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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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Examining the significance of peptides regulating the intake of food and the nutritional state of children and adolescents
Bronský, Jiří ; Průša, Richard (advisor) ; Pelouch, Václav (referee) ; Hyánek, Josef (referee)
Introduction: Regulation of food intake and nutritional status is mediated by complex interactions of regulatory peptides of the central nervous system, gastrointestinal tract and adipose tissue. These systems are connected by feedback loops which inform the centre about amount of ingested food and energy reserves in the organism. Dysfunction of any of these regulatory areas may lead to changes in nutritional status of the organism. Methods: We used radioimmunoassay to measure plasma levels of orexin A, total ghrelin and serum levels of leptin and enzyme immunoassay to measure serum levels of adiponectin in healthy subjects and in children with obesity, anorexia nervosa, Crohn's disease and celiac disease and we evaluated the influence of nutritional therapy on these levels. Moreover, we evaluated relationship of these regulatory peptides to other biochemical and anthropometrical factors of nutritional status. We also measured plasma levels of total and unreduced amylin by enzyme immunoassay with immunofluorescence detection in adult patients with osteoporosis, type II diabetes mellitus and in the control group. Results: During reduction of body weight in obese children and adolescents, there were statistically significant changes of plasma orexin A levels and total ghrelin levels, but we haven't seen any...
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