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- 저자 : Tetsuyuki Kitamoto (검색결과 2 건)
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Kohei Suzuyama,Makoto Eriguchi,Hiromu Minagawa,Hiroyuki Honda,Keita Kai,Tetsuyuki Kitamoto,Hideo Hara 대한신경과학회 2024 Journal of Clinical Neurology Vol.20 No.3
Background and Purpose The coast of Kyushu Island on Ariake Sea in Japan is known to be an accumulation area for patients with a proline-to-leucine substitution mutation at residue 102 (P102L) of the human prion protein gene (PRNP), which is associated with Gerstmann– Sträussler–Scheinker disease. We designated this geographical distribution as the “Ariake PRNP P102L variant.” The purpose of this study was to characterize the clinical features of this variant. Methods We enrolled patients with the PRNP P102L variant who were followed up at the Saga University Hospital from April 2002 to November 2019. The clinical information of patients were obtained from medical records, including clinical histories, brain magnetic resonance imaging (MRI), and electroencephalography (EEG). A brain autopsy was performed on one of the participants. Results We enrolled 24 patients from 19 family lines, including 12 males. The mean age at symptom onset was 60.6 years (range, 41–77 years). The incidence rate of the Ariake PRNP P102L variant was 3.32/1,000,000 people per year in Saga city. The initial symptoms were ataxia (ataxic gait or dysarthria) in 19 patients (79.2%), cognitive impairment in 3 (12.5%), and leg paresthesia in 2 (8.3%). The median survival time from symptom onset among the 18 fatal cases was 63 months (range, 23–105 months). Brain MRI revealed no localized cerebellar atrophy, but sparse diffusion-weighted imaging abnormalities were detected in 16.7% of the patients. No periodic sharp-wave complexes were identified in EEG. Neuropathological investigations revealed uni- and multicentric prion protein (PrP) plaques in the cerebral cortex, putamen, thalamus, and cerebellum of one patient. Western blot analysis revealed 8-kDa proteinase-Kresistant PrP. Conclusions This is the first report of the accumulation area of a PRNP P102L variant on the coast of Ariake Sea. The Ariake PRNP P102L variant can be characterized by a relatively long disease duration with sparse abnormalities in brain MRI and EEG relative to previous reports. Detailed interviews to obtain information on the birthplace and the family history of related symptoms are important to diagnosing a PRNP P102L variant.
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Yamamoto, Akira,Shin, Ryong-Woon,Hasegawa, Kazuhiro,Naki, Hironobu,Sato, Hiroyuki,Yoshimasu, Fumio,Kitamoto, Tetsuyuki 한림대학교 환경·생명과학연구소 2002 [일송 국제심포지엄] 노화와 만성퇴행성 신경질환 Vol.- No.4
Iron as well as aluminum is reported to accumulate in neurons with neurofibrillary tangles (NFTs) of Alzheimer's disease(AD) brain. Previously we demonstrated that aluminum(Ⅲ) shows phosphate-dependent binding with hyperphosphorylated τ(PHFτ), the major constituent of NFTs, thereby inducing aggregation of PHFτ. Herein we report that iron(Ⅲ) can also induce aggregation of soluble PHFτ to occur, iron in the oxidized state (Ⅲ) is essential since iron in the reduced state (Ⅲ) lacks such ability. Furthermore, iron (Ⅲ)-induced aggregation is reversed by reducing iron (Ⅲ) to iron (Ⅱ). Thus the iron-participating aggregation is mediated not only by τ phosphorylation but also by the transition of iron between reduced (Ⅱ) and oxidized (Ⅲ) states. Further incubation of insoluble PHFτ aggregates isolated from AD brain with reducing agents produced liberation of solubilized PHFτ and iron (Ⅱ), indicating that PHFτ in association with iron (Ⅲ) constitutes the insoluble pool of PHFτ. These results indicate that iron might play a role in the aggregation of PHFτ leading to the formation of NFTs in AD brain.
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