title: Nestin immunoreactivity of Purkinje cells in Creutzfeldt-Jakob disease.
abstract: Nestin, an intermediate filament protein, is mainly expressed in neural progenitor/stem cells in the central nervous system. Recently, we reported that nestin is expressed in Purkinje cells in patients with Creutzfeldt-Jakob disease (CJD). In this study, we examined a total of 19 CJD cerebella to analyze the intensity and pattern of nestin immunoreactivity of Purkinje cells in different pathological stages of degeneration in the cerebellar cortex. The results showed that the Purkinje cells were immunoreactive with nestin regardless of the severity of degenerative cerebellar cortex. Furthermore, we noted several different types of nestin immunoreactivity, indicated by diffuse and fine, coarse, and inclusion-like immunostainings within Purkinje cell bodies as well as dot-like staining outside of the cell bodies. In contrast, on examination of cerebella from non-CJD patients, 6 of 30 cases showed nestin immunoreactivity to a lesser extent. Thus, nestin-positive Purkinje cells are more common in CJD cerebella than in non-CJD cerebella. Although the mechanism of nestin expression in Purkinje cells is not yet understood, we suggest that such nestin-positive Purkinje cells are being reactivated to survive the cell death.
authors.affiliation: Department of Neurology, Gunma University Graduate School of Medicine, 3-39-22 Showa-machi, Maebashi, Gunma 371-8511, Japan. mizunoy@med.gunma-u.ac.jp
authors.forename: Yuji
authors.initials: Y
authors.lastname: Mizuno
authors.forename: Eisaku
authors.initials: E
authors.lastname: Ohama
authors.forename: Junko
authors.initials: J
authors.lastname: Hirato
authors.forename: Yoichi
authors.initials: Y
authors.lastname: Nakazato
authors.forename: Hitoshi
authors.initials: H
authors.lastname: Takahashi
authors.forename: Masamitsu
authors.initials: M
authors.lastname: Takatama
authors.forename: Toshiyuki
authors.initials: T
authors.lastname: Takeuchi
authors.forename: Koichi
authors.initials: K
authors.lastname: Okamoto
mesh_terms: D000293:Adolescent; D000328:Adult; D000544:Alzheimer Disease / Q000378:metabolism / Q000473:pathology; D000690:Amyotrophic Lateral Sclerosis / Q000378:metabolism / Q000473:pathology; D002525:Cerebellar Cortex / Q000378:metabolism / Q000473:pathology; D002531:Cerebellum / Q000378:metabolism / Q000473:pathology; D002648:Child; D007562:Creutzfeldt-Jakob Syndrome / Q000235:genetics* / Q000473:pathology; D003593:Cytoplasm / Q000378:metabolism / Q000473:pathology; D003712:Dendrites / Q000378:metabolism / Q000473:pathology; D005260:Female; D006801:Humans; D006816:Huntington Disease / Q000378:metabolism / Q000473:pathology; D007150:Immunohistochemistry; D007381:Intermediate Filament Proteins / Q000235:genetics*; D007965:Leukodystrophy, Globoid Cell / Q000378:metabolism / Q000473:pathology; D008297:Male; D008875:Middle Aged; D019578:Multiple System Atrophy / Q000378:metabolism / Q000473:pathology; D009157:Myasthenia Gravis / Q000378:metabolism / Q000473:pathology; D009419:Nerve Tissue Proteins / Q000235:genetics*; D064231:Nestin; D011689:Purkinje Cells / Q000473:pathology*
chemical_list: D007381:Intermediate Filament Proteins; C577484:NES protein, human; D009419:Nerve Tissue Proteins; D064231:Nestin