Nestin immunoreactivity of Purkinje cells in Creutzfeldt-Jakob disease.
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.
Mizuno
Yuji
Y
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
Ohama
Eisaku
E
Hirato
Junko
J
Nakazato
Yoichi
Y
Takahashi
Hitoshi
H
Takatama
Masamitsu
M
Takeuchi
Toshiyuki
T
Okamoto
Koichi
K
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*
D007381:Intermediate Filament Proteins; C577484:NES protein, human; D009419:Nerve Tissue Proteins; D064231:Nestin