Global ETD Search
Search theses and dissertations gathered from participating repositories worldwide. Every result links back to the library that holds it. No account is needed.
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Showing 1 to 20 of 221 for “"Mitochondrial Dysfunction"”.
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Mitochondrial dysfunction in hereditary optic neuropathies
MITOCHONDRIAL DYSFUNCTION IN HEREDITARY OPTIC NEUROPATHIES Mitochondrial pathologies are a heterogeneous group of clinical manifestations characterized by oxidative phosphorylation impairment. At the beginning of their recognition mitochondrial pathologies were regarded as rare disorders but indeed …
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The Role of ECSIT in Mitochondrial Dysfunction Mediated Cardiomyopathy
… embryonic development. It also functions as a mitochondrial complex I assembly factor and loss of function leads to a reduction in fully assembled complex I. As part of a large scale ENU mutagenesis screen, The Harwell Ageing Screen, a novel missense mutation (N209I) was identified in ECSIT …
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Mitochondrial dysfunction in a cell model of thyroid oncocytoma
The role of mitochondrial dysfunction in cancer has long been a subject of great interest. In this study, such dysfunction has been examined with regards to thyroid oncocytoma, a rare form of cancer, accounting for less than 5% of all thyroid cancers. A peculiar characteristic of thyroid oncocytic …
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Proteomic analysis of cellular models of neurodegeneration and mitochondrial dysfunction
Mitochondrial dysfunction is thought to contribute to neurodegenerative processes. As an example, dysfunction of complex I of the electron transport chain has been observed in Parkinson’s disease patients and 1-methyl-4-phenyl-1,2,3,6 tetrahydropyridine (MPTP), a complex I inhibitor, produces a …
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Mitochondrial Dysfunction and Stress Responses in CHCHD10 Myopathy and Neurodegeneration
In the last decade, dominant mutations in the mitochondrial protein CHCHD10 and its paralogue CHCHD2 were shown to cause familial amyotrophic lateral sclerosis and Parkinson’s disease, respectively, with phenotypes that often resemble the idiopathic forms of the diseases. Different mutations in …
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Mitochondrial dysfunction during ischaemia reperfusion injury in models of organ transplantation
… pathways activated by factors released upon mitochondrial damage, such as succinate or mtDNA. In order to understand these processes better and be able to develop novel therapies, I utilised a range of models for IRI in heart, liver and kidney organ transplantation. These ranged from basic …
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Autism Spectrum Disorder and Mitochondrial Dysfunction: The Role of Mitochondrial Dynamics
… disorder (ASD). Our research group reported that mitochondrial dysfunction was associated with ASD in South African children diagnosed with ASD using differential methylation and metabolomics studies. Propionyl-CoA Carboxylase Subunit Beta (PCCB) was differentially methylated in our cohort ASD …
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Novel Diagnostic Approaches for Genetic and Environmental Sources of Mitochondrial Dysfunction
… that the primary cause of these diseases is mitochondrial dysfunction in the affected patients. While genetics plays a role in these conditions, lifestyle choices and exposure to toxins also significantly contribute to their development. Unfortunately, early-stage diagnosis can be difficult …
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The role of ischaemia-reperfusion injury and mitochondrial dysfunction in organ transplantation
… The role of ischaemia-reperfusion injury and mitochondrial dysfunction in organ transplantation Ischaemia and subsequent reperfusion is inherent to solid organ transplantation and contributes to tissue damage, organ dysfunction, and worse recipient outcome. Demand for organs for …
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Design and Synthesis of Mitochondrial Uncouplers for the Treatment of Mitochondrial Dysfunction
Within the body, mitochondrial has a leading role in many cellular functions including energy production and cell signaling. When mitochondria become dysfunctional diseases such as obesity, type II diabetes mellitus, and metabolic dysfunction-associated steatohepatitis can occur. Mitochondrial …
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Effects of mitochondrial dysfunction on neurofilament turnover and distribution in human neuroblastoma cells
… used as a cellular model of neurodegeneration. Mitochondrial dysfunction, dopamine (DA) mediated oxidative stress and impaired protein degradation have all been implicated in PD pathogenesis. The complex I inhibitor MPTP and its active metabolite (MPP+) induce Parkinsonism in humans and other …
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The role of L-carnitine in preventing mitochondrial dysfunction after neonatal hypoxia-ischemia
… of cell death induced by neonatal HI is mitochondrial dysfunction leading to metabolic crisis and apoptosis. L-carnitine (LCAR) is an endogenous compound that transports fatty acids across the mitochondrial membrane for metabolism, buffers endogenous acyl-coA pools and improves the health …
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The role of L-carnitine in preventing mitochondrial dysfunction after neonatal hypoxia-ischemia
… of cell death induced by neonatal HI is mitochondrial dysfunction leading to metabolic crisis and apoptosis. L-carnitine (LCAR) is an endogenous compound that transports fatty acids across the mitochondrial membrane for metabolism, buffers endogenous acyl-coA pools and improves the health …
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Skeletal muscle weakness and mitochondrial dysfunction in the osteogenesis imperfecta murine (oim) model
… weakness primarily through the investigation of mitochondrial health in a mouse modeling a severe human type III OI (oim/oim), as mitochondria are important regulators of energy metabolism and overall cell health. We hypothesized that oim/oim mice, exhibiting severe skeletal muscle weakness would …
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Targeting neurodegenerative phenotypes and mitochondrial dysfunction in a Drosophila Melanogaster model of Parkinson’s Disease
… disorders, sleep disturbances, and autonomic dysfunction. Mitochondrial dysfunction, particularly involving Complex I of the electron transport system and mitophagy pathways, is significantly affected in the pathogenesis of PD. This study investigates the roles of NUBPL and PINK1 mutations in …
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Unraveling dual dysfunction in Friedreich’s ataxia: how mitochondrial dysfunction and microglial activation drive neurodegeneration
… of frataxin. It has been speculated that mitochondrial function could be contributing to FRDA. Frataxin is a mitochondrial protein crucial for iron-sulfur cluster biogenesis and mitochondrial function. When frataxin levels are depleted, mitochondrial dysfunction, increased oxidative …
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Investigating the role of mitochondrial dysfunction in a Drosophila model of C9orf72 ALS/FTD
Mitochondrial dysfunction is a prevalent feature in many neurodegenerative diseases including Amyotrophic Lateral Sclerosis (ALS) and Frontotemporal Dementia (FTD). ALS is a debilitating and incurable disease characterised by the loss of upper and lower motor neurons leading to symptoms such as …
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Mechanisms of ventricular arrhythmogenesis in the age dependent Pgc-1β -/- model of mitochondrial dysfunction.
… experimental evidence links metabolic disease, mitochondrial dysfunction and ageing as independent risk factors for arrhythmogenesis beyond the risk they confer to coronary artery disease. To date, researchers have focused upon investigation of monogenic ion channel disorders as well …
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