※ をクリックすると外部の論文のサイトが開きます。
論文
- タイトル
- タイトル（英）
- Uptake of a recombinant human alpha-L-iduronidase (laronidase) by cultured fibroblasts and osteoblasts.
- 参照URL
- https://researchmap.jp/youichi01/published_papers/46293619
- 著者
- 著者（英）
- Takahiro Tsukimura,Youichi Tajima,Ikuo Kawashima,Tomoko Fukushige,Tamotsu Kanzaki,Takuro Kanekura,Masahiko Ikekita,Kanako Sugawara,Toshihiro Suzuki,Tadayasu Togawa,Hitoshi Sakuraba
- 担当区分
- 概要
- 概要（英）
- To examine the uptake of a recombinant human alpha-L-iduronidase (laronidase) by cultured fibroblasts from a patient with mucopolysaccharidosis I (MPS I) and its effect on the cleavage of accumulated substrates, we performed enzymological, Western blotting, immunocytochemical and morphological studies. Laronidase was incorporated into the MPS I cells dose-dependently mainly via mannose 6-phosphate (M6P) receptors. Then the incorporated enzyme was transported to lysosomes and processed to the mature form, the pathological changes of the cells being improved. Furthermore, we compared the uptake of laronidase by cultured mouse osteoblasts with that by cultured mouse fibroblasts. The enzyme was incorporated into the cultured mouse osteoblasts mainly via M6P receptors, although mannose (Man) receptors were partially involved in the uptake of the enzyme, as in the cultured fibroblasts. But the uptake by the former was apparently lower than that by the latter. The administration of a high dose of the enzyme or development of a recombinant alpha-L-iduronidase containing many M6P residues is required for further improvement of enzyme replacement therapy for skeletal disorders caused by MPS I.
- 出版者・発行元
- 出版者・発行元（英）
- 誌名
- 誌名（英）
- Biological & pharmaceutical bulletin
- 巻
- 31
- 号
- 9
- 開始ページ
- 1691
- 終了ページ
- 5
- 出版年月
- 2008年9月
- 査読の有無
- 招待の有無
- 掲載種別
- 研究論文（学術雑誌）
- ISSN
- 0918-6158
- DOI URL
- 共同研究・競争的資金等の研究課題
研究者
田島 陽一
(タジマ ヨウイチ)