title: Cell-specific expression of the epm1 (cystatin B) gene in developing rat cerebellum.
abstract: Cystatin B (cystB) is an anti-protease implicated in EPM1, a degenerative disease of the central nervous system. This work analyzes the pattern of expression of cystB in developing and adult cerebellum, identifying the cystB positive cells by double immune-fluorescence microscopy using specific cell markers. In primary glial cells, cystB is found in progenitor and differentiated oligodendrocytes as well as in astrocytes. In the cerebellum, only oligodendrocyte progenitors express cystB. In myelin-producing cells, cystB synthesis is strongly down-regulated and the protein is not detectable. Astrocytes and Bergmann glia express cystB at all the developmental stages analyzed both in the cell body and in the fibers. Most neurons of developing and adult rat cerebellum do not express detectable amounts of cystB, with the exception of the Purkinje cells and of some cells of the differentiated molecular layer. In human cerebellum, cystB is present in Purkinje cells and Bergmann glial fibers only. cystB is also found in the cortical neurons of the dentate gyrus of the hippocampus. In rat cerebellum, cystB forms a complex with a number of proteins, two of which are specific to the nervous system. The cellular co-localization of cystB and its partners in developing and adult cerebellum is also shown.
authors.affiliation: Laboratory of Cell Biology and Electron Microscopy, I.O.R., 40136 Bologna, Italy.
authors.forename: Massimo
authors.initials: M
authors.lastname: Riccio
authors.forename: Spartaco
authors.initials: S
authors.lastname: Santi
authors.forename: Maja
authors.initials: M
authors.lastname: Dembic
authors.forename: Rossella
authors.initials: R
authors.lastname: Di Giaimo
authors.forename: Elena
authors.initials: E
authors.lastname: Cipollini
authors.forename: Elvira
authors.initials: E
authors.lastname: Costantino-Ceccarini
authors.forename: Davide
authors.initials: D
authors.lastname: Ambrosetti
authors.forename: Nadir M
authors.initials: NM
authors.lastname: Maraldi
authors.forename: Marialuisa
authors.initials: M
authors.lastname: Melli
mesh_terms: D000375:Aging / Q000378:metabolism; D000818:Animals; D000831:Animals, Newborn; D001253:Astrocytes / Q000378:metabolism; D002454:Cell Differentiation / Q000235:genetics; D002478:Cells, Cultured; D002531:Cerebellum / Q000254:growth & development* / Q000378:metabolism; D055313:Cystatin B; D015891:Cystatins / Q000235:genetics*; D005455:Fluorescent Antibody Technique; D018507:Gene Expression Regulation, Developmental / Q000235:genetics*; D006801:Humans; D009413:Nerve Fibers, Myelinated / Q000378:metabolism; D016900:Neurofilament Proteins / Q000378:metabolism; D009457:Neuroglia / Q000378:metabolism*; D009479:Neuropeptides / Q000378:metabolism; D009836:Oligodendroglia / Q000378:metabolism; D011689:Purkinje Cells / Q000378:metabolism; D051381:Rats; D017207:Rats, Sprague-Dawley; D000075370:Receptors for Activated C Kinase; D013049:Spectrin / Q000378:metabolism; D013234:Stem Cells / Q000378:metabolism*
chemical_list: C524994:CSTB protein, human; C524992:Cstb protein, mouse; D015891:Cystatins; D016900:Neurofilament Proteins; D009479:Neuropeptides; C090294:RACK1 protein, mouse; D000075370:Receptors for Activated C Kinase; C044882:neurofilament protein L; D013049:Spectrin; D055313:Cystatin B