title: Selective Transgenic Expression of Mutant Ubiquitin in Purkinje Cell Stripes in the Cerebellum.
abstract: The ubiquitin-proteasome system (UPS) is one of the major mechanisms for protein breakdown in cells, targeting proteins for degradation by enzymatically conjugating them to ubiquitin molecules. Intracellular accumulation of ubiquitin-B+1(UBB+1), a frameshift mutant of ubiquitin-B, is indicative of a dysfunctional UPS and has been implicated in several disorders, including neurodegenerative disease. UBB+1-expressing transgenic mice display widespread labeling for UBB+1in brain and exhibit behavioral deficits. Here, we show that UBB+1is specifically expressed in a subset of parasagittal stripes of Purkinje cells in the cerebellar cortex of a UBB+1-expressing mouse model. This expression pattern is reminiscent of that of the constitutively expressed Purkinje cell antigen HSP25, a small heat shock protein with neuroprotective properties.
authors.affiliation: Department of Neuroscience, Faculty of Health, Medicine and Life Sciences, Maastricht University, Maastricht, The Netherlands. l.m.verheijen-3@umcutrecht.nl.|Lab of Experimental Neurology, Brain Center Rudolf Magnus, University Medical Center Utrecht, Utrecht, The Netherlands. l.m.verheijen-3@umcutrecht.nl.
authors.forename: Bert M
authors.initials: BM
authors.lastname: Verheijen
authors.affiliation: Department of Neuroscience, Faculty of Health, Medicine and Life Sciences, Maastricht University, Maastricht, The Netherlands.
authors.forename: Romina J G
authors.initials: RJG
authors.lastname: Gentier
authors.affiliation: Department of Neuroscience, Faculty of Health, Medicine and Life Sciences, Maastricht University, Maastricht, The Netherlands.
authors.forename: Denise J H P
authors.initials: DJHP
authors.lastname: Hermes
authors.affiliation: Department of Neuroscience, Faculty of Health, Medicine and Life Sciences, Maastricht University, Maastricht, The Netherlands.
authors.forename: Fred W
authors.initials: FW
authors.lastname: van Leeuwen
authors.affiliation: Department of Neuroscience, Faculty of Health, Medicine and Life Sciences, Maastricht University, Maastricht, The Netherlands.|Department of Medical Neuroscience, Dalhousie University, Halifax, Nova Scotia, Canada.
authors.forename: David A
authors.initials: DA
authors.lastname: Hopkins
mesh_terms: D000818:Animals; D002525:Cerebellar Cortex / Q000378:metabolism; D002531:Cerebellum / Q000378:metabolism*; D015870:Gene Expression / Q000235:genetics; D008810:Mice, Inbred C57BL; D008822:Mice, Transgenic; D009154:Mutation / Q000235:genetics*; D011689:Purkinje Cells / Q000378:metabolism*; D025801:Ubiquitin / Q000235:genetics*
chemical_list: C527284:Ubb protein, mouse; D025801:Ubiquitin
keywords: Cerebellum; Heat shock protein 25; Purkinje cell stripes; Ubiquitin-B+1; Ubiquitin-proteasome system; Zebrin II