| http://purl.uniprot.org/citations/18521935 | http://www.w3.org/1999/02/22-rdf-syntax-ns#type | http://purl.uniprot.org/core/Journal_Citation |
| http://purl.uniprot.org/citations/18521935 | http://www.w3.org/2000/01/rdf-schema#comment | "Spinal muscular atrophy (SMA) is a genetic disorder caused by depletion of survival motor neuron (SMN) protein and characterized by degeneration of alpha-motor neurons in the spinal cord. We investigated the morphology and differentiation of neurosphere-derived neural stem cells (NSCs) generated from the brains of a hypomorphic series of SMA mice. Neurospheres from the Smn(-/-);SMN2 mice, which represent a model of very severe SMA, produced NSCs with increased proliferation during growth and differentiation. These cells produced fewer Tuj1-positive neuronal cells, which displayed morphological alterations and had fewer and shorter neurites. The decrease in the number of Tuj1-positive cells was not a result of enhanced apoptosis but was accompanied by an increase in the number of nestin-positive cells. These results provide insight into the most severe model of SMA, in which SMN is nearly completely depleted, and suggest that SMN has a role in neurodevelopment as well as in neuromaintenance. Our work raises the possibility that SMN depletion affects neurodevelopment and neuromaintenance to varying extents, leading to SMA pathogenesis."xsd:string |
| http://purl.uniprot.org/citations/18521935 | http://purl.org/dc/terms/identifier | "doi:10.1002/jnr.21743"xsd:string |
| http://purl.uniprot.org/citations/18521935 | http://purl.uniprot.org/core/author | "MacKenzie A.E."xsd:string |
| http://purl.uniprot.org/citations/18521935 | http://purl.uniprot.org/core/author | "Kothary R."xsd:string |
| http://purl.uniprot.org/citations/18521935 | http://purl.uniprot.org/core/author | "Shafey D."xsd:string |
| http://purl.uniprot.org/citations/18521935 | http://purl.uniprot.org/core/date | "2008"xsd:gYear |
| http://purl.uniprot.org/citations/18521935 | http://purl.uniprot.org/core/name | "J Neurosci Res"xsd:string |
| http://purl.uniprot.org/citations/18521935 | http://purl.uniprot.org/core/pages | "2839-2847"xsd:string |
| http://purl.uniprot.org/citations/18521935 | http://purl.uniprot.org/core/title | "Neurodevelopmental abnormalities in neurosphere-derived neural stem cells from SMN-depleted mice."xsd:string |
| http://purl.uniprot.org/citations/18521935 | http://purl.uniprot.org/core/volume | "86"xsd:string |
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