『徳島大学 教育・研究者情報データベース (EDB)』---[学外] /
ID: Pass:

登録内容 (EID=205774)

EID=205774EID:205774, Map:0, LastModified:2012年9月14日(金) 15:29:14, Operator:[三木 ちひろ], Avail:TRUE, Censor:承認済, Owner:[伊藤 孝司], Read:継承, Write:継承, Delete:継承.
○種別 (必須): □学術論文 (審査論文) [継承]
○言語 (必須): □英語 [継承]
○招待 (推奨):
○審査 (推奨):
○カテゴリ (推奨):
○共著種別 (推奨):
○学究種別 (推奨):
○組織 (推奨):
○著者 (必須): 1. (英) Tajima Youichi (日) (読)
○役割 (任意):
○貢献度 (任意):
○学籍番号 (推奨):
[継承]
2. (英) Kawashima Ikuo (日) (読)
○役割 (任意):
○貢献度 (任意):
○学籍番号 (推奨):
[継承]
3. (英) Tsukimura Takahiro (日) (読)
○役割 (任意):
○貢献度 (任意):
○学籍番号 (推奨):
[継承]
4. (英) Sugawara Kanako (日) (読)
○役割 (任意):
○貢献度 (任意):
○学籍番号 (推奨):
[継承]
5. (英) Kuroda Mayuko (日) (読)
○役割 (任意):
○貢献度 (任意):
○学籍番号 (推奨):
[継承]
6. (英) Suzuki Toshihiro (日) (読)
○役割 (任意):
○貢献度 (任意):
○学籍番号 (推奨):
[継承]
7. (英) Togawa Tadayasu (日) (読)
○役割 (任意):
○貢献度 (任意):
○学籍番号 (推奨):
[継承]
8. (英) Chiba Yasunori (日) (読)
○役割 (任意):
○貢献度 (任意):
○学籍番号 (推奨):
[継承]
9. (英) Jigami Yoshifumi (日) (読)
○役割 (任意):
○貢献度 (任意):
○学籍番号 (推奨):
[継承]
10. (英) Ohno Kazuki (日) (読)
○役割 (任意):
○貢献度 (任意):
○学籍番号 (推奨):
[継承]
11. (英) Fukushige Tomoko (日) (読)
○役割 (任意):
○貢献度 (任意):
○学籍番号 (推奨):
[継承]
12. (英) Kanekura Takuro (日) (読)
○役割 (任意):
○貢献度 (任意):
○学籍番号 (推奨):
[継承]
13.伊藤 孝司
○役割 (任意):
○貢献度 (任意):
○学籍番号 (推奨):
[継承]
14. (英) Ohashi Toya (日) (読)
○役割 (任意):
○貢献度 (任意):
○学籍番号 (推奨):
[継承]
15. (英) Sakuraba Hitoshi (日) (読)
○役割 (任意):
○貢献度 (任意):
○学籍番号 (推奨):
[継承]
○題名 (必須): □(英) Use of a Modified alpha-N-Acetylgalactosaminidase in the Development of Enzyme Replacement Therapy for Fabry Disease  (日)    [継承]
○副題 (任意):
○要約 (任意): □(英) A modified alpha-N-acetylgalactosaminidase (NAGA) with alpha-galactosidase A (GLA)-like substrate specificity was designed on the basis of structural studies and was produced in Chinese hamster ovary cells. The enzyme acquired the ability to catalyze the degradation of 4-methylumbelliferyl-alpha-D-galactopyranoside. It retained the original NAGA's stability in plasma and N-glycans containing many mannose 6-phosphate (M6P) residues, which are advantageous for uptake by cells via M6P receptors. There was no immunological cross-reactivity between the modified NAGA and GLA, and the modified NAGA did not react to serum from a patient with Fabry disease recurrently treated with a recombinant GLA. The enzyme cleaved globotriaosylceramide (Gb3) accumulated in cultured fibroblasts from a patient with Fabry disease. Furthermore, like recombinant GLA proteins presently used for enzyme replacement therapy (ERT) for Fabry disease, the enzyme intravenously injected into Fabry model mice prevented Gb3 storage in the liver, kidneys, and heart and improved the pathological changes in these organs. Because this modified NAGA is hardly expected to cause an allergic reaction in Fabry disease patients, it is highly promising as a new and safe enzyme for ERT for Fabry disease.  (日)    [継承]
○キーワード (推奨): 1. (英) Amino Acid Substitution (日) (読) [継承]
2. (英) Animals (日) (読) [継承]
3. (英) Binding Sites (日) (読) [継承]
4. (英) CHO Cells (日) (読) [継承]
5. (英) Catalysis (日) (読) [継承]
6. (英) Cells, Cultured (日) (読) [継承]
7. (英) Cricetinae (日) (読) [継承]
8. (英) Cricetulus (日) (読) [継承]
9. (英) Culture Media, Conditioned (日) (読) [継承]
10. (英) DNA, Complementary (日) (読) [継承]
11. (英) Disease Models, Animal (日) (読) [継承]
12. (英) Drug Stability (日) (読) [継承]
13. (英) Enzyme Replacement Therapy (日) (読) [継承]
14. (英) Fabry Disease (日) (読) [継承]
15. (英) Fibroblasts (日) (読) [継承]
16. (英) Fluorescent Dyes (日) (読) [継承]
17. (英) Galactosides (日) (読) [継承]
18. (英) Genetic Vectors (日) (読) [継承]
19. (英) Humans (日) (読) [継承]
20. (英) Hydrogen-Ion Concentration (日) (読) [継承]
21. (英) Hymecromone (日) (読) [継承]
22.免疫組織化学 (immunohistochemistry) [継承]
23. (英) Kidney (日) (読) [継承]
24. (英) Liver (日) (読) [継承]
25. (英) Mice (日) (読) [継承]
26. (英) Mice, Knockout (日) (読) [継承]
27. (英) Models, Molecular (日) (読) [継承]
28.分子量 (molecular weight) [継承]
29.心筋 (myocardium) [継承]
30. (英) Recombinant Proteins (日) (読) [継承]
31. (英) Retroviridae (日) (読) [継承]
32. (英) Transfection (日) (読) [継承]
33. (英) Trihexosylceramides (日) (読) [継承]
34. (英) alpha-N-Acetylgalactosaminidase (日) (読) [継承]
○発行所 (推奨):
○誌名 (必須): □American Journal of Human Genetics ([The American Society of Human Genetics])
(pISSN: 0002-9297, eISSN: 1537-6605)

○ISSN (任意): □1537-6605
ISSN: 0002-9297 (pISSN: 0002-9297, eISSN: 1537-6605)
Title: American journal of human genetics
Title(ISO): Am J Hum Genet
Publisher: Cell Press
 (NLM Catalog)   (Scopus)   (CrossRef)  (Scopus information is found. [need login])
[継承]
[継承]
○巻 (必須): □85 [継承]
○号 (必須): □5 [継承]
○頁 (必須): □569 580 [継承]
○都市 (任意):
○年月日 (必須): □西暦 2009年 11月 初日 (平成 21年 11月 初日) [継承]
○URL (任意):
○DOI (任意): □10.1016/j.ajhg.2009.09.016    (→Scopusで検索) [継承]
○PMID (任意): □19853240    (→Scopusで検索) [継承]
○CRID (任意):
○Scopus (任意):
○researchmap (任意):
○評価値 (任意):
○被引用数 (任意):
○指導教員 (推奨):
○備考 (任意): 1.(英) Article.Affiliation: Department of Clinical Genetics, Meiji Pharmaceutical University, Tokyo 204-8588, Japan.  (日)    [継承]
2.(英) Article.PublicationTypeList.PublicationType: Journal Article  (日)    [継承]
3.(英) Article.PublicationTypeList.PublicationType: Research Support, Non-U.S. Gov't  (日)    [継承]
4.(英) OtherID: PMC2775840  (日)    [継承]

標準的な表示

和文冊子 ● Youichi Tajima, Ikuo Kawashima, Takahiro Tsukimura, Kanako Sugawara, Mayuko Kuroda, Toshihiro Suzuki, Tadayasu Togawa, Yasunori Chiba, Yoshifumi Jigami, Kazuki Ohno, Tomoko Fukushige, Takuro Kanekura, Kouji Itou, Toya Ohashi and Hitoshi Sakuraba : Use of a Modified alpha-N-Acetylgalactosaminidase in the Development of Enzyme Replacement Therapy for Fabry Disease, American Journal of Human Genetics, 85, 5, 569-580, 2009.
欧文冊子 ● Youichi Tajima, Ikuo Kawashima, Takahiro Tsukimura, Kanako Sugawara, Mayuko Kuroda, Toshihiro Suzuki, Tadayasu Togawa, Yasunori Chiba, Yoshifumi Jigami, Kazuki Ohno, Tomoko Fukushige, Takuro Kanekura, Kouji Itou, Toya Ohashi and Hitoshi Sakuraba : Use of a Modified alpha-N-Acetylgalactosaminidase in the Development of Enzyme Replacement Therapy for Fabry Disease, American Journal of Human Genetics, 85, 5, 569-580, 2009.

関連情報

Number of session users = 5, LA = 0.76, Max(EID) = 444248, Max(EOID) = 1178123.