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Mitochondrial protein import in genetically and chemically-derived mitochondrial defectsRungi, Arne. January 2001 (has links)
Thesis (M. Sc.)--York University, 2001. Graduate Programme in Kinesiology and Health Science. / Typescript. Includes bibliographical references (leaves 84-88). Also available on the Internet. MODE OF ACCESS via web browser by entering the following URL: http://wwwlib.umi.com/cr/yorku/fullcit?pMQ66403.
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The role of nuclear-encoded subunit genes in mitochondrial complex 1 deficiency /Worgan, Lisa Catherine. January 2005 (has links)
Thesis (M. Sc.)--University of New South Wales, 2005. / Also available online.
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The structure of mitochondria /Renken, Christian Wolfgang, January 2004 (has links)
Thesis (Ph. D.)--University of California, San Diego, and San Diego State University, 2004. / Vita. Includes bibliographical references (leaves 146-163).
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The Roles of F-box and Leucine-Rich Repeat Protein 4 (FBXL4) in Mitochondrial Encephalopathy and T-cell Acute Lymphoblastic LeukemiaHaydu, Julie Erika M. January 2015 (has links)
The F-box and leucine-rich repeat factor (FBXL4) locus is altered in two distinct diseases, a pediatric mitochondrial encephalopathy associated with early death, and the highly aggressive hematological malignancy T-cell Acute Lymphoblastic Leukemia (T-ALL). As an F-box protein, FBXL4 is predicted to target specific protein substrates for proteasomal degradation. Notably, not much is known about the roles of FBXL4 in homeostasis or disease, and thus I generated conditional Fbxl4 knockout mice to characterize the contributions of Fbxl4 to mitochondrial encephalopathy and to T-ALL. Homozygous mutations in FBXL4 are associated with pediatric-onset mitochondrial encephalopathy, but the molecular and cellular mechanisms driving disease pathogenesis are unknown. Here, I show that constitutive loss of Fbxl4 recapitulates key features of human mitochondrial encephalopathy, including microcephaly, failure to thrive, and perinatal lethality. Moreover, Fbxl4 inactivation drives profound metabolic alterations in the perinatal period. On the cellular level, loss of Fbxl4 results in mitochondria DNA depletion and disrupts oxidative phosphorylation and mitochondria membrane potential. Isolation of the FBXL4 protein complex reveals that FBXL4 interacts with a diverse set of mitochondrial factors crucial for normal mitochondrial function. Overall, these findings underscore the importance of FBXL4 in development, metabolism, and mitochondrial dynamics, and may be used to develop novel therapies for patients with mitochondrial encephalopathy associated with FBXL4 mutations and for patients with 6q- T-ALL.
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Investigations into pathophysiologic mechanisms and treatment of primary mitochondrial diseasesSiegmund, Stephanie January 2018 (has links)
The present work addresses outstanding questions within the field of primary mitochondrial disease biology and treatment, by incorporating methods from structural biology, molecular biology, and animal studies. First, we utilize a mouse model of mitochondrial deoxyribose nucleic acid (mtDNA) disease to demonstrate the potential therapeutic benefit of low-dose chronic rapamycin treatment. Interestingly, rapamycin therapy significantly extends survival, but does so in the absence of correcting the underlying mitochondrial defect. Next, we focus on human cellular models of mtDNA-based diseases, and show that rapamycin treatment does not induce mitochondrial quality control-mediated clearance of pathogenic mtDNA mutation-harboring organelles. Finally, we investigate a mitochondrial disease phenotype at the level of the organelle, by utilizing in situ cryo-electron tomography to demonstrate the ultrastructural consequences of a pathogenic mutation affecting mitochondrial energy production. We conclude by highlighting the insights into disease biology and treatment that can be gained through a multi-level approach integrating techniques from multiple biomedical fields.
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Mitochondrial response to hypoxia and assessment of sub-cellular directed DNA repair on mitigating the effects of ROS induced DNA damage /Bowen, Lance Daniel. January 2006 (has links)
Thesis (Ph. D.)--University of Nevada, Reno, 2006. / "December 2006." Includes bibliographical references. Online version available on the World Wide Web. Library also has microfilm. Ann Arbor, Mich. : ProQuest Information and Learning Company, [2006]. 1 microfilm reel ; 35 mm.
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The role of mitochondria in regulating MAPK signalling pathways during oxidative stress /Pang, Wei Wei. January 2006 (has links)
Thesis (Ph.D.)--University of Western Australia, 2006.
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Pten-induced kinase 1 (PINK1) and its role in mitochondrial function and dynamicsThomas, Kelly Jean. January 2008 (has links)
Thesis (Ph.D.)--Georgetown University, 2008. / Includes bibliographical references.
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The role of L-carnitine in preventing mitochondrial dysfunction after neonatal hypoxia-ischemiaRau, Thomas Fredrick. January 1900 (has links)
Thesis (Ph. D.)--University of Montana, 2007. / Title from title screen. Description based on contents viewed Aug. 20, 2007. Includes bibliographical references (p. 116-120).
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Role of phenylalanyl-tRNA synthetase in translation quality controlLing, Jiqiang, January 2008 (has links)
Thesis (Ph. D.)--Ohio State University, 2008. / Title from first page of PDF file. Includes vita. Includes bibliographical references (p. 119-137).
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