Werner, Hauke B.

[["dc.bibliographiccitation.firstpage","533"],["dc.bibliographiccitation.issue","4"],["dc.bibliographiccitation.journal","The American Journal of Human Genetics"],["dc.bibliographiccitation.lastpage","546"],["dc.bibliographiccitation.volume","94"],["dc.contributor.author","Prukop, Thomas"],["dc.contributor.author","Epplen, Dirk B."],["dc.contributor.author","Nientiedt, Tobias"],["dc.contributor.author","Wichert, Sven P."],["dc.contributor.author","Fledrich, Robert"],["dc.contributor.author","Stassart, Ruth Martha"],["dc.contributor.author","Rossner, Moritz J."],["dc.contributor.author","Edgar, Julia M."],["dc.contributor.author","Werner, Hauke B."],["dc.contributor.author","Nave, Klaus-Armin"],["dc.contributor.author","Sereda, Michael W."],["dc.date.accessioned","2018-11-07T09:41:24Z"],["dc.date.available","2018-11-07T09:41:24Z"],["dc.date.issued","2014"],["dc.description.abstract","Pelizaeus-Merzbacher disease (PMD) is a severe hypomyelinating disease, characterized by ataxia, intellectual disability, epilepsy, and premature death. In the majority of cases, PMD is caused by duplication of PLP1 that is expressed in myelinating oligodendrocytes. Despite detailed knowledge of PLP1, there is presently no curative therapy for PMD. We used a Plp1 transgenic PMD mouse model to test the therapeutic effect of Lonaprisan, an antagonist of the nuclear progesterone receptor, in lowering Plp1 mRNA overexpression. We applied placebo-controlled Lonaprisan therapy to PMD mice for 10 weeks and performed the grid slip analysis to assess the clinical phenotype. Additionally, mRNA expression and protein accumulation as well as histological analysis of the central nervous system were performed. Although Plp1 mRNA levels are increased 1.8-fold in PMD mice compared to wild-type controls, daily Lonaprisan treatment reduced overexpression at the RNA level to about 1.5-fold, which was sufficient to significantly improve the poor motor phenotype. Electron microscopy confirmed a 25% increase in the number of myelinated axons in the corticospinal tract when compared to untreated PMD mice. Microarray analysis revealed the upregulation of proapoptotic genes in PMD mice that could be partially rescued by Lonaprisan treatment, which also reduced microgliosis, astrogliosis, and lymphocyte infiltration."],["dc.identifier.doi","10.1016/j.ajhg.2014.03.001"],["dc.identifier.isi","000333765300005"],["dc.identifier.pmid","24680886"],["dc.identifier.uri","https://resolver.sub.uni-goettingen.de/purl?gro-2/33720"],["dc.notes.status","zu prüfen"],["dc.notes.submitter","Najko"],["dc.publisher","Cell Press"],["dc.relation.issn","1537-6605"],["dc.relation.issn","0002-9297"],["dc.title","Progesterone Antagonist Therapy in a Pelizaeus-Merzbacher Mouse Model"],["dc.type","journal_article"],["dc.type.internalPublication","yes"],["dc.type.peerReviewed","yes"],["dc.type.status","published"],["dspace.entity.type","Publication"]]

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[["dc.bibliographiccitation.firstpage","1815"],["dc.bibliographiccitation.issue","16"],["dc.bibliographiccitation.journal","Glia"],["dc.bibliographiccitation.lastpage","1824"],["dc.bibliographiccitation.volume","57"],["dc.contributor.author","Edgar, Julia M."],["dc.contributor.author","McLaughlin, Mark"],["dc.contributor.author","Werner, Hauke B."],["dc.contributor.author","McCulloch, Mailis C."],["dc.contributor.author","Barrie, Jennifer A."],["dc.contributor.author","Brown, Angus"],["dc.contributor.author","Faichney, Andrew Blyth"],["dc.contributor.author","Snaidero, Nicolas"],["dc.contributor.author","Nave, Klaus-Armin"],["dc.contributor.author","Griffiths, Ian R."],["dc.date.accessioned","2021-12-08T12:30:33Z"],["dc.date.available","2021-12-08T12:30:33Z"],["dc.date.issued","2009"],["dc.identifier.doi","10.1002/glia.20893"],["dc.identifier.uri","https://resolver.sub.uni-goettingen.de/purl?gro-2/96474"],["dc.language.iso","en"],["dc.notes.intern","DOI-Import GROB-476"],["dc.relation.eissn","1098-1136"],["dc.relation.issn","0894-1491"],["dc.rights.uri","http://doi.wiley.com/10.1002/tdm_license_1.1"],["dc.title","Early ultrastructural defects of axons and axon-glia junctions in mice lacking expression of Cnp1"],["dc.type","journal_article"],["dc.type.internalPublication","yes"],["dspace.entity.type","Publication"]]

[["dc.bibliographiccitation.artnumber","glia.24290"],["dc.bibliographiccitation.journal","Glia"],["dc.contributor.author","Steyer, Anna M."],["dc.contributor.author","Buscham, Tobias J."],["dc.contributor.author","Lorenz, Charlotta"],["dc.contributor.author","Hümmert, Sophie"],["dc.contributor.author","Eichel‐Vogel, Maria A."],["dc.contributor.author","Schadt, Leonie C."],["dc.contributor.author","Edgar, Julia M."],["dc.contributor.author","Köster, Sarah"],["dc.contributor.author","Möbius, Wiebke"],["dc.contributor.author","Nave, Klaus‐Armin"],["dc.contributor.author","Werner, Hauke B."],["dc.date.accessioned","2022-12-01T08:31:03Z"],["dc.date.available","2022-12-01T08:31:03Z"],["dc.date.issued","2022"],["dc.description.sponsorship"," Deutsche Forschungsgemeinschaft https://doi.org/10.13039/501100001659"],["dc.identifier.doi","10.1002/glia.24290"],["dc.identifier.uri","https://resolver.sub.uni-goettingen.de/purl?gro-2/118053"],["dc.language.iso","en"],["dc.notes.intern","DOI-Import GROB-621"],["dc.relation.eissn","1098-1136"],["dc.relation.issn","0894-1491"],["dc.relation.orgunit","Institut für Röntgenphysik"],["dc.relation.workinggroup","RG Köster (Cellular Biophysics)"],["dc.rights.uri","http://creativecommons.org/licenses/by/4.0/"],["dc.subject.gro","molecular biophysics"],["dc.title","Focused ion beam‐scanning electron microscopy links pathological myelin outfoldings to axonal changes in mice lacking Plp1 or Mag"],["dc.type","journal_article"],["dc.type.internalPublication","yes"],["dspace.entity.type","Publication"]]