title: Peroxisomal localization in the developing mouse cerebellum: implications for neuronal abnormalities related to deficiencies in peroxisomes.
abstract: In subjects with Zellweger syndrome, the most severe phenotype of peroxisomal biogenesis disorder, brain abnormalities include cortical dysplasia, neuronal heterotopia, and dysmyelination. To clarify the relationship between the lack of peroxisomes and neuronal abnormalities, we investigated peroxisomal localization in the mouse cerebellum, using double immunofluorescent staining for peroxisomal proteins. On immunostaining for peroxisomal matrix protein, while there are few peroxisomes in Purkinje cells, many locate in astroglia, especially soma of Bergmann glia. Clusters of peroxisomes were seen on the inferior side of the Purkinje cell layer in mice on postnatal days 3-5, and with time there was a shift to the superior side. The peroxisomal punctate pattern was seen to be radial and co-localized with Bergmann glial fibers. In cultured cells from the mouse cerebellum, peroxisomes were few in Purkinje cells, whereas many were evident in glial fibrillary acidic protein-positive cells. On the other hand, on immunostaining for peroxisomal membrane protein Pex14p, many particles were seen in Purkinje cells during all developmental stages, which means Purkinje cells possessed empty peroxisomal structures similar to findings of fibroblasts from the Zellweger patients. As peroxisomes in glial cells may control the development of neurons, the neuron-glial interaction and mechanisms of developing central nervous systems deserve ongoing attention.
authors.affiliation: Department of Pediatrics, Gifu University School of Medicine, 40 Tsukasa-machi, Gifu 500-8705, Japan. tomoko-n@cc.gifu-u.ac.jp
authors.forename: Tomoko
authors.initials: T
authors.lastname: Nagase
authors.forename: Nobuyuki
authors.initials: N
authors.lastname: Shimozawa
authors.forename: Yasuhiko
authors.initials: Y
authors.lastname: Takemoto
authors.forename: Yasuyuki
authors.initials: Y
authors.lastname: Suzuki
authors.forename: Masayuki
authors.initials: M
authors.lastname: Komori
authors.forename: Naomi
authors.initials: N
authors.lastname: Kondo
mesh_terms: D015067:17-Hydroxysteroid Dehydrogenases; D015094:3-Hydroxyacyl CoA Dehydrogenases / Q000378:metabolism; D000102:Acetyl-CoA C-Acyltransferase / Q000378:metabolism; D042962:Acyl-CoA Oxidase / Q000378:metabolism; D000367:Age Factors; D000818:Animals; D015415:Biomarkers; D064026:Calbindins; D015220:Calcium Channels / Q000378:metabolism; D002352:Carrier Proteins / Q000378:metabolism; D002374:Catalase / Q000378:metabolism; D002478:Cells, Cultured; D002531:Cerebellum / Q000166:cytology* / Q000254:growth & development*; D004622:Embryo, Mammalian / Q000033:anatomy & histology; D004746:Enoyl-CoA Hydratase; D005904:Glial Fibrillary Acidic Protein / Q000378:metabolism; D006801:Humans; D006836:Hydro-Lyases / Q000378:metabolism; D007150:Immunohistochemistry; D053496:Inositol 1,4,5-Trisphosphate Receptors; D008565:Membrane Proteins / Q000378:metabolism; D051379:Mice; D009097:Multienzyme Complexes / Q000378:metabolism; D009474:Neurons / Q000166:cytology* / Q000378:metabolism / Q000473:pathology; D064029:Peroxisomal Multifunctional Protein-2; D020675:Peroxisomes / Q000378:metabolism* / Q000473:pathology; D018160:Receptors, Cytoplasmic and Nuclear / Q000378:metabolism; D012097:Repressor Proteins; D064030:S100 Calcium Binding Protein G / Q000378:metabolism; D014746:Vimentin / Q000378:metabolism; D015211:Zellweger Syndrome / Q000473:pathology
chemical_list: D015415:Biomarkers; D064026:Calbindins; D015220:Calcium Channels; D002352:Carrier Proteins; D005904:Glial Fibrillary Acidic Protein; C506926:ITPR1 protein, human; D053496:Inositol 1,4,5-Trisphosphate Receptors; D008565:Membrane Proteins; D009097:Multienzyme Complexes; C455539:PEX14 protein, human; C499193:Pex14 protein, mouse; D018160:Receptors, Cytoplasmic and Nuclear; D012097:Repressor Proteins; D064030:S100 Calcium Binding Protein G; D014746:Vimentin; D015067:17-Hydroxysteroid Dehydrogenases; D015094:3-Hydroxyacyl CoA Dehydrogenases; C491288:Hsd17b4 protein, mouse; D002374:Catalase; D042962:Acyl-CoA Oxidase; D000102:Acetyl-CoA C-Acyltransferase; D006836:Hydro-Lyases; D064029:Peroxisomal Multifunctional Protein-2; C104587:HSD17B4 protein, human; D004746:Enoyl-CoA Hydratase