Differential expression of metallothioneins in human prion diseases

Citation
T. Kawashima et al., Differential expression of metallothioneins in human prion diseases, DEMENT G C, 11(5), 2000, pp. 251-262
Citations number
34
Language
INGLESE
art.tipo
Article
Categorie Soggetti
Neurology,"Neurosciences & Behavoir
Journal title
DEMENTIA AND GERIATRIC COGNITIVE DISORDERS
ISSN journal
1420-8008 → ACNP
Volume
11
Issue
5
Year of publication
2000
Pages
251 - 262
Database
ISI
SICI code
1420-8008(200009/10)11:5<251:DEOMIH>2.0.ZU;2-P
Abstract
We herein report an immunohistochemical and a Western blot analysis on meta l/free radical chelating proteins, metallothioneins (MTs; MT-I/II and MT-II I), in the brains of human prion disease patients with or without prion pro tein gene mutation and polymorphism. Irrespective of the isoforms of MTs, t he immunoreaction was detected in the cytoplasm and processes of the astroc ytes in the cerebral cortex and white matter in normal controls and prion d isease brains. Although the immunoreactivities for MTs in Creutzfeldt-Jakob disease (CJD) brains varied from case to case, they were generally depende nt upon the disease duration. In CJD patients with a relatively long diseas e course, the immunoreaction for both MT-I/II and MT-III in the astrocytes was significantly reduced, and this finding was not modified by the genotyp es of the patients. On the other hand, in patients with Gerstmann-Straussle r-Scheinker syndrome, MT-I/II immunoreactivity in the astrocytes was exclus ively reduced, while the immunoreaction for MT-III was relatively well pres erved. Especially the astrocytes in the vicinities of the kuru plaques exhi bited a weak or no immunoreaction even for MTs but a strong immunoreaction for glial fibrillary acidic protein. A quantitative Western blot analysis a lso revealed that MT-I/II protein accumulated in CJD brain with a short dis ease duration, whereas MT-III in CJD brain with a long disease duration was statistically significantly reduced in compa- rison to the normal brains. These findings suggest that the protein expression of MTs in the astrocytes is thus regulated differentially among human prion diseases and modified l ocally by such abnormal prion protein depositions as kuru plaques. Copyrigh t (C) 2000 S. Karger AG, Basel.