Cognitive dysfunction in Duchenne muscular dystrophy: a possible role for neuromodulatory immune molecules

Show simple item record Rae, Mark G. O'Malley, Dervla 2016-10-18T08:36:30Z 2016-10-18T08:36:30Z 2016-09-01
dc.identifier.citation Rae, M.G. and O'Malley, D. (2016) 'Cognitive dysfunction in Duchenne muscular dystrophy: a possible role for neuromodulatory immune molecules', Journal of Neurophysiology, 116(3), pp.1304-1315. doi: 10.1152/jn.00248.2016 en
dc.identifier.volume 116 en
dc.identifier.issued 3 en
dc.identifier.startpage 1304 en
dc.identifier.endpage 1315 en
dc.identifier.issn 0022-3077
dc.identifier.doi 10.1152/jn.00248.2016
dc.description.abstract Duchenne muscular dystrophy (DMD) is an X chromosome-linked disease characterized by progressive physical disability, immobility, and premature death in affected boys. Underlying the devastating symptoms of DMD is the loss of dystrophin, a structural protein that connects the extracellular matrix to the cell cytoskeleton and provides protection against contraction-induced damage in muscle cells, leading to chronic peripheral inflammation. However, dystrophin is also expressed in neurons within specific brain regions, including the hippocampus, a structure associated with learning and memory formation. Linked to this, a subset of boys with DMD exhibit nonprogressing cognitive dysfunction, with deficits in verbal, short-term, and working memory. Furthermore, in the genetically comparable dystrophin-deficient mdx mouse model of DMD, some, but not all, types of learning and memory are deficient, and specific deficits in synaptogenesis and channel clustering at synapses has been noted. Little consideration has been devoted to the cognitive deficits associated with DMD compared with the research conducted into the peripheral effects of dystrophin deficiency. Therefore, this review focuses on what is known about the role of full-length dystrophin (Dp427) in hippocampal neurons. The importance of dystrophin in learning and memory is assessed, and the potential importance that inflammatory mediators, which are chronically elevated in dystrophinopathies, may have on hippocampal function is also evaluated. en
dc.format.mimetype application/pdf en
dc.language.iso en en
dc.publisher American Physiological Society en
dc.rights © 2016, The American Physiological Society. en
dc.subject Duchenne muscular dystrophy en
dc.subject Dystrophin en
dc.subject Hippocampus en
dc.subject Learning en
dc.subject Memory en
dc.title Cognitive dysfunction in Duchenne muscular dystrophy: a possible role for neuromodulatory immune molecules en
dc.type Article (peer-reviewed) en
dc.internal.authorcontactother Dervla O'Malley, Physiology, University College Cork, Cork, Ireland. +353-21-490-3000 Email: en
dc.internal.availability Full text available en Access to this article is restricted until 12 months after publication by request of the publisher. en 2017-09-01
dc.description.version Accepted Version en
dc.internal.rssid 356937683
dc.contributor.funder Muscular Dystrophy Ireland
dc.description.status Peer reviewed en
dc.identifier.journaltitle Journal of Neurophysiology en
dc.internal.IRISemailaddress en

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