Accumulation of Astrocytic Aquaporin 4 and Aquaporin 1 in Prion Protein Plaques

Shoko Sadashima, Hiroyuki Honda, Satoshi O. Suzuki, Masahiro Shijo, Shinichi Aishima, Keita Kai, Junichi Kira, Toru Iwaki

研究成果: ジャーナルへの寄稿学術誌査読

4 被引用数 (Scopus)


Gerstmann-Sträussler-Scheinker (GSS) disease with P102L mutation and familial Creutzfeldt-Jakob disease (CJD) with V180I mutation are 2 major hereditary prion diseases in Japan. GSS and some familial CJD [V180I] exhibit characteristic prion protein (PrP) plaques. Overexpression of the astrocytic water channel proteins aquaporin (AQP) 1 and AQP4 was recently reported in sporadic CJD. To clarify the pathological characteristics of AQP1 and AQP4 in prion disease patient brains with plaque-type deposition, we investigated 5 patients with GSS, 2 patients with CJD [V180I], and 2 age-matched control cases without neurological diseases using immunohistochemistry and double immunofluorescence methods. We demonstrated that there is the intense expression of AQP1 and AQP4 around prion plaques, especially in distal astrocytic processes deep inside these plaques. Similar results have been reported in the senile plaques and ghost tangles of Alzheimer disease brains and a protective role of AQP4 in which AQP4 is redistributed toward the plaques and works as a barrier against the deleterious effects of these plaques has been suggested. Our results, which show a similar clustering of AQPs around PrP plaques, therefore support the possibility that AQPs also have a protective role in plaque formation in prion diseases.

ジャーナルJournal of neuropathology and experimental neurology
出版ステータス出版済み - 4月 1 2020

!!!All Science Journal Classification (ASJC) codes

  • 病理学および法医学
  • 神経学
  • 臨床神経学
  • 細胞および分子神経科学


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