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Aberrant accumulation of ErbB4 in progressive supranuclear palsy. Aberrant accumulation of ErbB4 in progressive supranuclear palsy. （進行性核上性麻痺における ErbB4 の異常蓄積） 村上, 綾 ムラカミ, アヤ Murakami, A Nakamura, M Kaneko, S Lin, W-L Dickson, D W Kusaka, H Aged Aged, 80 and over Brain Female Humans Male Middle Aged Neuregulin-1 Neurofibrillary Tangles Neurons Receptor, ErbB-4 Supranuclear Palsy, Progressive 平成30年度 The human epidermal growth factor receptor family consists of four members that belong to the ErbB lineage of proteins (ErbB1-4). Neuregulin-1 (NRG1)/ErbB signalling regulates brain development and function. Abnormalities in this signalling have been implicated in the aetiology or development of neurodegenerative diseases such as Alzheimer's disease, Parkinson's disease and amyotrophic lateral sclerosis. So, we aimed at investigating whether the expression of NRG1 or ErbB proteins are altered in progressive supranuclear palsy (PSP). The brains of 10 PSP and six control patients were investigated by immunohistochemical analysis. Whereas C-terminal ErbB4 immunoreacitivity was partially but distinctly present in the cytoplasm and/or in the nucleus of neurons in control patients, it was rarely observed in the neuronal nuclei in PSP patients. In contrast, neurofibrillary tangles, coiled bodies and threads were robustly immunoreactive for C-terminal ErbB4 in PSP. Double immunofluorescence for C-terminal ErbB4 and phospho-tau revealed co-localization of these proteins within neuronal and glial inclusions. To the contrary, there was no difference in the subcellular localization of NRG1, ErbB1, ErbB2, and N-terminal ErbB4 between control and PSP patients. These proteins were localized in the cytoplasm of neurons. Our present results suggest that NRG1/ErbB4 signalling could be an important event in the pathogenesis of PSP. 2018 eng thesis https://kmu.repo.nii.ac.jp/records/122 Neuropathology and applied neurobiology Neuropathology and applied neurobiology 44 6 563 573 課博第1028号 博士（医学） 2018-09-25 関西医科大学 https://kmu.repo.nii.ac.jp/record/122/files/甲1028　論文要旨（要約）.pdf application/pdf 209.7 kB 2020-08-25 https://kmu.repo.nii.ac.jp/record/122/files/甲1028　審査結果要旨.pdf application/pdf 91.2 kB 2020-08-25