研究者詳細 - 小野寺　理

論文 - 小野寺　理

分割表示 492 件中 61 - 80 件目／全件表示 >>

Dysregulation of stress granule dynamics by DCTN1 deficiency exacerbates TDP-43 pathology in Drosophila models of ALS/FTD

Tetsuhiro Ueda, Toshihide Takeuchi, Nobuhiro Fujikake, Mari Suzuki, Eiko N. Minakawa, Morio Ueyama, Yuzo Fujino, Nobuyuki Kimura, Seiichi Nagano, Akio Yokoseki, Osamu Onodera, Hideki Mochizuki, Toshiki Mizuno, Keiji Wada, Yoshitaka Nagai

Acta Neuropathologica Communications 12 ( 1 ) 2024年2月

掲載種別：研究論文（学術雑誌）出版者・発行元：Springer Science and Business Media LLC

Abstract

The abnormal aggregation of TDP-43 into cytoplasmic inclusions in affected neurons is a major pathological hallmark of amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). Although TDP-43 is aberrantly accumulated in the neurons of most patients with sporadic ALS/FTD and other TDP-43 proteinopathies, how TDP-43 forms cytoplasmic aggregates remains unknown. In this study, we show that a deficiency in DCTN1, a subunit of the microtubule-associated motor protein complex dynactin, perturbs the dynamics of stress granules and drives the formation of TDP-43 cytoplasmic aggregation in cultured cells, leading to the exacerbation of TDP-43 pathology and neurodegeneration in vivo. We demonstrated using a Drosophila model of ALS/FTD that genetic knockdown of DCTN1 accelerates the formation of ubiquitin-positive cytoplasmic inclusions of TDP-43. Knockdown of components of other microtubule-associated motor protein complexes, including dynein and kinesin, also increased the formation of TDP-43 inclusions, indicating that intracellular transport along microtubules plays a key role in TDP-43 pathology. Notably, DCTN1 knockdown delayed the disassembly of stress granules in stressed cells, leading to an increase in the formation of pathological cytoplasmic inclusions of TDP-43. Our results indicate that a deficiency in DCTN1, as well as disruption of intracellular transport along microtubules, is a modifier that drives the formation of TDP-43 pathology through the dysregulation of stress granule dynamics.

DOI： 10.1186/s40478-024-01729-8

researchmap

その他リンク： https://link.springer.com/article/10.1186/s40478-024-01729-8/fulltext.html

Validation study of the Japanese version of the King's Parkinson's Disease Pain Scale and the King's Parkinson's Disease Pain Questionnaire. 国際誌

Kanako Kurihara, Shinsuke Fujioka, Yasuaki Mizutani, Hirohisa Watanabe, Kazuhiro Iwaoka, Tetsuya Maeda, Morinobu Seki, Toshiki Tezuka, Jin Nakahara, Takuya Konno, Takanobu Ishiguro, Osamu Onodera, Yuri Asano, Kazushi Takahashi, Alexandra Rizos, K Ray Chaudhuri, Yoshio Tsuboi

Parkinsonism & related disorders 120 106012 - 106012 2024年1月

記述言語：英語掲載種別：研究論文（学術雑誌）

INTRODUCTION: The King's Parkinson's Disease Pain Scale (KPPS)/King's Parkinson's Disease Pain Questionnaire (KPPQ) was developed as a tool to quantitatively assess pain in patients with Parkinson's disease (PwPD). Here, we conducted a Japanese multicenter validation study to verify the reliability of KPPS/KPPQ in Japanese PwPD. METHODS: PwPD, ≥20 years, with unexplained pain were included; those with a definitive primary cause of pain other than PD were excluded. A total of 151 patients who fulfilled the criteria were analyzed, and test-retest reliability was investigated in 25 individuals. RESULTS: The 151 patients included 101 women (66.9 %); mean age 68.3 ± 9.9 years, mean disease duration 9.2 ± 5.2 years. The most frequent pain type in the KPPS classification was musculoskeletal pain (82.8 %). There was a positive correlation between KPPS total score and the Non-Motor Symptoms Scale (NMSS) total score, NMSS item 27, the Parkinson's disease sleep scale-version 2 (PDSS-2) total score, PDSS-2 item 10, the Parkinson's Disease Questionnaire-8 (PDQ-8) summary index and PDQ-8 item 7. Cronbach's alpha of KPPS was 0.626 (0.562-0.658) and the intraclass correlation coefficient of test-retest reliability was 0.740. Cronbach's alpha of KPPQ was 0.660 (0.617-0.705) and a test-retest reliability of kappa coefficient was 0.593 (0.0-1.0). CONCLUSIONS: KPPS correlated well with other scales for assessing pain. KPPS correlated well with patients' quality of life, non-motor symptoms, and sleep disturbances. The reproducibility of KPPS/KPPQ makes it suitable for continuous evaluation of the same patient. On the other hand, the internal consistency of KPPS/KPPQ is rather low.

DOI： 10.1016/j.parkreldis.2024.106012

PubMed

researchmap

Corrigendum: Regeneration of the cerebral cortex by direct chemical reprogramming of macrophages into neuronal cells in acute ischemic stroke. 国際誌

Itaru Ninomiya, Akihide Koyama, Yutaka Otsu, Osamu Onodera, Masato Kanazawa

Frontiers in cellular neuroscience 18 1372045 - 1372045 2024年

The Japan MSA registry: A multicenter cohort study of multiple system atrophy

Ayaka Chikada, Kenta Orimo, Jun Mitsui, Takashi Matsukawa, Hiroyuki Ishiura, Tatsushi Toda, Hidehiro Mizusawa, Yuji Takahashi, Masahisa Katsuno, Kazuhiro Hara, Osamu Onodera, Tomohiko Ishihara, Masayoshi Tada, Satoshi Kuwabara, Atsuhiko Sugiyama, Yoshitaka Yamanaka, Ryosuke Takahashi, Nobukatsu Sawamoto, Yusuke Sakato, Tomoyuki Ishimoto, Ritsuko Hanajima, Yasuhiro Watanabe, Hiroshi Takigawa, Tadashi Adachi, Koji Abe, Toru Yamashita, Hiroshi Takashima, Keiko Higashi, Junichi Kira, Ichiro Yabe, Masaaki Matsushima, Katsuhisa Ogata, Kinya Ishikawa, Yoichiro Nishida, Taro Ishiguro, Kokoro Ozaki, Tetsuya Nagata, Shoji Tsuji

Neurology and Clinical Neuroscience 2024年

掲載種別：研究論文（学術雑誌）

Background: Multiple system atrophy (MSA) is a neurodegenerative disorder characterized by autonomic failure and various motor symptoms. While MSA-C (cerebellar type) predominates in East Asia, MSA-P (parkinsonian type) predominates in Europe and North America. This nationwide patient registry aimed to (1) conduct a prospective natural history study of MSA in Japan, (2) facilitate patient recruitment for clinical trials, and (3) deposit bioresources and clinical information in a biobank. Methods: Thirteen institutions participated in this study. Clinical information was obtained by neurologists from the patients visiting the hospital every 12 months to assess the UMSARS Part 2 scores and by telephone interviews by nurses every 6 months to assess UMSARS Part 1 scores and to determine whether clinical events had occurred. Results: Demographic data from 329 MSA patients (216 MSA-C and 113 MSA-P) were analyzed. The mean age at symptom onset was 58.2 years (standard deviation, 8.9); the mean duration of symptoms at enrollment was 3.5 years (standard deviation, 2.2). The mean 12-month changes in the UMSARS Part 1 and Part 2 scores were 7.9 (standard deviation, 5.6) and 6.4 (standard deviation, 5.9), respectively. The patient registry proved useful in recruiting participants for clinical trials, including those with gene variants. Clinical information and biospecimens were deposited in a biobank. Discussion: The study highlighted the importance of telephone interviews in minimizing drop-out rates in natural history studies and demonstrated similar MSA progression rates across populations. The deposited bioresources are available to researchers upon request, aiming to contribute to future MSA researches.

DOI： 10.1111/ncn3.12809

Scopus

researchmap

Reliability study for the Japanese version of the Columbia Muscle Cramp Scale in amyotrophic lateral sclerosis

Masahiro Sawada, Takehisa Hirayama, Masaru Yanagihashi, Koji Fukushima, Yuishin Izumi, Tameto Naoi, Mitsuya Morita, Hitoshi Warita, Masashi Aoki, Yohei Iguchi, Masahisa Katsuno, Nobuhiro Ogawa, Makoto Urusitani, Tomohiko Ishihara, Osamu Onodera, Yoshitaka Murakami, Hiroshi Mitsumoto, Osamu Kano

Neurology and Clinical Neuroscience 2024年

掲載種別：研究論文（学術雑誌）

Background: The Columbia Muscle Cramp Scale (CMCS) is a useful tool for evaluating muscle cramps commonly observed in patients with amyotrophic lateral sclerosis (ALS). The CMCS comprises five subdomains: triggering factors, frequency, location, severity, and the degree to which cramps affect overall daily living. Aim: This study aimed to evaluate the reliability of the CMCS, which was translated into Japanese. Methods: Thirty patients with ALS (17 men and 13 women) from seven facilities were evaluated twice by two different evaluators (doctors, nurses, physical therapists, occupational therapists, and speech-language-hearing therapists). The degree of inter- and intra-rater agreement was evaluated using kappa statistics. Weighted kappa statistics and 95% confidence intervals accounted for multiple-order responses. Results: The average age was 60.4 (standard deviation: 10.4, range: 39–77) years. The kappa statistics for the inter-rater reliability of the CMCS items showed moderate to very good agreement, ranging from 0.56 (cramp triggering) to 0.91 (daily activity) at visit 1 and 0.51 (severity) to 0.96 (daily activity) at visit 2. The kappa statistics for the intra-rater reliability of the CMCS items also showed moderate to good agreement, ranging from 0.45 (frequency) to 0.79 (cramp triggering). Conclusion: The Japanese version of the CMCS demonstrated intra- and inter-rater reliability agreement and is expected to be useful for evaluating muscle cramps in patients with ALS.

DOI： 10.1111/ncn3.12838

Scopus

researchmap

Two cases of cerebral hemorrhage with ipsilateral hemichorea-hemiballism

Shinya Oginezawa, Shoichiro Ando, Yukiko Kinoshita, Shoko Aburatani, Ryota Kishi, Yutaka Otsu, Takanobu Ishiguro, Etsuji Saji, Masato Kanazawa, Osamu Onodera

Japanese Journal of Stroke 2024年

Japanese longitudinal biomarker study in progressive supranuclear palsy and corticobasal degeneration: Clinical features of the first registered patients and short-term follow-up analysis

Hiroshi Takigawa, Ritsuko Hanajima, Ikuko Aiba, Takayoshi Shimohata, Takahiko Tokuda, Mitsuya Morita, Osamu Onodera, Shigeo Murayama, Kazuko Hasegawa, Aya M. Tokumaru, Hisanori Kowa, Masato Kanazawa, Tameto Naoi, Kenji Nakashima, Takeshi Ikeuchi

Clinical Parkinsonism & Related Disorders 2024年

Case report: Progressive multifocal leukoencephalopathy co-occurring with neurosarcoidosis: early brain biopsy and appropriate therapy for PML resulted in a favorable prognosis. 国際誌

Qiannan Wang, Shintaro Tsuboguchi, Kouichirou Okamoto, Mari Tada, Akiyoshi Kakita, Kazuo Nakamichi, Makoto Oishi, Masato Kanazawa, Osamu Onodera

Frontiers in immunology 15 1447992 - 1447992 2024年

記述言語：英語掲載種別：研究論文（学術雑誌）

Progressive multifocal leukoencephalopathy (PML) is a rare central nervous system disease caused by JC virus (JCV) infection. Human immunodeficiency virus (HIV) infection is the greatest risk factor for PML. Other immunological diseases, including systemic sarcoidosis, have also been reported as risk factors for PML. Herein, we report a case of PML co-occurring with neurosarcoidosis. Early diagnosis using brain biopsy and appropriate therapeutic interventions achieved favorable outcomes. PML in patients with active intracranial neurosarcoidosis is extremely rare. We believe that it is important to perform brain biopsy at an early stage to allow diagnosis, even for central nervous system involvement with a progressive parenchymal lesion in patients with sarcoidosis, if PML is possible.

DOI： 10.3389/fimmu.2024.1447992

PubMed

researchmap

Clinicopathologic features of two unrelated autopsied patients with Charcot-Marie-Tooth disease carrying MFN2 gene mutation. 国際誌

Hideki Hayashi, Rie Saito, Hidetomo Tanaka, Norikazu Hara, Shin Koide, Yosuke Yonemochi, Tetsuo Ozawa, Mariko Hokari, Yasuko Toyoshima, Akinori Miyashita, Osamu Onodera, Kouichirou Okamoto, Takeshi Ikeuchi, Takashi Nakajima, Akiyoshi Kakita

Acta neuropathologica communications 11 ( 1 ) 207 - 207 2023年12月

Missense Variants in COL4A1/2 Are Associated with Cerebral Aneurysms: A Case Report and <span aria-describedby="tippy-31">Literature Review

Masahiro Uemura, Natsuki Tanaka, Shoichiro Ando, Takehiko Yanagihara, Osamu Onodera

Neurology International 2023年12月

Neuroprotective effects of oral metformin before stroke on cerebral small-vessel disease. 国際誌

Natsuki Akiyama, Takayuki Yamashiro, Itaru Ninomiya, Masahiro Uemura, Yorito Hattori, Masafumi Ihara, Osamu Onodera, Masato Kanazawa

Journal of the neurological sciences 456 122812 - 122812 2023年11月

記述言語：英語掲載種別：研究論文（学術雑誌）

BACKGROUND: Metformin (MET) treatment prior to stroke might have neuroprotective effects other than hypoglycemic effects. This study evaluated whether MET treatment prior to stroke is associated with neurological severity and functional outcome in patients with stroke who were not indicated for endovascular treatment and whether the effects of MET differ for each ischemic stroke subtype. METHODS: We investigated 160 type 2 diabetes mellitus patients with ischemic stroke without endovascular treatment who were taking some oral antidiabetic agents prior to stroke in two tertiary hospitals. Lower neurological severity was defined as a National Institutes of Health Stroke Scale score of 3 or lower on admission, and favorable functional outcome was defined as a modified Rankin Scale score = 0-2 at discharge. We analyzed the effects of MET on the neurological severity and functional outcome in each ischemic stroke subtype on logistic regression analysis with adjustments for multiple confounding factors. RESULTS: MET treatment prior to stroke was associated with lower stroke severity and favorable functional outcome. In the stroke subtypes, MET use affected both neurological severity (P = 0.037) and functional outcome (P = 0.041) in only patients with small-vessel disease (SVD). CONCLUSIONS: MET may be useful to improve the outcome of patients with SVD.

DOI： 10.1016/j.jns.2023.122812

PubMed

researchmap

Marked laterality of olivopontocerebellar pathology in an autopsied patient with MSA: Implications for degeneration and α-synuclein propagation. 国際誌

Misato Ozawa, Rie Saito, Takuya Konno, Reiji Koide, Shigeru Fujimoto, Osamu Onodera, Akiyoshi Kakita

Journal of neurology, neurosurgery, and psychiatry 2023年11月

小脳出血で失語症を呈した一例

木下悠紀子, 畠山公大, 大槻美佳, 石黒敬信, 佐治越爾, 金澤雅人, 小野寺理

臨床神経学 63 ( 11 ) 772 - 772 2023年11月

A case report of reversible cerebral vasoconstriction syndrome with thunderclap headache significantly exacerbated in the supine position and alleviated in the standing position. 国際誌

Genri Toyama, Shintaro Tsuboguchi, Kazuya Igarashi, Etsuji Saji, Takuya Konno, Osamu Onodera

BMC neurology 23 ( 1 ) 348 - 348 2023年10月

記述言語：英語掲載種別：研究論文（学術雑誌）

BACKGROUND: Reversible cerebral vasoconstriction syndrome (RCVS) is characterized by sudden onset thunderclap headache and multiple segmental reversible cerebral vasoconstrictions that improve within 3 months. The postpartum period is a well-known precipitating factor for the onset of RCVS. Cerebral venous thrombosis (CVT) causes thunderclap headaches in the postpartum period. While headache in CVT is sometimes exacerbated in the supine position, the severity of the headache in RCVS is usually independent of body position. In this study, we report a case of RCVS with thunderclap headache exacerbated in the supine position, and headache attacks that resolved quickly in the standing position during the postpartum period. CASE PRESENTATION: A 33-year-old woman presented with a sudden increase in blood pressure and thunderclap headache on the fifth postpartum day (day 1: the first sick day). The headache was severe and pulsatile, with onset in the supine position in bed, and peaked at approximately 10 s. It was accompanied by nausea and chills but there were no scintillating scotomas or ophthalmic symptoms. The headache resolved in the standing or sitting position but was exacerbated and became unbearable within a few seconds when the patient was in the supine position. Therefore, she was unable to lie supine at night. Computed tomography angiography (CTA) of the head on day 2 and magnetic resonance imaging (MRI) and magnetic resonance angiography (MRA) on day 3 showed no abnormalities. However, considering the possibility of RCVS, verapamil was initiated on day 3. The headache resolved the following day. MRA of the head on day 10 revealed diffuse and segmental stenoses in the bilateral middle and posterior cerebral arteries and basilar artery. Therefore, the patient was diagnosed with RCVS. The headache gradually resolved and disappeared completely on day 42. Cerebral vasoconstriction was also improved on MRA on day 43. CONCLUSIONS: This postpartum RCVS case was notable for the exacerbation of headaches in the supine position. For the diagnosis of thunderclap headache in the postpartum period, RCVS should be considered in addition to CVT when the patient presents with a headache that is exacerbated in the supine position.

DOI： 10.1186/s12883-023-03381-6

PubMed

researchmap

アルツハイマー連続体における脳脊髄液αシヌクレインの臨床的有用性の検討

五十嵐一也, 春日健作, 月江珠緒, 菊地正隆, 宮下哲典, 小野寺理, 岩坪威, 池内健, Japanese Alzheimer's Disease Neuroimaging Initiative

Dementia Japan 37 ( 4 ) 687 - 687 2023年10月

若年性認知症の初診時臨床的特徴と脳脊髄液バイオマーカーの比較

大滝悠莉, 春日健作, 月江珠緒, 宮下哲典, 小野寺理, 池内健

Dementia Japan 37 ( 4 ) 684 - 684 2023年10月

脳アミロイドアンギオパチー関連炎症における脳脊髄液バイオマーカー

徳武孝允, 春日健作, 月江珠緒, 石黒敬信, 五十嵐一也, 小野寺理, 池内健

Dementia Japan 37 ( 4 ) 664 - 664 2023年10月

進行性核上性麻痺の脳脊髄液バイオマーカーによるAT(N)分類と臨床像

石黒敬信, 春日健作, 渡邉緑, 木下悠紀子, 月江珠緒, 小野寺理, 池内健

Dementia Japan 37 ( 4 ) 662 - 662 2023年10月

脳脊髄液アミロイドβ(Aβ)38の臨床的意義の検討

月江珠緒, 春日健作, 菊地正隆, 五十嵐一也, 大滝悠莉, 石黒敬信, 宮下哲典, 小野寺理, 岩坪威, 池内健, J-ADNI

Dementia Japan 37 ( 4 ) 658 - 658 2023年10月

リアルワールドにおける血漿バイオマーカーの有用性の検討

春日健作, 月江珠緒, 五十嵐一也, 石黒敬信, 宮下哲典, 小野寺理, 池内健

Dementia Japan 37 ( 4 ) 658 - 658 2023年10月

　前のページ - 次のページ　

<小野寺 理>

論文 - 小野寺 理

<小野寺　理>

論文 - 小野寺　理