Enhanced Aquaporin-4 immunoreactivity in sporadic Creutzfeldt-Jakob disease

Yasushi Iwasaki, Maya Mimuro, Mari Yoshida, Yoshio Hashizume, Masumi Ito, Tetsuyuki Kitamoto, Yoshihiro Wakayama, Gen Sobue

Research output: Contribution to journalArticlepeer-review

14 Citations (Scopus)


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.

Original languageEnglish
Pages (from-to)314-323
Number of pages10
Issue number4
Publication statusPublished - 2007 Aug


  • Aquaporin-4
  • Astrocyte
  • Prion protein
  • Spongiform change
  • Sporadic Creutzfeldt-Jakob disease

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Clinical Neurology


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