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 here and here.
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
Wang, M. - Sips, P. - Khin, E. - Rotival, M. - Sun, X. - Ahmed, R. - Widjaja, A. A. - Schafer, S. - Yusoff, P. - Choksi, P. K. - Ko, N. S. J. - Singh, M. K. - Epstein, D. - Guan, Y. - Houštěk, Josef - Mráček, Tomáš - Nůsková, Hana - Mikell, B. - Tan, J. - Pesce, F. - Kolář, František - Bottolo, L. - Mancini, M. - Hubner, N. - Pravenec, Michal - Petretto, E. - MacRae, C. - Cook, S. A.
Wars2 is a determinant of angiogenesis
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Nature Communications. 2016, roč. 7, Jul, p. 12061
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IF = 11.329
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doi
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Kovářová, Nikola - Pecina, Petr - Nůsková, Hana - Vrbacký, Marek - Zeviani, M. - Mráček, Tomáš - Viscomi, C. - Houštěk, Josef
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Tissue- and species-specific differences in cytochrome c oxidase assembly induced by SURF1 defects
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Biochimica Et Biophysica Acta-Molecular Basis of Disease. 2016, roč. 1862, 4, p. 705-715
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IF = 5.158
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doi
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Česneková, J. - Rodinová, M. - Hansíková, H. - Houštěk, Josef - Zeman, J. - Stibůrek, L.
The mammalian homologue of yeast Afg1 ATPase (lactation elevated 1) mediates degradation of nuclear-encoded complex IV subunits
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Biochemical Journal. 2016, roč. 473, 6, p. 797-804
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IF = 3.562
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doi
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Ng, Y. S. - Alston, Ch. L. - Diodato, D. - Morris, A. A. - Ulrick, N. - Kmoch, S. - Houštěk, Josef - Martinelli, D. - Haghighi, A. - Atiq, M. - Gamero, M. A. - Garcia-Martinez, E. - Kratochvílová, H. - Santra, S. - Brown, R. M. - Brown, G. K. - Ragge, N. - Monavari, A. - Pysden, K. - Ravn, K. - Casey, J. P. - Khan, A. - Chakrapani, A. - Vassallo, G. - Simons, C. - McKeever, K. - O´Sullivan, S. - Childs, A.-M. - Ostergaard, E. - Vanderver, A. - Goldstein, A. - Vogt, J. - Taylor, R. W. - McFarland, R.
The clinical, biochemical and genetic features associated with RMND1-related mitochondrial disease
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Journal of Medical Genetics. 2016, roč. 53, 11, p. 768-775
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IF = 5.650
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doi
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Folbergrová, Jaroslava - Ješina, Pavel - Kubová, Hana - Druga, Rastislav - Otáhal, Jakub
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Status Epilepticus in Immature Rats Is Associated with Oxidative Stress and Mitochondrial Dysfunction
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Frontiers in Cellular Neuroscience. 2016, roč. 10, May 26, p. 136
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IF = 4.609
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doi
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Milerová, Marie - Drahota, Zdeněk - Chytilová, Anna - Tauchmannová, Kateřina - Houštěk, Josef - Ošťádal, Bohuslav
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Sex difference in the sensitivity of cardiac mitochondrial permeability transition pore to calcium load
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Molecular and Cellular Biochemistry. 2016, roč. 412, 1-2, p. 147-154
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IF = 2.613
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doi
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Heřmanová, I. - Arruabarrena-Aristorena, A. - Vališ, Karel - Nůsková, Hana - Alberich-Jorda, Meritxell - Fišer, K. - Fernandez-Ruiz, S. - Kavan, Daniel - Pecinová, Alena - Niso-Santano, N. - Žaliová, M. - Novák, Petr - Houštěk, Josef - Mráček, Tomáš - Kroemer, G. - Carracedo, A. - Trka, J. - Starková, J.
Pharmacological inhibition of fatty-acid oxidation synergistically enhances the effect of L-asparaginase in childhood ALL cells
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Leukemia. 2016, roč. 30, 1, p. 209-218
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IF = 12.104
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doi
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Boukalová, Štěpána - Štursa, J. - Werner, L. - Ezrová, Zuzana - Černý, Jiří - Bezawork-Geleta, A. - Pecinová, Alena - Dong, L. - Drahota, Zdeněk - Neužil, Jiří
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Mitochondrial Targeting of Metformin Enhances Its Activity against Pancreatic Cancer
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Molecular Cancer Therapeutics. 2016, roč. 15, 12, p. 2875-2886
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IF = 5.579
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doi
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Česneková, J. - Spáčilová, J. - Hansíková, H. - Houštěk, Josef - Zeman, J. - Stibůrek, L.
LACE1 interacts with p53 and mediates its mitochondrial translocation and apoptosis
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OncoTarget. 2016, roč. 7, 30, p. 47687-47698
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IF = 5.008
[ASEP]
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doi
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Vrbacký, Marek - Kovalčíková, Jana - Chawengsaksophak, Kallayanee - Beck, Inken - Mráček, Tomáš - Nůsková, Hana - Sedmera, David - Papoušek, František - Kolář, František - Sobol, Margaryta - Hozák, Pavel - Sedláček, Radislav - Houštěk, Josef
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Knockout of Tmem70 alters biogenesis of ATP synthase and leads to embryonal lethality in mice
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Human Molecular Genetics. 2016, roč. 25, 21, p. 4674-4685
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IF = 5.985
[ASEP]
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doi
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Tissue cultures - our important model
2D electrophoresis of heart mitochondria
Blue native electrophoresis - important method to study membrane protein complexes
Lab members - summer 2012
