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Progress toward the development of treatment of spinal and bulbar muscular atrophy
http://hdl.handle.net/2237/26976
http://hdl.handle.net/2237/2697662064cbd-4692-41a0-bd97-5101daf91543
名前 / ファイル | ライセンス | アクション |
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Item type | 学術雑誌論文 / Journal Article(1) | |||||
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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× Hashizume, Atsushi× Sobue, Gen× Katsuno, Masahisa |
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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 |
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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 |