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INSTITUTE OF PHYSIOLOGY CAS

Cutting-edge science for health

Laboratory of Bioenergetics

Laboratory of 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.

 

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

Tibenská; V. - Benešová; A. - Vebr; P. - Liptáková; A. - Hejnová; L. - Elsnicová; B. - Drahota; Zdeněk - Horníková; D. - Galatík; F. - Kolář; D. - Vybíral; S. - Alánová; Petra - Novotný; J. - Kolář; František - Nováková; Olga - Žurmanová; J.M. Gradual cold acclimation induces cardioprotection without affecting adrenergic beta-receptor-mediated adenylyl cyclase signaling . Journal of Applied Physiology. 2020; 128(4); 1023-1032 . IF = 3.044 [ASEP] [ doi ]
Pajuelo-Reguera; David - Čunátová; Kristýna - Vrbacký; Marek - Pecinová; Alena - Houštěk; Josef - Mráček; Tomáš - Pecina; Petr . Cytochrome c Oxidase Subunit 4 Isoform Exchange Results in Modulation of Oxygen Affinity . Cells. 2020; 9(2)); 443 . IF = 4.366 [ASEP] [ doi ]
Nůsková; Hana - Mikešová; Jana - Efimova; Iuliia - Pecinová; Alena - Pecina; Petr - Drahota; Zdeněk - Houštěk; Josef - Mráček; Tomáš . Biochemical thresholds for pathological presentation of ATP synthase deficiencies . Biochemical and Biophysical Research Communications. 2020; 521(4); 1036-1041 . IF = 2.985 [ASEP] [ doi ]
Kubánek; M. - Schimerová; T. - Piherová; L. - Brodehl; A. - Krebsová; A. - Ratnavadivel; S. - Stanasiuk; C. - Hansíková; H. - Zeman; J. - Paleček; T. - Houštěk; Josef - Drahota; Zdeněk - Nůsková; Hana - Mikešová; Jana - Zámečník; J. - Macek Jr.; M. - Ridzoň; P. - Malusková; J. - Stránecký; V. - Melenovský; V. - Milting; H. - Kmoch; S. Desminopathy: Novel Desmin Variants; a New Cardiac Phenotype; and Further Evidence for Secondary Mitochondrial Dysfunction . Journal of Clinical Medicine. 2020; 9(4)); 937 . IF = 3.303 [ASEP] [ doi ]
Hložková; K. - Pecinová; Alena - Alquezar-Artieda; N. - Pajuelo-Reguera; D. - Šimčíková; M. - Hovorková; L. - Rejlová; K. - Žaliová; M. - Mráček; Tomáš - Kolenová; A. - Starý; J. - Trka; J. - Stárková; J. Metabolic profile of leukemia cells influences treatment efficacy of L-asparaginase . Bmc Cancer. 2020; 20(1)); 526 . IF = 3.150 [ASEP] [ doi ]

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People

RNDr. Tomáš Mráček, Ph.D.

Head of the Laboratory

MUDr. Josef Houštěk, DrSc.

Deputy Head of the Laboratory

RNDr. Zdeněk Drahota, DrSc. 

Senior Researcher (emeritus)

RNDr. Alena Pecinová, Ph.D.
Senior Researcher

Mgr. Petr Pecina, Ph.D.

Senior Researcher

RNDr. Marek Vrbacký, Ph.D.
Senior Researcher
Mgr. Vilma Kaplanová, Ph.D.
Senior Researcher

Mgr. Kateřina Tauchmannová, Ph.D. 

Postdoctoral Fellow

Mgr. Lukáš Alán, Ph.D.

Postdoctoral Fellow

Ing. Mgr. Eliška Koňaříková

PhD Student

Mgr. Kristýna Čunátová

PhD Student

Mgr. Zuzana Korandová

PhD Student

Aleksandra Marković MSc.

PhD Student

Guillermo Puertas Frias MSc.

PhD Student

Jan Eliáš

Undergraduate Student

Vladimíra Brožková

Technician