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
Using lymphocytes isolated from peripheral blood, we try to develop new diagnostic protocols for patients with suspected mitochondrial diseases.
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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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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
Animal models of human mitochondrial diseases enable detailed insight into pathogenic mechanisms, from molecular to organismal levels.
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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.
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Fourth year of the scienticic photography contest means success for our young guns.
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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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Publications
Marković; Aleksandra - Tauchmannová; Kateřina - Šimáková; Miroslava - Mlejnek; Petr - Kaplanová; Vilma - Pecina; Petr - Pecinová; Alena - Papoušek; František - Liška; František - Šilhavý; Jan - Mikešová; Jana - Neckář; Jan - Houštěk; Josef - Pravenec; Michal - Mráček; Tomáš
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Genetic Complementation of ATP Synthase Deficiency Due to Dysfunction of TMEM70 Assembly Factor in Rat
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Biomedicines. 2022; 10(2)); 276
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IF = 6.081
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Pravenec; Michal - Šilhavý; Jan - Mlejnek; Petr - Šimáková; Miroslava - Mráček; Tomáš - Pecinová; Alena - Tauchmannová; Kateřina - Hüttl; M. - Malínská; H. - Kazdová; L. - Neckář; Jan - Kolář; František - Žurmanová; J. - Novotný; J. - Houštěk; Josef
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Conplastic strains for identification of retrograde effects of mitochondrial dna variation on cardiometabolic traits in the spontaneously hypertensive rat
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Physiological Research. 2021; 70(Suppl.4); S471-S481
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IF = 1.881
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doi
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Oláhová; M. - Peter; B. - Szilagyi; Z. - Diaz-Maldonado; H. - Singh; M. - Sommerville; E. W. - Blakely; E. L. - Collier; J. J. - Hoberg; E. - Stránecký; V. - Hartmannová; H. - Bleyer; A. J. - McBride; K. L. - Bowden; S. A. - Korandová; Zuzana - Pecinová; Alena - Ropers; H.-H. - Kahrizi; K. - Najmabadi; H. - Tarnopolsky; M. A. - Brady; L. I. - Weaver; K. N. - Prada; C. E. - Ounap; K. - Wojcik; M. H. - Pajusalu; S. - Syeda; S. B. - Pais; L. - Estrella; E. A. - Bruels; Ch. C. - Kunkel; L. M. - Kang; P. B. - Bonnen; P. E. - Mráček; Tomáš - Kmoch; S. - Gorman; G. S. - Falkenberg; M. - Gustafsson; C. M. - Taylor; R. W.
POLRMT mutations impair mitochondrial transcription causing neurological disease
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Nature Communications. 2021; 12(1)); 1135
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IF = 14.919
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doi
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Folbergrová; Jaroslava - Ješina; Pavel - Otáhal; Jakub
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Treatment With Resveratrol Ameliorates Mitochondrial Dysfunction During the Acute Phase of Status Epilepticus in Immature Rats
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Frontiers in Neuroscience. 2021; 15(Mar 5)); 634378
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IF = 4.667
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doi
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Endlicher; R. - Drahota; Zdeněk - Kučera; O. - Červinková; Z.
Age-Dependent Changes in the Function of Mitochondrial Membrane Permeability Transition Pore in Rat Liver Mitochondria
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Physiological Research. 2021; 70(6); 905-911
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IF = 1.881
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doi
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Group photo - 2019
Blue native electrophoresis - important method to study membrane protein complexes
2D electrophoresis of heart mitochondria
Tissue cultures - our important model
