研究者詳細 - 小野寺　理

論文 - 小野寺　理

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Genotype-phenotype correlations in early onset ataxia with ocular motor apraxia and hypoalbuminaemia 査読

Akio Yokoseki, Tomohiko Ishihara, Akihide Koyama, Atsushi Shiga, Mitsunori Yamada, Chieko Suzuki, Yoshiki Sekijima, Kyoko Maruta, Miyuki Tsuchiya, Hidetoshi Date, Tatsuya Sato, Masayoshi Tada, Takeshi Ikeuchi, Shoji Tsuji, Masatoyo Nishizawa, Osamu Onodera

BRAIN 134 ( Pt 5 ) 1387 - 1399 2011年5月

Cerebral small-vessel disease protein HTRA1 controls the amount of TGF-β1 via cleavage of proTGF-β1. 査読

Shiga A, Nozaki H, Yokoseki A, Nihonmatsu M, Kawata H, Kato T, Koyama A, Arima K, Ikeda M, Katada S, Toyoshima Y, Takahashi H, Tanaka A, Nakano I, Ikeuchi T, Nishizawa M, Onodera O

Human molecular genetics 20 ( 9 ) 1800 - 1810 2011年5月

Polyglutamine Diseases: Where does Toxicity Come from? What is Toxicity? Where are We Going? 査読

Toshiaki Takahashi, Shinichi Katada, Osamu Onodera

JOURNAL OF MOLECULAR CELL BIOLOGY 2 ( 4 ) 180 - 191 2010年8月

Association of HTRA1 Mutations and Familial Ischemic Cerebral Small-Vessel Disease 査読

Kenju Hara, Atsushi Shiga, Toshio Fukutake, Hiroaki Nozaki, Akinori Miyashita, Akio Yokoseki, Hirotoshi Kawata, Akihide Koyama, Kunimasa Arima, Toshiaki Takahashi, Mari Ikeda, Hiroshi Shiota, Masato Tamura, Yutaka Shimoe, Mikio Hirayama, Takayo Arisato, Sohei Yanagawa, Akira Tanaka, Imaharu Nakano, Shu-ichi Ikeda, Yutaka Yoshida, Tadashi Yamamoto, Takeshi Ikeuchi, Ryozo Kuwano, Masatoyo Nishizawa, Shoji Tsuji, Osamu Onodera

NEW ENGLAND JOURNAL OF MEDICINE 360 ( 17 ) 1729 - 1739 2009年4月

TDP-43 mutation in familial amyotrophic lateral sclerosis 査読

Akio Yokoseki, Atsushi Shiga, Chun-Feng Tan, Asako Tagawa, Hiroyuki Kaneko, Akihide Koyama, Hiroto Eguchi, Akira Tsujino, Takeshi Ikeuchi, Akiyoshi Kakita, Koichi Okamoto, Masatoyo Nishizava, Hitoshi Takahashi, Osamu Onodera

ANNALS OF NEUROLOGY 63 ( 4 ) 538 - 542 2008年4月

Soluble polyglutamine oligomers formed prior to inclusion body formation are cytotoxic 査読

Toshiaki Takahashi, Shinya Kikuchi, Shinichi Katada, Yoshitaka Nagai, Masatoyo Nishizawa, Osamu Onodera

HUMAN MOLECULAR GENETICS 17 ( 3 ) 345 - 356 2008年2月

Aprataxin, causative gene product for EAOH/AOA1, repairs DNA single-strand breaks with damaged 3 '-phosphate and 3 '-phosphoglycolate ends 査読

Tetsuya Takahashi, Masayoshi Tada, Shuichi Igarashi, Akihide Koyama, Hidetoshi Date, Akio Yokoseki, Atsushi Shiga, Yutaka Yoshida, Shoji Tsuji, Masatoyo Nishizawa, Osamu Onodera

NUCLEIC ACIDS RESEARCH 35 ( 11 ) 3797 - 3809 2007年6月

Early development of autonomic dysfunction may predict poor prognosis in patients with multiple system atrophy 査読

Mari Tada, Osamu Onodera, Masayoshi Tada, Tetsutaro Ozawa, Yue-Shan Piao, Akiyoshi Kakita, Hitoshi Takahashi, Masatoyo Nishizawa

ARCHIVES OF NEUROLOGY 64 ( 2 ) 256 - 260 2007年2月

Early-onset ataxia with ocular motor apraxia and hypoalbuminemia is caused by mutations in a new HIT superfamily gene 査読

H Date, O Onodera, H Tanaka, K Iwabuchi, K Uekawa, S Igarashi, R Koike, T Hiroi, T Yuasa, Y Awaya, T Sakai, T Takahashi, H Nagatomo, Y Sekijima, Kawachi, I, Y Takiyama, M Nishizawa, N Fukuhara, K Saito, S Sugano, S Tsuji

NATURE GENETICS 29 ( 2 ) 184 - 188 2001年10月

Progressive atrophy of cerebellum and brainstem as a function of age and the size of the expanded CAG repeats in the MJD1 gene in Machado-Joseph disease 査読

O Onodera, J Idezuka, S Igarashi, Y Takiyama, K Endo, H Takano, M Oyake, H Tanaka, T Inuzuka, T Hayashi, T Yuasa, J Ito, T Miyatake, S Tsuji

ANNALS OF NEUROLOGY 43 ( 3 ) 288 - 296 1998年3月

Somatic mosaicism of expanded CAG repeats in brains of patients with dentatorubral-pallidoluysian atrophy: Cellular population-dependent dynamics of mitotic instability 査読

H Takano, O Onodera, H Takahashi, S Igarashi, M Yamada, M Oyake, T Ikeuci, R Koide, H Tanaka, K Iwabuchi, S Tsuji

AMERICAN JOURNAL OF HUMAN GENETICS 58 ( 6 ) 1212 - 1222 1996年6月

MOLECULAR-CLONING OF A FULL-LENGTH CDNA FOR DENTATORUBRAL-PALLIDOLUYSIAN ATROPHY AND REGIONAL EXPRESSIONS OF THE EXPANDED ALLELES IN THE CNS 査読

O ONODERA, M OYAKE, H TAKANO, T IKEUCHI, S IGARASHI, S TSUJI

AMERICAN JOURNAL OF HUMAN GENETICS 57 ( 5 ) 1050 - 1060 1995年11月

GENETIC ASSOCIATION OF THE VERY-LOW-DENSITY LIPOPROTEIN (VLDL) RECEPTOR GENE WITH SPORADIC ALZHEIMERS-DISEASE 査読

K OKUIZUMI, O ONODERA, Y NAMBA, K IKEDA, T YAMAMOTO, K SEKI, A UEKI, S NANKO, H TANAKA, H TAKAHASHI, K OYANAGI, H MIZUSAWA, KANAZAWA, I, S TSUJI

NATURE GENETICS 11 ( 2 ) 207 - 209 1995年10月

UNSTABLE EXPANSION OF CAG REPEAT IN HEREDITARY DENTATORUBRAL-PALLIDOLUYSIAN ATROPHY (DRPLA) 査読

R KOIDE, T IKEUCHI, O ONODERA, H TANAKA, S IGARASHI, K ENDO, H TAKAHASHI, R KONDO, A ISHIKAWA, T HAYASHI, M SAITO, A TOMODA, T MIIKE, H NAITO, F IKUTA, S TSUJI

NATURE GENETICS 6 ( 1 ) 9 - 13 1994年1月

Export‑biased, 3′UTR‑preserving TDP‑43 model links nuclear loss to cytoplasmic aggregation in ALS/FTLD

Genri Toyama, Shingo Koide, Takuma Yamagishi, Aya Washida, Ryutaro Hanyu, Osamu Onodera, Akihiro Sugai

2025年11月

Tongue shear wave elastography for bulbar dysfunction in amyotrophic lateral sclerosis

Kaori Yanagawa, Makiko Ike, Azusa Aoyama, Takeshi Yokoo, Shuji Terai, Takafumi Hayashi, Osamu Onodera

Clinical Neurophysiology 2025年11月

Association of Plasma Placental Growth Factor with White Matter Hyperintensities in Alzheimer’s Disease

Kazuya Igarashi, Tamao Tsukie, Kazuo Washiyama, Kiyoshi Onda, Yuki Miyagi, Shoya Inagawa, Soichiro Shimizu, Akinori Miyashita, Osamu Onodera, Takeshi Ikeuchi, Kensaku Kasuga

Biomolecules 2025年9月

Late-onset multiple system atrophy: Neuropathological features associated with slow disease progression. 国際誌

Misato Ozawa, Rie Saito, Takuya Konno, Yasuko Kuroha, Tetsuhiko Ikeda, Akio Yokoseki, Takashi Tani, Tomoe Sato, Jiro Idezuka, Reiji Koide, Shigeru Fujimoto, Osamu Onodera, Mari Tada, Akiyoshi Kakita

Brain pathology (Zurich, Switzerland) 35 ( 5 ) e70016 2025年9月

記述言語：英語掲載種別：研究論文（学術雑誌）

Patients with late-onset (LO) multiple system atrophy (MSA), whose initial symptoms appear at age 75 years or older, are more common than previously assumed, but their clinicopathological characteristics remain unclear. We aimed to clarify the clinicopathological features of LO-MSA. Of 102 patients with autopsy-confirmed MSA, 5 were identified as having LO-MSA and 24 as having usual-age-onset MSA (UO-MSA) with a similar disease duration. On the basis of previous reports, we defined UO-MSA as the appearance of initial symptoms between the ages of 55 and 65 years. We compared the clinical pictures of the two groups and assessed their histopathological features using quantitative and semi-quantitative methods. The investigated features included the severity of degeneration in the striatonigral (StrN) and olivopontocerebellar (OPC) systems, the numbers of neurons in the brainstem autonomic and spinal intermediolateral nuclei, and the density of α-synuclein-immunopositive inclusions in the putamen, inferior olivary nucleus, and ventrolateral medulla (VLM). Most patients with both LO-MSA and UO-MSA exhibited the MSA-olivopontocerebellar atrophy (OPCA) subtype (3/5 and 18/24, respectively). The median disease duration for LO-MSA patients was 5.5 years, which was comparable to that for patients in our cohort who had developed symptoms below 75 years of age. Pathologically, degeneration of the StrN and OPC systems in LO-MSA was less severe than that observed in UO-MSA. Quantitative analysis revealed better preservation of neuron numbers in the brainstem autonomic nuclei in LO-MSA than in UO-MSA, with a significantly higher number of serotonergic neurons in the VLM (p = 0.013). The density of α-synuclein-positive inclusions in the putamen was significantly lower in LO-MSA than in UO-MSA (p < 0.001). Neuronal degeneration in LO-MSA may progress more slowly than in UO-MSA. Accordingly, the prognosis of LO-MSA may not necessarily be less favorable than that of MSA generally, especially with appropriate care.

DOI： 10.1111/bpa.70016

PubMed

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Triheptanoin treatment in ataxia-telangiectasia: the significance of innovative clinical trials targeting mitochondrial dysfunction 国際誌

Osamu Onodera

eBioMedicine 119 105882 - 105882 2025年9月

Early-onset alopecia and variable age of cerebrovascular disease onset in a Japanese family with a heterozygous HTRA1 mutation. 国際誌

Manato Yasuda, Masahiro Mori, Atsuhiko Sugiyama, Tomoki Suichi, Toshio Fukutake, Masahiro Uemura, Osamu Onodera, Satoshi Kuwabara

Journal of the neurological sciences 475 123567 - 123567 2025年8月

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