guest ::
login
Digital Repository
Search
Submit
Help
About
Home
> Search Results: keywords:"F1FO-ATPsyntáza"
Search:
any field
title
authors
keywords
publication date
Institution
document type
conference title
ISBN/ISSN
project number
fulltext
record ID
Search Tips
::
Advanced Search
Search collections:
*** any public collection ***
Agritec
Agrotest fyto, s.r.o.
Agrovýzkum Rapotin Ltd.
Academy of Performing Arts in ...
Academy of Fine Arts in Prague
Analytical and methodological ...
Analysis
Institute of Archaeology, Brno
Institute of Archaeology, Prag...
Architectural Institute in Pra...
Archiv ing. arch. Jana Moučky
Arnika
Czech Water Association
Astronomical Institute
Author works
Bachelor's theses
Banks
Personal bibliographies
Institute of Biophysics
Biology Centre
Institute of Biotechnology
Institute of Botany
Brochures
Newsletters
CENIA
Transport Research Centre
Centre for Transport and Energ...
The Centre for Regional Develo...
Centre for Higher Education St...
Global Change Research Institu...
Czech Association of Occupatio...
Czech Paraplegic Association
The Czech National Bank
Czech Council of Children and ...
Czech Society for Ornithology
Czech University of Life Scien...
Czech Technical University in ...
Czech Statistical Office
CESNET
Travel reports
Hop Research Institute
People in Need Czech Republic
data
National Repository of Grey Li...
Master’s theses
Doctoral theses
doc. RNDr. Jiří Souček, DrSc.
Ekodomov
Institute of Entomology
Institute of Ethnology
European Values o.s.
Fairtrade Czech Republic & Slo...
Charles University Faculties (...
Institute of Pharmacology
Institute of Philosophy
Trade literature
Trade print
Institute of Physics
Institute of Physiology
Galleries
The Gallery of Graphic Arts in...
Gender Studies, o.p.s.
Institute of Geophysics
Department of Geography, Masar...
Institute of Geology
GLE o.p.s.
Grant reports
Habilitation theses
HESTIA
Institute of History
Institute of Hydrobiology
The Arts and Theatre Institute
Iuridicum Remedium
University of South Bohemia in...
South Moravian Museum in Znojm...
Product catalogues
Exhibition catalogues
Department of Social Geography...
Library of the ASCR
Libraries
Conference materials
Culture
Flyers
Literary Academy (Internationa...
The Map Collection at Charles ...
Masaryk University
Masaryk Institute and Archives...
Institute of Mathematics
Mendel University in Brno
Methods
Institute of Microbiology
Ministries
Ministry of Defence
Ministry of Justice
Ministry of the Environment
Monographs
The Moravian Gallery in Brno
Moravian Library
Museums
Museum of the Brno Region
Museum of Glass and Jewellery ...
Museum of Eastern Bohemia in H...
Karel Komárek Proměny Foundati...
National archives
National Film Archive
The National Stud at Kladruby ...
National Information and Co...
National Library of the Czech ...
National Medical Library
National Museum
National Heritage Institute
National Library of Technology
National technical museum
National Institute of Folk Cul...
National Institute for Educati...
The National Museum of Agricul...
National Open-Air Museum
Economics Institute
Nonprofit organizations
Oriental Institute
OSEVA Development and Research...
Personal archives
Miscellaneous
University of Ostrava
The Museum of Czech Literature
Institute of Parasitology
Parliamentary Institute
Biographies
Portfólia
Posters
Postprints
Prague College
Preprints
Papers
Programmes
Promotional and educational ma...
Progress reports
Exhibition guides
Institute of Psychology
Reviews
Rigorous theses
Proceedings
Association for Integration an...
The North Bohemian Museum in L...
SIRIRI
Universities and colleges
Silesian University in Opava
The Silesian Museum
Institute of Slavonic Studies
Institute of Sociology
Institute of Sociology (CVVM)
Private colleges
Specialized departments
Czech Radioactive Waste Reposi...
Statistical reports
Administration
State Office for Nuclear Safet...
Czech Agriculture and Food Ins...
Studies
Technical Museum in Brno
Technical reports
Technology Centre
Thematic collections
Press releases
The Museum of Decorative Arts ...
University of Hradec Králové
Jan Evangelista Purkyně Univer...
Charles University
Palacký University Olomouc
University of Pardubice
Industrial Property Office of ...
Institute of Analytical Chemis...
Institute of Inorganic Chemist...
Institute of Archeological and...
Institute of Vertebrate Biolog...
Institute of Chemical Process ...
Institute of Art History
Institute of Experimental Bota...
Institute of Experimental Medi...
Institute of Photonics and Ele...
J. Heyrovsky Institute of Phys...
Institute of Physical Engineer...
Institute of Atmospheric Physi...
Institute of Physics of Materi...
Institute of Plasma Physics
Institute of Geonics
Institute of Computer Science
Nuclear Physics Institute
Institute of Macromolecular Ch...
Institute of Plant Molecular B...
Institute of Molecular Genetic...
Institute of Organic Chemistry...
Institute of Scientific Instru...
Institute of Czech Literature
Institute of Art History, Char...
Institute of Electrical Engine...
Institute of Hydrodynamics
Institute of the Czech Languag...
Institute for Classical Studie...
Institute of Contemporary Hist...
The Institute for the Study of...
Institute of Soil Biology
Institute of Slavonic-German S...
Institute of State and Law
Institute of Rock Structure an...
Institute of Theoretical and A...
Institute of Information Theor...
Institute of Thermomechanics
Institute of Animal Physiology...
Institutes ASCR
Research
Public universities
Gazettes
Research Institute of Geodesy,...
Scientific research institutio...
Annual reports
University of Chemistry and Te...
University of Economics, Pragu...
The College of European and Re...
The University of Finance and ...
College of Information Managem...
Universities
Brno University of Technology
Academic theses (ETDs)
Research institutes
Research reports
Timber Research and Developmen...
Occupational Safety Research I...
Forestry and Game Management R...
Food Research Institute Prague
Research Institute for Labour ...
Crop Research Institute
The Silva Tarouca Research Ins...
T. G. Masaryk Water Research I...
Woodexpert
University of West Bohemia
Westbohemian Museum in Pilsen
Final reports
Biographies
Reports
Status reports
Survey reports
Sort by:
Display results:
Output format:
latest first
publication date
author
record ID
asc.
desc.
- or rank by -
word similarity
10 results
25 results
50 results
100 results
single list
split by collection
citace
HTML brief
HTML brief + PSH
HTML citesummary
MARC
Refman RIS
National Repository of Grey Literature
3
records found
Search took 0.01 seconds.
Human F1Fo-ATPsynthase deficiency
Suldovská, Sabina
;
Tesařová, Markéta
(advisor) ;
Černá, Leona
(referee)
F1FO-ATPsynthase is a key enzyme in energy metabolism of the cell. Its deficit is caused usually by mutations in two structural genes MT-ATP6 and MT-ATP8 encoded by the mitochondrial DNA or in nuclear genes ATPAF2 and TMEM70 encoding the biogenesis factors and structural gene ATP5E. Deficiency of the F1FO-ATPsynthase leads to progressive and serious phenotype affecting organs with high energy demands. The first symptoms usually occurs in neonatal age and prognosis of the disease is fatal. Mutations in these genes result in both qualitative and quantitative defects of the F1FO-ATPsynthase. The study of molecular bases of mitochondrial disorders including F1FO-ATPsynthase deficiency uses large number of biochemical and molecular-genetic methods to determine a proper diagnosis which is essential for the symptomatic therapy and genetic counselling in affected families. The aim of the diploma thesis was to characterise the F1FO-ATPsynthase deficiency in isolated mitochondria from the lines of cultured cells by the determination oligomycin- sensitive ATP-hydrolytic activity of the F1FO-ATPsynthase, enzymatic activities of the respiratory chain complexes and to analyse changes in the steady-state levels of the representative subunits and whole complex of the F1FO-ATPsynthase in comparison with controls. 3...
Detailed record
Impact of isolate deficiency of F1FO-ATP syntthase on other complexes of oxidative phosphorylation in skin fibroblasts depending on cullture conditions
Kedrová, Kateřina
;
Stiborová, Marie
(advisor) ;
Poljaková, Jitka
(referee)
Isolated deficiency of F1FO-ATPsynthase is a soubgroup of mitochondrial diseases caused by mutations in nuclear and mitochondrial-encoded structural subunits, or nuclear-encoded assembly factors of F1FO-ATPsynthase. The most often mutations are found in a MTATP6 gene localized in the mitochondrial DNA and a TMEM70 gene, localized in the nuclear DNA. A MTATP6 gene encodes subunit a of F1FO-ATPsynthase and its mutation usually leads to reduced phosphorylation activity of F1FO-ATPsynthase. A TMEM70 gene encodes a 21 kDa mitochondrial protein of the inner mitochondrial membrane of not completely explained function and its mutation results in the decrease in a content of fully assembled F1FO- ATPsynthase. The aim of this thesis was to investigate the impact of isolated F1FO- ATPsynthase deficiency on the oxidative phosphorylation system (complex I-IV), other selected mitochondrial proteins, and mitochondrial network in two cell lines of primary human skin fibroblasts with an isolated deficiency of F1FO-ATPsynthase (mutation m.8851T>C in MTATP6 and mutation c.317-2A>G in TMEM70) during the first days of their cultivation in media containing galactose or glucose as a carbohydrate source with a presence or absence of L-glutamine. The control cell line was found to have higher amounts of respiratory chain...
Detailed record
Human F1Fo-ATPsynthase deficiency
Suldovská, Sabina
;
Tesařová, Markéta
(advisor) ;
Černá, Leona
(referee)
F1FO-ATPsynthase is a key enzyme in energy metabolism of the cell. Its deficit is caused usually by mutations in two structural genes MT-ATP6 and MT-ATP8 encoded by the mitochondrial DNA or in nuclear genes ATPAF2 and TMEM70 encoding the biogenesis factors and structural gene ATP5E. Deficiency of the F1FO-ATPsynthase leads to progressive and serious phenotype affecting organs with high energy demands. The first symptoms usually occurs in neonatal age and prognosis of the disease is fatal. Mutations in these genes result in both qualitative and quantitative defects of the F1FO-ATPsynthase. The study of molecular bases of mitochondrial disorders including F1FO-ATPsynthase deficiency uses large number of biochemical and molecular-genetic methods to determine a proper diagnosis which is essential for the symptomatic therapy and genetic counselling in affected families. The aim of the diploma thesis was to characterise the F1FO-ATPsynthase deficiency in isolated mitochondria from the lines of cultured cells by the determination oligomycin- sensitive ATP-hydrolytic activity of the F1FO-ATPsynthase, enzymatic activities of the respiratory chain complexes and to analyse changes in the steady-state levels of the representative subunits and whole complex of the F1FO-ATPsynthase in comparison with controls. 3...
Detailed record
Interested in being notified about new results for this query?
Subscribe to the
RSS feed
.