dc.contributor.author | Chen, P | |
dc.contributor.author | Peng, C | |
dc.contributor.author | Luff, J | |
dc.contributor.author | Spring, K | |
dc.contributor.author | Watters, D | |
dc.contributor.author | Bottle, S | |
dc.contributor.author | Furuya, S | |
dc.contributor.author | Lavin, MF | |
dc.contributor.editor | David C Van Essen | |
dc.date.accessioned | 2017-05-03T12:20:08Z | |
dc.date.available | 2017-05-03T12:20:08Z | |
dc.date.issued | 2003 | |
dc.date.modified | 2009-09-29T23:13:14Z | |
dc.identifier.issn | 0270-6474 | |
dc.identifier.uri | http://hdl.handle.net/10072/6325 | |
dc.description.abstract | Atm gene-disrupted mice recapitulate the majority of characteristics observed in patients with the genetic disorder ataxia-telangiectasia (A-T). However, although they exhibit defects in neuromotor function and a distinct neurological phenotype, they do not show the progressive neurodegeneration seen in human patients, but there is evidence that ataxia-telangiectasia mutated (Atm)-deficient animals have elevated levels of oxidized macromolecules and some neuropathology. We report here that in vitro survival of cerebellar Purkinje cells from both Atm "knock-out" and Atm "knock-in" mice was significantly reduced compared with their wild-type littermates. Although most of the Purkinje neurons from wild-type mice exhibited extensive dendritic elongation and branching under these conditions, most neurons from Atm-deficient mice had dramatically reduced dendritic branching. An antioxidant (isoindoline nitroxide) prevented Purkinje cell death in Atm-deficient mice and enhanced dendritogenesis to wild-type levels. Furthermore, administration of the antioxidant throughout pregnancy had a small enhancing effect on Purkinje neuron survival in Atm gene-disrupted animals and protected against oxidative stress in older animals. These data provide strong evidence for a defect in the cerebellum of Atm-deficient mice and suggest that oxidative stress contributes to this phenotype. | |
dc.description.peerreviewed | Yes | |
dc.description.publicationstatus | Yes | |
dc.format.extent | 47661 bytes | |
dc.format.extent | 320335 bytes | |
dc.format.mimetype | text/plain | |
dc.format.mimetype | application/pdf | |
dc.language | English | |
dc.language.iso | eng | |
dc.publisher | Society for Neuroscience | |
dc.publisher.place | USA | |
dc.publisher.uri | http://www.jneurosci.org/content/23/36/11453 | |
dc.relation.ispartofpagefrom | 11453 | |
dc.relation.ispartofpageto | 11460 | |
dc.relation.ispartofjournal | Journal of Neuroscience | |
dc.relation.ispartofvolume | 23 | |
dc.subject.fieldofresearch | Biomedical and clinical sciences | |
dc.subject.fieldofresearch | Psychology | |
dc.subject.fieldofresearchcode | 32 | |
dc.subject.fieldofresearchcode | 52 | |
dc.title | Oxidative Stress Is Responsible for Deficient Survival and Dendritogenesis in Purkinje Neurons from Ataxia-Telangiectasia Mutated Mutant Mice | |
dc.type | Journal article | |
dc.type.description | C1 - Articles | |
dc.type.code | C - Journal Articles | |
gro.rights.copyright | © 2003 Society for Neuroscience. Reproduced in accordance with the copyright policy of the publisher. Please refer to the journal website for access to the definitive, published version. | |
gro.date.issued | 2003 | |
gro.hasfulltext | Full Text | |
gro.griffith.author | Watters, Dianne J. | |