title: Heat shock protein 25 expression and preferential Purkinje cell survival in the lurcher mutant mouse cerebellum.
abstract: The spatial organization of the mouse cerebellum into transverse zones and parasagittal stripes is reflected during the temporal progression of Purkinje cell death in the Lurcher mutant mouse (+/Lc). Neurodegeneration in the +/Lc mutant is apparent by the second postnatal week and is initially seen in all four transverse zones: the anterior (lobules I-V), central (lobules VI, VII), posterior (lobules VIII, dorsal IX), and nodular (ventral lobule IX and lobule X) zone. However, from postnatal day (P)25-P36, Purkinje cell loss proceeds more rapidly in the anterior zone, followed by the posterior and central zones, and is significantly delayed in the nodular zone. Coronal sections through the +/Lc cerebellum reveal that surviving Purkinje cells are restricted to the paraflocculus/flocculus and the nodular zone and could be detected as late as P146 (approximately 5 months). Within this region, the pattern of preferentially surviving calbindin-immunoreactive Purkinje cells reflects the expression of the constitutively expressed small heat shock protein HSP25 in the wild-type cerebellum. Although the role of constitutively expressed HSP25 in the wild-type cerebellum is not clear, it appears to play a neuroprotective role in the flocculonodular region of the +/Lc mutant cerebellum as the percentage of surviving Purkinje cells that are HSP25-immunopositive significantly increases over time.
authors.affiliation: Department of Biomedical Science, Ontario Veterinary College, University of Guelph, Guelph, Ontario, Canada.
authors.forename: C A
authors.initials: CA
authors.lastname: Duffin
authors.forename: R
authors.initials: R
authors.lastname: McFarland
authors.forename: J R
authors.initials: JR
authors.lastname: Sarna
authors.forename: M W
authors.initials: MW
authors.lastname: Vogel
authors.forename: C L
authors.initials: CL
authors.lastname: Armstrong
mesh_terms: D000818:Animals; D064026:Calbindins; D002470:Cell Survival; D002531:Cerebellum / Q000473:pathology; D006360:Heat-Shock Proteins / Q000235:genetics / Q000378:metabolism*; D051379:Mice; D008818:Mice, Neurologic Mutants; D018832:Molecular Chaperones; D009363:Neoplasm Proteins / Q000235:genetics / Q000378:metabolism*; D009410:Nerve Degeneration / Q000235:genetics / Q000378:metabolism* / Q000473:pathology; D011689:Purkinje Cells / Q000166:cytology / Q000473:pathology* / Q000502:physiology*; D064030:S100 Calcium Binding Protein G / Q000378:metabolism
chemical_list: D064026:Calbindins; D006360:Heat-Shock Proteins; C486113:Hsbp1 protein, mouse; D018832:Molecular Chaperones; D009363:Neoplasm Proteins; D064030:S100 Calcium Binding Protein G