Intranet Čeština

Bioenergetics

Bioenergetics

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

Functional characterization of ATP synthase deficiencies

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). More

The role of mitochondria in heart failure

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. More

Bioenergetics of rare diseases

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. More

Achievements

TMEM70 disease

Animal models of human mitochondrial diseases enable detailed insight into pathogenic mechanisms, from molecular to organismal levels. More

Mitochondria dysfunction and Fanconi syndrome

Inborn disorders of energy provision by mitochondrial respiratory chain are the primary cause of numerous serious diseases, ranging from most severe encephalo-cardio-myopathies manifesting early after birth to various tissues-specific and milder disorders affecting mainly adults. More

Our new publication: High resistance to mitochondrial mutations

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.  More

Publications

Neckář, Jan - Svatoňová, Anna - Weissová, Romana - Drahota, Zdeněk - Zajíčková, Pavlína - Brabcová, I. - Kolář, D. - Alánová, Petra - Vašinová, Jana - Šilhavý, Jan - Hlaváčková, Markéta - Tauchmannová, Kateřina - Milerová, Marie - Ošťádal, Bohuslav - Červenka, L. - Žurmanová, J. - Kalous, M. - Nováková, Olga - Novotný, J. - Pravenec, Michal - Kolář, František . Selective replacement of mitochondrial DNA increases the cardioprotective effect of chronic continuous hypoxia in spontaneously hypertensive rats . Clinical science. 2017, roč. 131, 9, p. 865-881 . IF = 4.936 [ASEP] [ doi ]
Melenovský, V. - Petrák, J. - Mráček, Tomáš - Beneš, J. - Borlaug, B. A. - Nůsková, Hana - Pluháček, T. - Špatenka, J. - Kovalčíková, Jana - Drahota, Zdeněk - Kautzner, J. - Pirk, J. - Houštěk, Josef . Myocardial iron content and mitochondrial function in human heart failure: a direct tissue analysis . European Journal of Heart Failure. 2017, roč. 19, 4, p. 522-530 . IF = 6.968 [ASEP] [ doi ]
Endlicher, R. - Drahota, Zdeněk - Červinková, Z. In vitro and in vivo activation of mitochondrial membrane permeability transition pore using triiodothyronine . Physiological Research. 2016, roč. 65, 2, p. 321-331 . IF = 1.461 [ASEP]
Pravenec, Michal - Mlejnek, Petr - Zídek, Václav - Landa, Vladimír - Šimáková, Miroslava - Šilhavý, Jan - Strnad, Hynek - Eigner, Sebastian - Eigner-Henke, Kateřina - Škop, V. - Malínská, H. - Trnovská, J. - Kazdová, L. - Drahota, Zdeněk - Mráček, Tomáš - Houštěk, Josef . Autocrine effects of transgenic resistin reduce palmitate and glucose oxidation in brown adipose tissue . Physiological Genomics. 2016, roč. 48, 6, p. 420-427 . IF = 3.044 [ASEP] [ doi ]
Česneková, J. - Spáčilová, J. - Hansíková, H. - Houštěk, Josef - Zeman, J. - Stibůrek, L. LACE1 interacts with p53 and mediates its mitochondrial translocation and apoptosis . OncoTarget. 2016, roč. 7, 30, p. 47687-47698 . IF = 5.178 [ASEP] [ doi ]
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 . Nature Communications. 2016, roč. 7, Jul, p. 12061 . IF = 12.124 [ASEP] [ doi ]
Boukalová, Štěpána - Štursa, J. - Werner, L. - Ezrová, Zuzana - Černý, Jiří - Bezawork-Geleta, A. - Pecinová, Alena - Dong, L. - Drahota, Zdeněk - Neužil, Jiří . Mitochondrial Targeting of Metformin Enhances Its Activity against Pancreatic Cancer . Molecular Cancer Therapeutics. 2016, roč. 15, 12, p. 2875-2886 . IF = 5.764 [ASEP] [ doi ]
Milerová, Marie - Drahota, Zdeněk - Chytilová, Anna - Tauchmannová, Kateřina - Houštěk, Josef - Ošťádal, Bohuslav . Sex difference in the sensitivity of cardiac mitochondrial permeability transition pore to calcium load . Molecular and Cellular Biochemistry. 2016, roč. 412, 1-2, p. 147-154 . IF = 2.669 [ASEP] [ doi ]
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 . Leukemia. 2016, roč. 30, 1, p. 209-218 . IF = 11.702 [ASEP] [ doi ]
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 . Journal of Medical Genetics. 2016, roč. 53, 11, p. 768-775 . IF = 5.451 [ASEP] [ doi ]

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Contacts

Institute of Physiology AS CR, v.v.i.
Department of bioenergetics
Vídeňská 1083
14220 Prague 4
Czech Republic
tel. +420 241 063 727
fax. +420 241 062 149
   

tomas.mracek@fgu.cas.cz

People

RNDr. Tomáš Mráček, Ph.D.
head of the department
MUDr. Josef Houštěk, DrSc.
deputy head of the department
RNDr. Zdeněk Drahota, DrSc. 
senior researcher (emeritus)
Mgr. Petr Pecina, Ph.D.
senior researcher
Mgr. Vilma Kaplanová, Ph.D.
senior researcher
RNDr. Alena Pecinová, Ph.D.
senior researcher
RNDr. Marek Vrbacký, Ph.D.
senior researcher

Mgr. Kateřina Tauchmannová, Ph.D.

(née Hejzlarová)
postdoc

Mgr. Lukáš Alán, Ph.D.

postdoc

David Pajuelo Reguera, Ph.D.

postdoc

Ing. Andrea Brázdová, Ph.D.

postdoc

Mgr. Jana Kovalčíková
PhD student
Mgr. Michal Zima
PhD student
BSc. Hien Ho Dieu
master student

Kristýna Čunátová

undergraduate student

Julia Effimova

undergraduate student

Vladimíra Brožková
lab technician

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