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論文
- タイトル
- タイトル（英）
- Establishment of immortalized Schwann cells from Fabry mice and their low uptake of recombinant alpha-galactosidase.
- 参照URL
- https://researchmap.jp/youichi01/published_papers/46293621
- 著者
- 著者（英）
- Ikuo Kawashima,Kazuhiko Watabe,Youichi Tajima,Tomoko Fukushige,Tamotsu Kanzaki,Takuro Kanekura,Kanako Sugawara,Naho Ohyanagi,Toshihiro Suzuki,Tadayasu Togawa,Hitoshi Sakuraba
- 担当区分
- 概要
- 概要（英）
- Peripheral neuropathy is one of the important manifestations of Fabry disease. Enzyme replacement therapy with presently available recombinant alpha-galactosidases does not always improve the Fabry neuropathy. But the reason has not been determined yet. We established a Schwann cell line from Fabry mice, characterized it, and then examined the uptake of alpha-galactosidase by cells and its effect on the degradation of accumulated substrate. The cells exhibited a distinct Schwann cell morphology and biochemical phenotype (alpha-Galactosidase activity was deficient, and numerous cytoplasmic inclusion bodies were present in the cells). A recombinant alpha-galactosidase added to the culture medium was incorporated into the cultured Fabry Schwann cells dose dependently. But the increase in cell-associated enzyme activity was less than that in the cases of human and mouse Fabry fibroblasts. The administration of a high dose of the enzyme improved the pathological changes in cells, although a low dose of it did not. Cellular uptake of the enzyme was strongly inhibited in the presence of mannose 6-phosphate. This suggests that the enzyme is incorporated via cation-independent mannose 6-phosphate receptors in Schwann cells. The low expression of cation-independent mannose 6-phosphate receptors in Schwann cells must be one of the reasons their uptake of the present enzymes was low. The administration of a high dose of the enzyme or the development of an enzyme containing many mannose 6-phosphate residues is required to improve Fabry neuropathy.
- 出版者・発行元
- 出版者・発行元（英）
- 誌名
- 誌名（英）
- Journal of human genetics
- 巻
- 52
- 号
- 12
- 開始ページ
- 1018
- 終了ページ
- 1025
- 出版年月
- 2007年
- 査読の有無
- 招待の有無
- 掲載種別
- 研究論文（学術雑誌）
- ISSN
- DOI URL
- https://doi.org/10.1007/s10038-007-0210-x
- 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)