414, 260, 10.1016/S0005-2736(98)00174-6 · DOI
- Paumard, 2002, Two ATP synthases can be linked through subunits i in the inner mitochondrial membrane of Saccharomyces cerevisiae, Biochemistry, 41, 10390, 10.1021/bi025923g · DOI
- Everard-Gigot, 2005, Functional analysis of subunit e of the F1FO-ATP synthase of the yeast Saccharomyces cerevisiae: importance of the N-terminal membrane anchor region, Eukaryot. Cell, 4, 346, 10.1128/EC.4.2.346-355.2005 · DOI
- Saddar, 2005, The yeast F1FO-ATP synthase. Analysis of the molecular organization of subunit g and the importance of a conserved GXXXG motif, J. Biol. Chem., 280, 24435, 10.1074/jbc.M502804200 · DOI
- Bustos, 2005, The modification of the conserved GXXXG motif of the membrane-spanning segment of subunit g destabilizes the supramolecular species of yeast ATP synthase, J. Biol. Chem., 280, 29004, 10.1074/jbc.M502140200 · DOI
- Minauro-Sanmiguel, 2005, Structure of dimeric mitochondrial ATP synthase: novel FO bridging features and the structural basis of mitochondrial cristae biogenesis, PNAS, 102, 12356, 10.1073/pnas.0503893102 · DOI · Paper page
- Dudkina, 2005, Structure of dimeric ATP synthase from mitochondria: an angular association of monomers induces the strong curvature of the inner membrane, FEBS Lett., 579, 5769, 10.1016/j.febslet.2005.09.065 · DOI · Paper page
- King, 1996, Isolation of human cell lines lacking mitochondrial DNA, Methods Enzymol., 264, 304, 10.1016/S0076-6879(96)64029-4 · DOI · Paper page
- Hadikusumo, 1988, The definition of mitochondrial H+-ATPase assembly defects in mit− mutants of Saccharomyces cerevisiae with a monoclonal antibody to the enzyme complex as an assembly probe, Biochem. Biophys. Acta, 933, 212
- Hermolin, 1995, Assembly of FO sector of Escherichia coli H+ ATP synthase, J. Biol. Chem., 270, 2815, 10.1074/jbc.270.6.2815 · DOI
- Tzagoloff, 2004, Atp10p assists assembly of Atp6p into the FO unit of the yeast mitochondrial ATPase, J. Biol. Chem., 279, 19775, 10.1074/jbc.M401506200 · DOI
- Stock, 1999, Molecular architecture of the rotary motor in ATP synthase, Science, 286, 1700, 10.1126/science.286.5445.1700 · DOI · Paper page
- Moraes, 1991, mtDNA depletion with variable tissue expression: a novel genetic abnormality in mitochondrial diseases, Am. J. Hum. Genet., 48, 492
- Taanman, 1997, Molecular mechanisms in mitochondrial DNA depletion syndrome, Hum. Mol. Genet., 6, 935, 10.1093/hmg/6.6.935 · DOI
- Santorelli, 2002, Hypertrophic cardiomyopathy and mtDNA depletion. Successful treatment with heart transplantation, Neuromuscul. Disord., 12, 56, 10.1016/S0960-8966(01)00248-6 · doi:10.1016/s0960-8966(01)00248-6
- Carrozzo, 2003, Mutation analysis in 16 patients with mtDNA depletion, Hum. Mutat., 21, 453, 10.1002/humu.9135 · DOI
- Vu, 1998, Clinical manifestations of mitochondrial DNA depletion, Neurology, 50, 1783, 10.1212/WNL.50.6.1783 · DOI
- Holt, 1990, A new mitochondrial disease associated with mitochondrial DNA heteroplasmy, Am. J. Hum. Genet., 46, 428
- DiMauro, 1996, Genetic heterogeneity in Leigh syndrome, Ann. Neurol., 40, 5, 10.1002/ana.410400104 · DOI · Paper page
- Carrozzo, 2001, The T9176G mtDNA mutation severly affects ATP production and results in Leigh syndrome, Neurology, 56, 687, 10.1212/WNL.56.5.687 · DOI
- Coenen, 2004, Mutant mitochondrial elongation factor G1 and combined oxidative phosphorylation deficiency, New Engl. J. Med., 351, 2080, 10.1056/NEJMoa041878 · DOI · Paper page
- Jacobs, 2005, Nuclear genes and mitochondrial translation: a new class of genetic disease, Trends Genet., 21, 312, 10.1016/j.tig.2005.04.003 · DOI
- Schägger, 2001, The ratio of oxidative phosphorylation complexes I–V in bovine heart mitochondria and the composition of respiratory chain supercomplexes, J. Biol. Chem., 276, 37861, 10.1074/jbc.M106474200 · DOI · Paper page
- Schägger, 2004, Significance of respirasomes for the assembly/stability of human respiratory chain complex I, J. Biol. Chem., 279, 36349, 10.1074/jbc.M404033200 · DOI · Paper page
- Schägger, 2002, Respiratory chain supercomplexes of mitochondria and bacteria, Biochem. Biophys. Acta, 1555, 154
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Metadata
- Authors
- Wittig, I., Carrozzo, R., Santorelli, F. M., & Schägger, H.
- Venue
- Biochimica et Biophysica Acta (BBA) - Bioenergetics
- Publisher
- Elsevier BV
- Published
- 2006-09
- Volume
- 1757
- Issue
- 9-10
- Pages
- 1066-1072
- ISSN
- 0005-2728 (ISSN-L)
- OpenAlex
- W2021467041
- Cited by
- 219
- References
- 0
- FWCI
- 5.40
- Type
- journal article
- Language
- en
- Open access
- bronze
- Licence
- www.elsevier.com/tdm/userlicense/1.0/ (tdm)
- www.elsevier.com/open-access/userlicense/1.0/ (vor, 2547 d)
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