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副腎白質ジストロフィーの分子病態の解明と治療薬開発
http://hdl.handle.net/10110/1749
http://hdl.handle.net/10110/1749e001de4b-3ebc-4436-930a-bfda9e2fe22c
名前 / ファイル | ライセンス | アクション |
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yakugakuzasshi07-1059.pdf (675.3 kB)
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Item type | 学術雑誌論文 / Journal Article(1) | |||||
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公開日 | 2008-01-10 | |||||
タイトル | ||||||
タイトル | 副腎白質ジストロフィーの分子病態の解明と治療薬開発 | |||||
言語 | ||||||
言語 | jpn | |||||
キーワード | ||||||
主題Scheme | Other | |||||
主題 | adrenoleukodystrophy | |||||
キーワード | ||||||
主題Scheme | Other | |||||
主題 | ATP-binding cassette protein | |||||
キーワード | ||||||
主題Scheme | Other | |||||
主題 | flavonoid | |||||
キーワード | ||||||
主題Scheme | Other | |||||
主題 | very long chain fatty acid | |||||
資源タイプ | ||||||
資源タイプ識別子 | http://purl.org/coar/resource_type/c_6501 | |||||
資源タイプ | journal article | |||||
著者 |
守田, 雅志
× 守田, 雅志 |
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著者別名 | ||||||
姓名 | Morita, Masashi | |||||
その他(別言語等)のタイトル | ||||||
その他のタイトル | Adrenoleukodystrophy : Molecular Pathogenesis and Development of Therapeutic Agents | |||||
抄録 | ||||||
内容記述タイプ | Abstract | |||||
内容記述 | Adrenoleukodystrophy (ALD) is an inherited disorder characterized by progressive demyelination of the central nervous system and adrenal dysfunction. The biochemical characterization is made based on the accumulation of pathgnomonic amounts of saturated very long chain fatty acid (VLCFA, >22) in all tissues, including brain white matter and adrenal glands. The accumulation of VLCFA is linked to a mutation in the ABCD1 gene that encodes ABCD1/ALDP, a peroxisomal ABC protein. ABCD1/ALDP is thought to be involved in the active ATP-driven transport of VLCFA-CoA from the cytoplasm into the peroxisomes. However, the precise function of ABCD1/ALDP is still unclear. The accumulation of VLCFA is caused by reducing peroxisomal VLCFA β-oxidation and/or increasing fatty acid elongation. Since the reduction of accumulated VLCFA in the brain is thought to be crucial for preventing the progression of neurologic symptoms in X-ALD, compounds that can cross the blood-brain barrier and decrease the VLCFA levels in the brain would be a highly attractive candidate for effective treatment of ALD patients. We found that baicalein 5,6,7-trimethyl ether, a flavonoid derivative, decreased the VLCFA level in X-ALD fibroblasts, possibly by activating peroxisomal fatty acid β-oxidation. Continued pharmacologic studies of flavonoids and chemically modified derivatives may lead to major advances in the pharmacologic therapy for X-ALD. | |||||
書誌情報 |
藥學雜誌 : Journal of the Pharmaceutical Society of Japan (薬学雑誌) 巻 127, 号 7, p. 1059-1064, 発行日 2007-07 |
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ISSN | ||||||
収録物識別子タイプ | ISSN | |||||
収録物識別子 | 316903 | |||||
書誌レコードID | ||||||
収録物識別子タイプ | NCID | |||||
収録物識別子 | AN00284903 | |||||
フォーマット | ||||||
内容記述タイプ | Other | |||||
内容記述 | application/pdf | |||||
著者版フラグ | ||||||
出版タイプ | VoR | |||||
出版タイプResource | http://purl.org/coar/version/c_970fb48d4fbd8a85 | |||||
出版者 | ||||||
出版者 | 社団法人日本薬学会 | |||||
資源タイプ(DSpace) | ||||||
内容記述タイプ | Other | |||||
内容記述 | Article |