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• W589050506 abstract "Spinal Muscular Atrophy (SMA) is a childhood form of motor neuron disease thatcauses a progressive paralysis that, in its most severe form, results in death beforetwo years of age. There is currently no cure or treatment for SMA. SMA is causedby a reduction in levels of Survival Motor Neuron (SMN) protein, which results inthe degeneration of lower motor neurons. This degeneration is first observed at theneuromuscular junction (NMJ), where pre-synaptic nerve terminals belonging to themotor neuron become dysfunctional and degenerate during the early stages ofdisease. Several previous studies have shown that the some populations of motorneurons appear to have a resistance to SMA pathology, while other neighbouringpopulations are vulnerable. In this study, we attempted to elucidate the cause of thisvulnerability spectrum. Initially, we characterised the relative vulnerability of tendifferent motor unit pools in an established mouse model of severe SMA andattempted to correlate these vulnerabilities with quantified aspects of motor unitmorphology. From this study, no significant correlation could be found with anyaspect of motor unit morphology examined, suggesting that morphologicalparameters of motor neurons do no influence their relative susceptibility. We thenattempted to identify changes in basal gene expression between protected andvulnerable pools of motor units using microarray analysis. Motor unit pools werelabelled using a retrograde tracer injected into muscles that had previously beenidentified as having highly vulnerable or resistant motor units. Labelled motorneuron cell bodies were then isolated from the spinal cord using laser capture micro-dissectionand RNA was extracted for microarray analysis. From this study, weidentified several molecular pathways and individual genes whose expression levelscompared the gene expression profiles of vulnerable and resistant motor units. Thus,molecular differences between motor neuron pools likely underlie their relativevulnerability to degeneration in SMA. Lastly, we attempted to identify a novelpeptide that could be used to label synapses, including neuromuscular junctions, invivo. This would allow us to non-invasively visualise degenerating NMJs and othersynapses in human patients without the need for a biopsy. Such a tool would beextremely valuable in assessing the effectiveness of drug trials, both in humanpatients and animal models, and may also contribute to earlier diagnosis of motorneuron disorders. To identify a potentially suitable peptide, we used a phage displaylibrary and panned for peptides that specifically bound to the outer surface ofsynapses using synaptosome preparations. From this panning we successfullyenriched two peptides, the sequences of which were used to manufacturefluorescently tagged peptides." @default.
• W589050506 created "2016-06-24" @default.
• W589050506 creator A5008883674 @default.
• W589050506 date "2014-06-28" @default.
• W589050506 modified "2023-09-23" @default.
• W589050506 title "Examining mechanisms underlying the selective vulnerability of motor units in a mouse model of Spinal Muscular Atrophy" @default.
• W589050506 hasPublicationYear "2014" @default.
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