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副腎白質ジストロフィー : ALDPの構造・機能とミスセンス変異ALDPの細胞内動態(誌上シンポジウム)
http://hdl.handle.net/10110/1750
http://hdl.handle.net/10110/175064c69378-1f69-482e-8b46-fd267abd16c4
名前 / ファイル | ライセンス | アクション |
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yakugakuzasshi07-163.pdf (1.2 MB)
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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 : ALDPの構造・機能とミスセンス変異ALDPの細胞内動態(誌上シンポジウム) | |||||
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 | ALDP (ABCD1) | |||||
キーワード | ||||||
主題Scheme | Other | |||||
主題 | missense mutation | |||||
キーワード | ||||||
主題Scheme | Other | |||||
主題 | targeting | |||||
キーワード | ||||||
主題Scheme | Other | |||||
主題 | degradation | |||||
キーワード | ||||||
主題Scheme | Other | |||||
主題 | proteasome | |||||
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 |
高橋, 則正
× 高橋, 則正× 守田, 雅志× 今中, 常雄 |
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著者別名 | ||||||
姓名 | Takahashi, Norimasa | |||||
著者別名 | ||||||
姓名 | Morita, Masashi | |||||
著者別名 | ||||||
姓名 | Imanaka, Tsuneo | |||||
その他(別言語等)のタイトル | ||||||
その他のタイトル | Adrenoleukodystrophy : Structure and Function of ALDP, and Intracellular Behavior of Mutant ALDP with Naturally Occurring Missense Mutations(Symposium Review) | |||||
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 based on the accumulation of pathgnomonic amounts of saturated very long-chain fatty acid (VLCFA; C>22) in all tissues, including the brain white matter, adrenal glands, and skin fibroblasts, of the patients. The accumulation of VLCFA in ALD is linked to a mutation in the ALD (ABCD1) gene, an ABC sub family D member. The ALD gene product, so-called ALDP (ABCD1), is thought to be involved in the transport of VLCFA or VLCFA-CoA into the peroxisomes. ALDP is a half-sized peroxisomal ABC protein and it has 745 amino acids in humans. ALDP is thought to be synthesized on free polysomes, posttranslationally transported to peroxisomes, and inserted into the membranes. During this process, ALDP interacts with Pex19p, a chaperone-like protein for intracellular trafficking of peroxisomal membrane protein (PMP), the complex targets Pex3p on the peroxisomal membranes, and ALDP is inserted into the membranes. After integration into the membranes, ALDP is thought to form mainly homodimers. Here, we chose nine arbitrary mutations of human ALDP with naturally occurring missense mutations and examined the intracellular behavior of their ALDPs. We found that mutant ALDP (S606L, R617H, and H667D) was degraded together with wild-type ALDP by proteasomes. These results suggest that the complex of mutant and wild-type ALDP is recognized as misfolded proteins and degraded by the protein quality control system associated with proteasomes. Further, we found fragmentation of mutant ALDP (R104C) on peroxisomes and it was not inhibited by proteasomes inhibitors, suggesting that an additional protease (s) is also involved in the quality control of mutant ALDP. In addition, mutation of ALDP (Y174C) suggests that a loop between transmembrane domains 2 and 3 is important for the targeting of ALDP to peroxisomes. | |||||
書誌情報 |
藥學雜誌 : Journal of the Pharmaceutical Society of Japan (薬学雑誌) 巻 127, 号 1, p. 163-172, 発行日 2007-01 |
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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 |