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dc.contributor.authorChen, P
dc.contributor.authorPeng, C
dc.contributor.authorLuff, J
dc.contributor.authorSpring, K
dc.contributor.authorWatters, D
dc.contributor.authorBottle, S
dc.contributor.authorFuruya, S
dc.contributor.authorLavin, MF
dc.contributor.editorDavid C Van Essen
dc.date.accessioned2017-05-03T12:20:08Z
dc.date.available2017-05-03T12:20:08Z
dc.date.issued2003
dc.date.modified2009-09-29T23:13:14Z
dc.identifier.issn0270-6474
dc.identifier.urihttp://hdl.handle.net/10072/6325
dc.description.abstractAtm 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.peerreviewedYes
dc.description.publicationstatusYes
dc.format.extent47661 bytes
dc.format.extent320335 bytes
dc.format.mimetypetext/plain
dc.format.mimetypeapplication/pdf
dc.languageEnglish
dc.language.isoeng
dc.publisherSociety for Neuroscience
dc.publisher.placeUSA
dc.publisher.urihttp://www.jneurosci.org/content/23/36/11453
dc.relation.ispartofpagefrom11453
dc.relation.ispartofpageto11460
dc.relation.ispartofjournalJournal of Neuroscience
dc.relation.ispartofvolume23
dc.subject.fieldofresearchBiomedical and clinical sciences
dc.subject.fieldofresearchPsychology
dc.subject.fieldofresearchcode32
dc.subject.fieldofresearchcode52
dc.titleOxidative Stress Is Responsible for Deficient Survival and Dendritogenesis in Purkinje Neurons from Ataxia-Telangiectasia Mutated Mutant Mice
dc.typeJournal article
dc.type.descriptionC1 - Articles
dc.type.codeC - 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.issued2003
gro.hasfulltextFull Text
gro.griffith.authorWatters, Dianne J.


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