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  1. C100 医学部/医学系研究科
  2. C100a 雑誌掲載論文
  3. 学術雑誌

Progress toward the development of treatment of spinal and bulbar muscular atrophy

http://hdl.handle.net/2237/26976
http://hdl.handle.net/2237/26976
62064cbd-4692-41a0-bd97-5101daf91543
名前 / ファイル ライセンス アクション
EOOD_Figure_2.pdf EOOD_Figure_2.pdf ファイル公開:2018/05/25 (73.4 kB)
EOOD_Figure_1.pdf EOOD_Figure_1.pdf ファイル公開:2018/05/25 (115.1 kB)
Article_EOOD_Sahashi.pdf Article_EOOD_Sahashi.pdf ファイル公開:2018/05/25 (204.7 kB)
Item type 学術雑誌論文 / Journal Article(1)
公開日 2017-09-08
タイトル
タイトル Progress toward the development of treatment of spinal and bulbar muscular atrophy
言語 en
著者 Sahashi, Kentaro

× Sahashi, Kentaro

WEKO 73561

en Sahashi, Kentaro

Search repository
Hashizume, Atsushi

× Hashizume, Atsushi

WEKO 73562

en Hashizume, Atsushi

Search repository
Sobue, Gen

× Sobue, Gen

WEKO 73563

en Sobue, Gen

Search repository
Katsuno, Masahisa

× Katsuno, Masahisa

WEKO 73564

en Katsuno, Masahisa

Search repository
アクセス権
アクセス権 open access
アクセス権URI http://purl.org/coar/access_right/c_abf2
権利
言語 en
権利情報 This is an Accepted Manuscript of an article published by Taylor & Francis Group in Expert Opinion on Orphan Drugs on 25/05/2017, available online: http://www.tandfonline.com/10.1080/21678707.2017.1329088.
キーワード
主題Scheme Other
主題 Androgen receptor
キーワード
主題Scheme Other
主題 leuprorelin
キーワード
主題Scheme Other
主題 polyglutamine disease
キーワード
主題Scheme Other
主題 proteostasis
キーワード
主題Scheme Other
主題 RNA
キーワード
主題Scheme Other
主題 spinal and bulbar muscular atrophy
抄録
内容記述 Introduction: With greater longevity, the prevalence of neurodegenerative diseases including Alzheimer’s disease, Parkinson’s disease, and amyotrophic lateral sclerosis is increasing, but these diseases remain intractable. This is also the case for a hereditary motor neuron disease, spinal and bulbar muscular atrophy or SBMA. Areas covered: SBMA typically affects adult males, eliciting motor deficits due to progressive loss of lower motor neurons, while females do not manifest neurological signs. It results from a CAG-trinucleotide repeat expansion in the androgen receptor or AR gene which is translated into an expanded polyglutamine tract within the encoded protein. Ligand-dependent nuclear accumulation of the mutant AR is implicated to lead to transcriptional dysregulation and subsequent defects in pivotal cellular functions, causing motor neuron death. Expert opinion: Even though the pathogenesis has not been fully understood, recent advances especially in the dissection of pathomechanisms associated with the pathogenic AR protein and aggregates accelerate the development of potential targeted therapeutics such as administration of leuprorelin acetate, a potent luteinizing hormone-releasing hormone analog, which has been successfully tested in clinical trials. Combination therapies antagonizing causative mutant AR and its downstream targets hold promise to further open a therapeutic avenue for the establishment of disease modifying drugs for SBMA.
言語 en
内容記述タイプ Abstract
出版者
言語 en
出版者 Taylor & Francis
言語
言語 eng
資源タイプ
資源タイプresource http://purl.org/coar/resource_type/c_6501
タイプ journal article
出版タイプ
出版タイプ AM
出版タイプResource http://purl.org/coar/version/c_ab4af688f83e57aa
DOI
関連タイプ isVersionOf
識別子タイプ DOI
関連識別子 https://doi.org/10.1080/21678707.2017.1329088
ISSN
収録物識別子タイプ EISSN
収録物識別子 2167-8707
書誌情報 en : Expert Opinion on Orphan Drugs

巻 5, 号 6, p. 503-514, 発行日 2017-05-25
著者版フラグ
値 author
URI
識別子 http://dx.doi.org/10.1080/21678707.2017.1329088
識別子タイプ DOI
URI
識別子 http://hdl.handle.net/2237/26976
識別子タイプ HDL
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