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Biochemical Analysis of Elastic Fiber Formation with a Frameshift-Mutated Tropoelastin (fmTE) at the C-Terminus of Tropoelastin
https://stella.repo.nii.ac.jp/records/442
https://stella.repo.nii.ac.jp/records/442349afefd-4048-47b0-84e1-6704e3fa11e8
名前 / ファイル | ライセンス | アクション |
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JHealthSci_52-3_p259.pdf (2.4 MB)
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Item type | 学術雑誌論文 / Journal Article(1) | |||||
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公開日 | 2016-02-01 | |||||
タイトル | ||||||
言語 | en | |||||
タイトル | Biochemical Analysis of Elastic Fiber Formation with a Frameshift-Mutated Tropoelastin (fmTE) at the C-Terminus of Tropoelastin | |||||
言語 | ||||||
言語 | eng | |||||
キーワード | ||||||
言語 | en | |||||
主題Scheme | Other | |||||
主題 | tropoelastin | |||||
キーワード | ||||||
言語 | en | |||||
主題Scheme | Other | |||||
主題 | molecular interaction | |||||
キーワード | ||||||
言語 | en | |||||
主題Scheme | Other | |||||
主題 | cross-linked amino acid | |||||
キーワード | ||||||
言語 | en | |||||
主題Scheme | Other | |||||
主題 | elastic fiber formation | |||||
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 |
Sato, Fumiaki
× Sato, Fumiaki× Wachi, Hiroshi× Starcher, Barry C.× Seyama, Yoshiyuki |
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抄録 | ||||||
内容記述タイプ | Abstract | |||||
内容記述 | Elastic fibers play an important role in characteristic elastic properties of tissues such as skin, lungs, ligaments, and blood vessels. Many of the disorders related to elastic fibers are due to errors in assembly, yet the basic mechanisms of this process remain unclear. Cutis laxa, which is caused by an elastin gene mutation, includes a rare and heterogeneous group of the disorders characterized by lax, inelastic skin. To investigate whether elastic fiber assembly insufficiency is a possible factor in cutis laxa, we compared normal tropoelastin (nTE) and frameshift-mutated tropoelastin (fmTE) from a patient with autosomal dominant cutis laxa using an in vitro elastic fiber assembly model, in which purified recombinant tropoelastin was added to the culture medium. Assembly was evaluated by immunofluorescence staining, the quantitative analysis of cross-linked amino acids, and semi-quantitative analysis of matrix-associated tropoelastin. Immunofluorescence microscopy indicated an approximate 50% decrease in the deposition of fmTE in the extracelluar matrix, in contrast to that of nTE. The amount of cross-linked amino acids unique to mature insoluble elastin in the fmTE assembly was also decreased by approximately 20%. In addition, we clarified that the molecular interaction between fmTE and the amino-terminal domain of fibrillin-1 or full-length fibulin-5 was significantly decreased. Our results suggest that the defect of fmTE fiber assembly is due, at least in part, to decreased molecular interactions between tropoelastin and the microfibrillar components fibulin-5 and fibrillin. | |||||
書誌情報 |
Journal of health science 巻 52, 号 3, p. 259-267, 発行日 2006-06-01 |
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出版者 | ||||||
出版者 | 日本薬学会 | |||||
ISSN | ||||||
収録物識別子タイプ | ISSN | |||||
収録物識別子 | 13449702 | |||||
書誌レコードID | ||||||
収録物識別子タイプ | NCID | |||||
収録物識別子 | AA11316464 | |||||
論文ID(NAID) | ||||||
関連タイプ | isIdenticalTo | |||||
識別子タイプ | NAID | |||||
関連識別子 | 110006251374 | |||||
DOI | ||||||
関連タイプ | isIdenticalTo | |||||
識別子タイプ | DOI | |||||
関連識別子 | info:10.1248/jhs.52.259 | |||||
権利 | ||||||
権利情報 | 日本薬学会 | |||||
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 |