NR AYHA
AU Iwasaki,Y.; Mimuro,M.; Yoshida,M.; Hashizume,Y.; Ito,M.; Kitamoto,T.; Wakayama,Y.; Sobue,G.
TI Enhanced Aquaporin-4 immunoreactivity in sporadic Creutzfeldt-Jakob disease
QU Neuropathology 2007 Aug; 27(4): 314-23
PT journal article
AB Aquaporin-4 (AQP-4) is a water channel protein located on the plasma membrane of astrocytes and is regulated under various conditions. In the present study, a series of brains with sporadic Creutzfeldt-Jakob disease (sCJD) were investigated to determine the possible contribution of AQP-4 in the development of sCJD pathology. Six cases of subacute spongiform encephalopathy (SSE) and four cases of panencephalopathic (PE)-type sCJD were included. Increased AQP-4 immunoreactivity compared to that in controls was observed in all sCJD patients, particularly in the cerebral neocortex and cerebellar cortex. AQP-4 immunoreactivity was present in the cell bodies and processes of protoplasmic astrocytes in SSE and around cell bodies and processes of hypertrophic astrocytes in PE-type sCJD. Analysis of serial sections showed the development of sCJD pathology, particularly in neocortical lesions, as follows: PrP deposition; spongiform change and gliosis; enhanced staining for AQP-4; hypertrophic astrocytosis; and neuronal loss and tissue rarefaction. Strong AQP-4 immunoreactivity was present in burnt-out lesions such as those of status spongiosus. These results indicate that increased AQP-4 expression in sCJD is an early pathologic event and appears to remain until the late disease stage. We suggest that increased expression of AQP-4 is a pathologic feature of sCJD.
MH Aged; Aged, 80 and over; Aquaporin 4/*metabolism; Blotting, Western; Brain/*metabolism/*pathology; Creutzfeldt-Jakob Syndrome/genetics/*metabolism/*pathology; Female; Humans; Immunohistochemistry; Male; Middle Aged; Polymerase Chain Reaction; Polymorphism, Restriction Fragment Length; Prions/genetics
AD Department of Neurology, Nagoya University Graduate School of Medicine, Nagoya, Japan. iwasaki@sc4.so-net.ne.jp
SP englisch
PO Australien