Please use this identifier to cite or link to this item: http://hdl.handle.net/10397/68978
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dc.contributorSchool of Optometry-
dc.creatorTse, D-
dc.creatorLotfi, P-
dc.creatorSimons, DL-
dc.creatorSardiello, M-
dc.creatorWu, SM-
dc.date.accessioned2017-10-30T07:54:46Z-
dc.date.available2017-10-30T07:54:46Z-
dc.identifier.urihttp://hdl.handle.net/10397/68978-
dc.language.isoenen_US
dc.publisherNature Publishing Groupen_US
dc.rightsThis work is licensed under a Creative Commons Attribution 4.0 International License. The images or other third party material in this article are included in the article’s Creative Commons license, unless indicated otherwise in the credit line; if the material is not included under the Creative Commons license, users will need to obtain permission from the license holder to reproduce the material. To view a copy of this license, visit http://creativecommons.org/licenses/by/4.0/en_US
dc.rightsThe following publication Tse, D., Lotfi, P., Simons, D. et al. Electrophysiological and Histological Characterization of Rod-Cone Retinal Degeneration and Microglia Activation in a Mouse Model of Mucopolysaccharidosis Type IIIB. Sci Rep 5, 17143 (2015) is available at https://dx.doi.org/10.1038/srep17143en_US
dc.subjectHereditary eye diseaseen_US
dc.subjectMetabolic disordersen_US
dc.subjectNeurodegenerative diseasesen_US
dc.subjectPathogenesisen_US
dc.titleElectrophysiological and histological characterization of rod-cone retinal degeneration and microglia activation in a mouse model of mucopolysaccharidosis Type IIIBen_US
dc.typeJournal/Magazine Articleen_US
dc.identifier.spage1-
dc.identifier.epage15-
dc.identifier.volume5-
dc.identifier.doi10.1038/srep17143-
dcterms.abstractSanfilippo syndrome Type B or Mucopolysaccharidosis IIIB (MPS IIIB) is a neurodegenerative autosomal recessive lysosomal storage disorder in which patients suffer severe vision loss from associated retinopathy. Here we sought to study the underlying retinal functional and morphological changes associated with MPS IIIB disease progression using the established model of MPS IIIB, the B6.129S6-Naglu(tm1Efn)/J mouse line. Electroretinogram (ERG) was recorded from MPS IIIB and wild-type (WT) mice at the age of 28 and 46 weeks, and retinal tissues were subsequently collected for immunohistochemistry analysis. At the 28th week, rod a- and b-wave amplitudes were significantly diminished in MPS IIIB compared to WT mice. The cone a- and b-waves of MPS IIIB mice were not significantly different from those of the control at the 28th week but were significantly diminished at the 46th week, when MPS IIIB mice showed a major loss of rods and rod bipolar cells in both central and peripheral regions and a minor loss of cones in the periphery. Activation of microglia and neovascularization were also detected in the MPS IIIB retina. The new findings that cones and rod bipolar cells also undergo degeneration, and that retinal microglia are activated, will inform future development of therapeutic strategies.-
dcterms.accessRightsopen accessen_US
dcterms.bibliographicCitationScientific reports, 26 2015, v. 5, no. , 17143, p. 1-15-
dcterms.isPartOfScientific reports-
dcterms.issued2015-
dc.identifier.eissn2045-2322-
dc.identifier.artn17143-
dc.identifier.rosgroupid2015002016-
dc.description.ros2015-2016 > Academic research: refereed > Publication in refereed journal-
dc.description.oaVersion of Recorden_US
dc.identifier.FolderNumberOA_IR/PIRAen_US
dc.description.pubStatusPublisheden_US
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