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Showing 1 to 5 of 5 for “"Friedreich's ataxia"”.
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A Protective Role Of Autophagy In A Drosophila Model Of Friedreich's Ataxia (frda)
<p>Friedreich’s ataxia (FRDA) is an inherited autosomal recessive neurodegenerative disease. It affects 1 in every 50,000 people in central Europe and North America. FRDA is caused by deficiency of Frataxin, an essential mitochondrial iron chaperone protein, and the associated oxidative stress …
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EXPLORING MITOCHONDRIAL AND REDOX VULNERABILITY IN FRIEDREICH'S ATAXIA USING GOLD NANOCLUSTERS IN HUMAN CELLULAR MODELS
Friedreich’s ataxia (FRDA) is a rare autosomal recessive neurodegenerative disorder caused by transcriptional silencing of the FXN gene, leading to frataxin deficiency, impaired iron–sulfur cluster biogenesis, mitochondrial dysfunction, and chronic oxidative stress. Despite significant advances in …
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Molecular Details Of The Mitochondrial Iron Sulfur Cluster Assembly Pathway
… this pathway results in several diseases such as Friedreich's Ataxia (FRDA), Sideroblastic Anemia and ISCU Myopathy. Therefore molecular details of the biogenesis pathway will provide deep insight in the pathway and treatment options for these diseases. FRDA is caused by deficiency of a single …
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Characterization Of Initial Iron Binding Location And The Structure/iron Binding Site On S.cerevisiae Isu And On D.melanogaster Frataxin
… pathology of diseases of iron overload such as Friedreich's Ataxia, a genetic disorder characterized by an accumulation of iron in actively metabolizing tissues ultimately leading to cardio- and neuro- degeneration and cell death. It is caused by an inability to synthesize the mitochondrial …
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Novel Diagnostic Approaches for Genetic and Environmental Sources of Mitochondrial Dysfunction
… molecular targets linked with Friedrich's Ataxia, an inherited metabolic disorder, through conducting functional in-vitro studies using human-derived cell samples, as well as developing inventive animal models created via Xenopus laevis tadpoles to evaluate the effects of environmental …