We study the physiology of mitochondria, cell organelles responsible for most of the energy production on the molecular level. We use both animal models and cells derived from patients harbouring various mitochondrial disorders. Our research is focused mainly on:
- Assembly of mitochondrial protein complexes and supercomplexes.
- Human diseases caused by mutations in assembly factors of these enzyme complexes.
- Development of protocols for diagnostics of mitochondrial diseases using patient-derived lymphocytes.
- Identification of new mitochondrial genes that play a causal role in the metabolic syndrome and heart failure.
Currently we are hiring new postdoscs into our team. Interested? Find out more...
Projects
ATP produced by the mitochondrial FoF1-ATP synthase represents a major source of energy for aerobic organisms. The proposed project is aimed to shedding light on the functional consequences of ATP synthase deficiencies using a model of knock-down of small subunits of the catalytic F1 part of the mammalian ATP synthase (γ, δ and ε).
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To shed more light on whether OXPHOS defects can play a role in the development of heart failure, we analyse samples from patients undergoing heart transplants. We search for characteristic markers, which would be suitable for identification of new patients, as well as new potential targets for treatment.
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Using lymphocytes isolated from peripheral blood, we try to develop new diagnostic protocols for patients with suspected mitochondrial diseases.
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ATP synthase defects represent an important subgroup of inborn errors of metabolism. It may not be surprising if we take into account that ATP synthase is one of the key energy producing enzymes in a cell. We study biogenesis of this enzyme complex and the role of various other proteins in this process.
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Achievements
A developing organism requires high amounts of energy in the form of ATP. In higher eukaryotes, and thus in humans, more than 90 % of ATP is produced in mitochondria, a key organelle of the cellular catabolism. It is therefore not surprising that mitochondrial defects belong to the most frequent causes of metabolic diseases in children.
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A brief list of what we published in 2014
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There is no fire without smoke. Similarly, cell respiration does not work perfectly and its by-products – reactive oxygen species (ROS), also known as free oxygen radicals – play a negative role in the development of many diseases as well as in aging.
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Publications
Trnovská, J. - Šilhavý, Jan - Zídek, Václav - Šimáková, Miroslava - Mlejnek, Petr - Landa, Vladimír - Eigner, Sebastian - Eigner-Henke, Kateřina - Škop, V. - Oliyarnyk, O. - Kazdová, L. - Mráček, Tomáš - Houštěk, Josef - Pravenec, Michal
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Gender-related effects on substrate utilization and metabolic adaptation in hairless spontaneously hypertensive rat
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Physiological Research. 2015, Vol. 64, 1, p. 51-60
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IF = 1.293
[ASEP]
Nůsková, Hana - Mráček, Tomáš - Mikulová, Tereza - Vrbacký, Marek - Kovářová, Nikola - Kovalčíková, Jana - Pecina, Petr - Houštěk, Josef
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Mitochondrial ATP synthasome: Expression and structural interaction of its components
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Biochemical and Biophysical Research Communications. 2015, Vol. 464, 3, p. 787-793
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IF = 2.297
[ASEP]
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doi
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Klučková, Katarína - Sticha, M. - Černý, Jiří - Mráček, Tomáš - Dong, L. - Drahota, Zdeněk - Gottlieb, E. - Neužil, Jiří - Rohlena, Jakub
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Ubiquinone-binding site mutagenesis reveals the role of mitochondrial complex II in cell death initiation
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Cell Death & Disease. 2015, Vol. 6, May 2015, e1749
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IF = 5.014
[ASEP]
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doi
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Hejzlarová, Kateřina - Kaplanová, Vilma - Nůsková, Hana - Kovářová, Nikola - Ješina, Pavel - Drahota, Zdeněk - Mráček, Tomáš - Seneca, S. - Houštěk, Josef
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Alteration of structure and function of ATP synthase and cytochrome c oxidase by lack of F-o-a and Cox3 subunits caused by mitochondrial DNA 9205delTA mutation
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Biochemical Journal. 2015, Vol. 466, 3, p. 601-611
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IF = 4.396
[ASEP]
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doi
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Cahová, M. - Páleníčková, E. - Danková, H. - Sticová, E. - Burian, M. - Drahota, Zdeněk - Červinková, Z. - Kučera, O. - Gladkova, Ch. - Stopka, Pavel - Křížová, Jana - Papáčková, Z. - Oliyarnyk, O. - Kazdová, L.
Metformin prevents ischemia reperfusion-induced oxidative stress in the fatty liver by attenuation of reactive oxygen species formation
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American Journal of Physiology-Gastrointestinal and Liver Physiology. 2015, Vol. 309, 2, G100-G111
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IF = 3.798
[ASEP]
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doi
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Cahová, M. - Chrastina, P. - Hansíková, H. - Drahota, Zdeněk - Trnovská, J. - Škop, V. - Spáčilová, J. - Malínská, H. - Oliyarnyk, O. - Papáčková, Z. - Páleníčková, E. - Kazdová, L.
Carnitine supplementation alleviates lipid metabolism derangements and protects against oxidative stress in non-obese hereditary hypertriglyceridemic rats
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Applied Physiology Nutrition and Metabolism-Physiologie appliquee nutrition et metabolisme. 2015, Vol. 40, 3, p. 280-291
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IF = 2.339
[ASEP]
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doi
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Shabalina, I.G. - Vrbacký, Marek - Pecinová, Alena - Kalinovich, A. V. - Drahota, Zdeněk - Houštěk, Josef - Mráček, Tomáš - Cannon, B. - Nedergaard, J.
ROS production in brown adipose tissue mitochondria: The question of UCP1-dependence
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Biochimica Et Biophysica Acta-Bioenergetics. 2014, roč. 1837, 12, p. 2017-2030
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IF = 5.353
[ASEP]
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doi
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Rauchová, Hana - Vokurková, Martina - Drahota, Zdeněk
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Inhibition of mitochondrial glycerol-3-phosphate dehydrogenase by alpha-tocopheryl succinate
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International Journal of Biochemistry and Cell Biology 2014, roč. 53, aug, p. 409-413. ISSN 1357-2725.
IF = 4.046
[ASEP]
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doi
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Mráček, Tomáš - Holzerová, Eliška - Drahota, Zdeněk - Kovářová, Nikola - Vrbacký, Marek - Ješina, Pavel - Houštěk, Josef
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ROS generation and multiple forms of mammalian mitochondrial glycerol-3-phosphate dehydrogenase
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Biochimica Et Biophysica Acta-Bioenergetics 2014, roč. 1837, 1, p. 98-111. ISSN 0005-2728.
IF = 5.353
[ASEP]
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doi
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Kratochvílová, H. - Hejzlarová, Kateřina - Vrbacký, Marek - Mráček, Tomáš - Karbanová, Vendula - Tesařová, M. - Gombitová, A. - Cmarko, D. - Wittig, I. - Zeman, J. - Houštěk, Josef
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Mitochondrial membrane assembly of TMEM70 protein
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Mitochondrion 2014, roč. 15, mar 2014, p. 1-9. ISSN 1567-7249.
IF = 3.249
[ASEP]
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doi
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Tissue cultures - our important model
2D electrophoresis of heart mitochondria
