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Study of mitochondrial ultrastructure and functions in selected mitochondrial and lysosomal storage disorders
Kostková, Olga ; Hansíková, Hana (advisor) ; Šmíd, František (referee) ; Hyánek, Josef (referee)
This thesis has been worked out in The laboratory for study of mitochondrial disorders (Departement of Pediatrics, 1st Faculty of Medcine, Chales university in Prague) and in cooperation with The Institute of Inherited Metabolic Disorders. Mitochondrial disorders represent a heterogeneous group of diseases with the onset at any age from neonatal period till adulthood, mostly presented with very severe clinical courses of disease. The mammalian organism is fully dependent on mitochondrial oxidative phosphorylation system as on the major energy producer of the cell. Therefore the mitochondrial disorders affect mainly high energy demanded tissues such as brain, heart or muscle. Simillar phenotype is observed in many lysosomal storage disorders. Despite of expanding knowledge of molecular basis of mitochondrial and lysosomal disorders, it may be still difficult to explain the exact pathogenesis of disease as well as the prognosis for patients and their families. Mitochondrial functions affect more than just energy production; they contribute in initiation of apoptosis, in cellular calcium homeostasis, and in production of reactive oxygene species. Disturbed mitochondria become a goal of autophagy mediated by the lysosomal compartement. The results of our study enable: 1. better understanding of the tissue...
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Study of mitochondrial ultrastructure and functions in selected mitochondrial and lysosomal storage disorders
Kostková, Olga
Charles University, Prague 1st Faculty of Medicine PhD thesis summary Study of mitochondrial ultrastructure and functions in selected mitochondrial and lysosomal storage disorders Mgr. Olga Kostková Prague 2009 Postgraduate studies in Biomedicine Charles University in Prague and Academy of Sciences of the Czech Republic Section: Biochemistry and pathobiochemisty Section chairman: Prof. MUDr. Jiří Kraml, DrSc. Workplace: Laboratory for study of mitochondrial disorders Department of Pediatrics 1st Faculty of Medicine, Charles University Ke Karlovu 2, 128 08 Praha 2 Author: Mgr. Olga Kostková Supervisor: RNDr. Hana Hansíková, CSc. Supervisor consultant: RNDr. Jana Sládková, CSc. Opponents: …………………………………… …………………………………… …………………………………… Abstract of PhD thesis was send: ................... Defense of PhD thesis: The full text of the thesis is available at the 1st Faculty of Medicine, Charles University in Prague. This PhD thesis was elaborated during Postgraduate Studies in Biomedicine at the 1st Faculty of Medicine, Charles University in Prague within years 2004-2009 and was supported by IGA MZ NR 8065-3; GACR 303/03/H065; the Center for Applied Genomics 1M6837805002; research project MSM0021620806 and HUEMAN LSHM-CT-2006-018692 SUMMARY 6 1. INTRODUCTION 8 1.1 Mitochondria 8 1.2 Mitochondrial structure 9 1.3...
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Study of mitochondrial ultrastructure and functions in selected mitochondrial and lysosomal storage disorders
Kostková, Olga
Charles University, Prague 1st Faculty of Medicine PhD thesis summary Study of mitochondrial ultrastructure and functions in selected mitochondrial and lysosomal storage disorders Mgr. Olga Kostková Prague 2009 Postgraduate studies in Biomedicine Charles University in Prague and Academy of Sciences of the Czech Republic Section: Biochemistry and pathobiochemisty Section chairman: Prof. MUDr. Jiří Kraml, DrSc. Workplace: Laboratory for study of mitochondrial disorders Department of Pediatrics 1st Faculty of Medicine, Charles University Ke Karlovu 2, 128 08 Praha 2 Author: Mgr. Olga Kostková Supervisor: RNDr. Hana Hansíková, CSc. Supervisor consultant: RNDr. Jana Sládková, CSc. Opponents: …………………………………… …………………………………… …………………………………… Abstract of PhD thesis was send: ................... Defense of PhD thesis: The full text of the thesis is available at the 1st Faculty of Medicine, Charles University in Prague. This PhD thesis was elaborated during Postgraduate Studies in Biomedicine at the 1st Faculty of Medicine, Charles University in Prague within years 2004-2009 and was supported by IGA MZ NR 8065-3; GACR 303/03/H065; the Center for Applied Genomics 1M6837805002; research project MSM0021620806 and HUEMAN LSHM-CT-2006-018692 SUMMARY 6 1. INTRODUCTION 8 1.1 Mitochondria 8 1.2 Mitochondrial structure 9 1.3...
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Study of mitochondrial ultrastructure and functions in selected mitochondrial and lysosomal storage disorders
Kostková, Olga ; Hansíková, Hana (advisor) ; Šmíd, František (referee) ; Hyánek, Josef (referee)
This thesis has been worked out in The laboratory for study of mitochondrial disorders (Departement of Pediatrics, 1st Faculty of Medcine, Chales university in Prague) and in cooperation with The Institute of Inherited Metabolic Disorders. Mitochondrial disorders represent a heterogeneous group of diseases with the onset at any age from neonatal period till adulthood, mostly presented with very severe clinical courses of disease. The mammalian organism is fully dependent on mitochondrial oxidative phosphorylation system as on the major energy producer of the cell. Therefore the mitochondrial disorders affect mainly high energy demanded tissues such as brain, heart or muscle. Simillar phenotype is observed in many lysosomal storage disorders. Despite of expanding knowledge of molecular basis of mitochondrial and lysosomal disorders, it may be still difficult to explain the exact pathogenesis of disease as well as the prognosis for patients and their families. Mitochondrial functions affect more than just energy production; they contribute in initiation of apoptosis, in cellular calcium homeostasis, and in production of reactive oxygene species. Disturbed mitochondria become a goal of autophagy mediated by the lysosomal compartement. The results of our study enable: 1. better understanding of the tissue...
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