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  1. 050 医学部, 大学院医学系研究科
  2. 0501 学術論文

Survival motor neuron (SMN) protein in the spinal anterior horn cells of patients with sporadic amyotrophic lateral sclerosis

http://hdl.handle.net/10091/16795
http://hdl.handle.net/10091/16795
9b381f7a-2efb-4732-a095-0feb33350e2f
名前 / ファイル ライセンス アクション
SMN_protein_spinal_anterior_horn_cells.pdf SMN_protein_spinal_anterior_horn_cells.pdf (4.8 MB)
Item type 学術雑誌論文 / Journal Article(1)
公開日 2013-03-14
タイトル
タイトル Survival motor neuron (SMN) protein in the spinal anterior horn cells of patients with sporadic amyotrophic lateral sclerosis
言語
言語 eng
DOI
関連識別子 https://doi.org/10.1016/j.brainres.2010.11.070
関連名称 10.1016/j.brainres.2010.11.070
キーワード
主題 Amyotrophic lateral sclerosis, Anterior horn cell, Survival of motor neuron protein
資源タイプ
資源 http://purl.org/coar/resource_type/c_6501
タイプ journal article
著者 Piao, Yingshan

× Piao, Yingshan

en Piao, Yingshan

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Hashimoto, Tomoyo

× Hashimoto, Tomoyo

en Hashimoto, Tomoyo

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Takahama, Sachiko

× Takahama, Sachiko

en Takahama, Sachiko

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Kakita, Akiyoshi

× Kakita, Akiyoshi

en Kakita, Akiyoshi

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Komori, Takashi

× Komori, Takashi

en Komori, Takashi

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Morita, Takashi

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en Morita, Takashi

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Takahashi, Hitoshi

× Takahashi, Hitoshi

en Takahashi, Hitoshi

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Mizutani, Toshio

× Mizutani, Toshio

en Mizutani, Toshio

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Oyanagi, Kiyomitsu

× Oyanagi, Kiyomitsu

en Oyanagi, Kiyomitsu

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信州大学研究者総覧へのリンク
氏名 Oyanagi, Kiyomitsu
URL http://soar-rd.shinshu-u.ac.jp/profile/ja.jhTCPpym.html
出版者
出版者 ELSEVIER SCIENCE BV
引用
内容記述 BRAIN RESEARCH. 1372:152-159 (2011)
書誌情報 BRAIN RESEARCH

巻 1372, p. 152-159, 発行日 2011-02-04
抄録
内容記述 Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease involving mainly the upper and lower motor neurons of adult humans. With regard to the pathomechanism of spinal anterior horn cell (AHC) degeneration in ALS, copy number abnormalities of the survival motor neuron (SMN) genes have been reported in sporadic (s) ALS. SMN protein is the protein responsible for the pathogenesis of spinal muscular atrophy (SMA), an autosomal recessive disease characterized by lower motor neuron loss and muscle atrophy. The disease is caused by deficiency of SMN protein induced by mutation of one of the SMA-associated genes, SMN1. To clarify the role of SMN protein in the degeneration of spinal AHCs in sALS, we examined the amount of cytoplasmic SMN protein in individual AHCs using cytofluorophotometry in 9 patients with sALS and 10 control subjects. It was found that: 1) SMN protein was present in the cytoplasm, nucleus and nucleolus of AHCs and in the nucleus of glial cells, 2) expression of SMN protein in AHCs was significantly associated with cell size in both sALS patients and controls, 3) expression of SMN protein per unit area in AHCs was similar in sALS patients and controls. These findings suggest that: 1) the amount of SMN protein in the cytoplasm of AHCs is strictly controlled in accordance with cell size, in both sALS patients and controls, 2) the amount of SMN protein in the AHCs of sALS patients may be reduced when the AHCs are atrophic, and 3) decrease of SMN protein in the AHCs of sALS patients may be a secondary, and not primary, phenomenon according to their sizes.
資源タイプ(コンテンツの種類)
ISSN
収録物識別子タイプ PISSN
収録物識別子 0006-8993
書誌レコードID
収録物識別子タイプ NCID
収録物識別子 AA0057324X
PubMed
識別子タイプ PMID
関連識別子 https://pubmed.ncbi.nlm.nih.gov/21122798
関連名称 21122798
権利
権利情報 Copyright© 2010 Elsevier B.V.
出版タイプ
出版タイプ AM
出版タイプResource http://purl.org/coar/version/c_ab4af688f83e57aa
WoS
URL http://gateway.isiknowledge.com/gateway/Gateway.cgi?&GWVersion=2&SrcAuth=ShinshuUniv&SrcApp=ShinshuUniv&DestLinkType=FullRecord&DestApp=WOS&KeyUT=000287293700016
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Cite as

Piao, Yingshan, Hashimoto, Tomoyo, Takahama, Sachiko, Kakita, Akiyoshi, Komori, Takashi, Morita, Takashi, Takahashi, Hitoshi, Mizutani, Toshio, Oyanagi, Kiyomitsu, 2011, Survival motor neuron (SMN) protein in the spinal anterior horn cells of patients with sporadic amyotrophic lateral sclerosis: ELSEVIER SCIENCE BV, 152–159 p.

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