ダウンロード数: 347
このアイテムのファイル:
ファイル | 記述 | サイズ | フォーマット | |
---|---|---|---|---|
s00223-011-9567-0.pdf | 8.19 MB | Adobe PDF | 見る/開く |
タイトル: | Skeletal analysis of the long bone abnormality (lbab/lbab) mouse, a novel chondrodysplastic C-type natriuretic peptide mutant. |
著者: | Kondo, Eri Yasoda, Akihiro Tsuji, Takehito Fujii, Toshihito Miura, Masako Kanamoto, Naotestu Tamura, Naohisa Arai, Hiroshi Kunieda, Tetsuo Nakao, Kazuwa |
著者名の別形: | 近藤, 絵里 八十田, 明宏 |
キーワード: | C-type natriuretic peptide Long bone abnormality (lbab) Chondrodysplasia Endochondral bone growth Organ culture |
発行日: | Apr-2012 |
出版者: | Springer |
誌名: | Calcified tissue international |
巻: | 90 |
号: | 4 |
開始ページ: | 307 |
終了ページ: | 318 |
抄録: | Long bone abnormality (lbab/lbab) is a strain of dwarf mice. Recent studies revealed that the phenotype is caused by a spontaneous mutation in the Nppc gene, which encodes mouse C-type natriuretic peptide (CNP). In this study, we analyzed the chondrodysplastic skeletal phenotype of lbab/lbab mice. At birth, lbab/lbab mice are only slightly shorter than their wild-type littermates. Nevertheless, lbab/lbab mice do not undergo a growth spurt, and their final body and bone lengths are only ~60% of those of wild-type mice. Histological analysis revealed that the growth plate in lbab/lbab mice, especially the hypertrophic chondrocyte layer, was significantly thinner than in wild-type mice. Overexpression of CNP in the cartilage of lbab/lbab mice restored their thinned growth plate, followed by the complete rescue of their impaired endochondral bone growth. Furthermore, the bone volume in lbab/lbab mouse was severely decreased and was recovered by CNP overexpression. On the other hand, the thickness of the growth plate of lbab/+ mice was not different from that of wild-type mice; accordingly, impaired endochondral bone growth was not observed in lbab/+ mice. In organ culture experiments, tibial explants from fetal lbab/lbab mice were significantly shorter than those from lbab/+ mice and elongated by addition of 10(-7) M CNP to the same extent as lbab/+ tibiae treated with the same dose of CNP. These results demonstrate that lbab/lbab is a novel mouse model of chondrodysplasia caused by insufficient CNP action on endochondral ossification. |
著作権等: | This is a post-peer-review, pre-copyedit version of an article published in 'Calcified tissue international'. The final authenticated version is available online at: https://doi.org/10.1007/s00223-011-9567-0. この論文は出版社版でありません。引用の際には出版社版をご確認ご利用ください。 This is not the published version. Please cite only the published version. |
URI: | http://hdl.handle.net/2433/155460 |
DOI(出版社版): | 10.1007/s00223-011-9567-0 |
PubMed ID: | 22271248 |
出現コレクション: | 学術雑誌掲載論文等 |
このリポジトリに保管されているアイテムはすべて著作権により保護されています。