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 32 for “"motor neuron disease"”.
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Proteomic Profiling of Animal Models of Myotonia and Motor Neuron Disease
… transformation from slow-to-fast by decreased motor nerve activity, hypogravity, physical inactivity and in diseased states. Similarly muscle transformation from fast-to-slow can be evoked by increased muscle nerve activity or exercise. The multitude of protein changes that has been identified …
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Phosphoglycerate kinase 1 as a therapeutic target in motor neuron disease
… features in neurodegenerative conditions like motor neuron disease (MND) and supporting cell energy production is an attractive strategy to improve the resilience of neurons to disease pathology. Phosphoglycerate kinase 1 (PGK1) is a key enzyme in the first ATP-producing step in the glycolysis …
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Proteomic Profiling of Animal Models of Motor Neuron Disease and Muscular Dystrophy
… pathobiochemical mechanisms that underlie these diseases. Detailed mass spectrometryQbased analysis of various diseased muscle groups from animal models of motor neuron disease and XQlinked muscular dystrophy is presented, in addition to motor neuron disease associated globozoospermia. This …
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Human leptomeninges; inflammatory roles in motor neuron disease, and their potential in gene therapy
… of CNS, in the contexts of health and disease. This thesis presents post-mortem human leptomeningeal explants as an ex vivo model to explore human leptomeninges. The suitability of the model was established by assessing cell composition and tissue health. Cell markers identified four …
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Motor neuron disease in an African population: A review of current literature and a case series of the flail arm variant in the Western Cape
Background: Motor neuron disease (MND) is a devastating neurodegenerative disorder, with recognised phenotypic subtypes. Although prevalent in all parts of the world, little is described in the literature with regards motor neuron disease as it occurs in African populations. Aims: This study had …
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Characterization of Peripherin Isoforms in Amyotrophic Lateral Sclerosis
… unclear, it is found upregulated after traumatic neuronal injury and in the devastating neurodegenerative disease amyotrophic lateral sclerosis (ALS). Interestingly, peripherin overexpressing transgenic mice succumb to motor neuron disease with pathological hallmarks reminiscent of those found in …
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How can virtual reality benefit the lives of persons with MND?
… in exercise interventions for individuals with motor neuron disease (MND) and other disabilities. We developed Stackly, a space-themed stacking game, as an example of how VR-based exercise programs could be designed for this population. The primary objective of the game is to stack cubes in a …
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Identifying modifier genes in SMA model mice
… (SMA) involves the loss of nerve cells called motor neurons in the spinal cord and is classified as a motor neuron disease, it affects 1 in 5000-10000 newborns, one of the leading genetic causes of infant death in USA. Mutations in the SMN1, UBA1, DYNC1H1 and VAPB genes cause spinal muscular …
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Myasthenia Gravis mimics: An audit of cases identified at Groote Schuur Hospital over 20 years
… 6 functional neurological symptoms (FNS), 6 motor neuron disease (MND) variants, 3 mitochondrial cytopathies, 4 had definite or probable muscular dystrophy (MD), 1 progressive supranuclear palsy and 1 with a multiple sclerosis brainstem relapse. Median age at symptom onset was 10 years for …
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The Role of Vps54 in Drosophila melanogaster Neuronal Development and Age Progressive Neurodegeneration
… and is subsequently used as a model for human motor neuron disease. Presently, it is unclear how disruption of GARP complex function leads to motor neuron degeneration. To better understand the role of Vps54 in motor neuron development, function, and age-related neurodegeneration, we disrupted …
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The Effects of Δ9-tetrahydrocannabinol (THC) on development and hyperekplexia in embryonic zebrafish model
… inhibitory synapses. Hyperekplexia is an upper motor neuron disease characterized by hypertonia and exaggerated startle response. In Hyperekplexia, there is a decrease in inhibitory synapse caused by a loss of glycine receptors. Because previous studies have demonstrated the efficacy of THC in …
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A Role for BDNF-Trkb Signaling in the Modulation of Superoxide Dismutase-1 Expression
… lateral sclerosis (fALS), a degenerative motor neuron disease more commonly known as Lou Gehrig’s disease. The mechanism by which it causes degeneration and the extent of its involvement are currently unknown, although the present consensus is that a toxic gain-of-function mutation is …
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Real-time control of a robot using brain activity
Background: Motor neuron disease (MND) describes a range of pathologies in which a person's voluntary muscle control becomes progressively impaired. Whether MND is on-set alone, or - as it commonly would be - paired with muscular atrophy, sufferers of the disease can face difficulty in establishing …
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Stigma, perceived control and health-related quality of life for individuals experiencing Parkinson’s disease
… wellbeing for individuals with neurodegenerative motor conditions. Section 1 describes a systematic literature review of quantitative correlates of stigma for individuals with neurodegenerative conditions, which result in visible motor differences. Five electronic databases were searched …
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Role of eEF1A-2 in the pathogenesis of Motor Neurone Disease
Motor neuron disease, or amyotrophic lateral sclerosis (ALS), is a disorder characterised by progressive weakness and atrophy of skeletal muscles, caused by the selective loss of motor neurons. It occurs in both familial and sporadic forms. Although the genetic basis of the disease in a minority of …
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Interaction between DVAP, the orthologue of human VAPB, and Drosophila Sac1
Amyotrophic Lateral Sclerosis (ALS) is a motor neuron disease characterizeby devastating symptoms, such as muscle weakness, paralysis, and death within 5 years of disease onset. Mutations in human (VAMP)-associated protein B (hVAPB) gene cause ALS8. Interestingly, the Drosophila VAPB (DVAP) is …
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The role of outer-sphere residues and substrate-binding at the 3-mercaptopropionic acid dioxygenase (3mdo) active site: a combined spectroscopic and computational investigation
… disorders such as Alzheimer’s, Parkinson’s, and motor neuron disease. The active site of this enzyme class is comprised of a mononuclear Fe(II) bound by three protein derived histidine residues. A conserved feature among structurally characterized TDOs is a sequence of serine, histidine, and …
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Antibodies to acetylcholinesterase: their detection and clinical significance
… antibodies in patients with autoimmune disease and all forms of Motor Neuron Disease. Positive findings of previous researchers could have been due to poor assay specificity, and / or detection of allogenic anti-AChE antibodies whose characterisation had occurred since much of the early …
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Traumatic Brain Injury As An Environmental Risk Factor For Neurodegeneration: Elucidating Causal Mechanisms With Drosophila
… force impacting the head, but is a complex disease process with repercussions that continue to develop decades after the injury. There are no clinically proven therapeutics to combat this extensive secondary injury process. Even single TBIs contribute to the degenerative mechanisms, putting …
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Mitochondrial protein SFXN3 as a novel target in neurodegenerative diseases
Neurodegenerative diseases are a group of heterogeneous disorders consisting of the progressive degeneration of the nervous system. Despite major scientific advances, most neurodegenerative diseases have no cure. This results in a significant emotional, social, and economic burden across the globe. …
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