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Title: Skeletal analysis of the long bone abnormality (lbab/lbab) mouse, a novel chondrodysplastic C-type natriuretic peptide mutant.
Authors: Kondo, Eri
Yasoda, Akihiro  kyouindb  KAKEN_id
Tsuji, Takehito
Fujii, Toshihito  kyouindb  KAKEN_id
Miura, Masako
Kanamoto, Naotestu
Tamura, Naohisa
Arai, Hiroshi
Kunieda, Tetsuo
Nakao, Kazuwa
Author's alias: 八十田, 明宏
Keywords: C-type natriuretic peptide
Long bone abnormality (lbab)
Endochondral bone growth
Organ culture
Issue Date: Apr-2012
Publisher: Springer Science+Business Media, LLC
Journal title: Calcified tissue international
Volume: 90
Issue: 4
Start page: 307
End page: 318
Abstract: 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.
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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.
DOI(Published Version): 10.1007/s00223-011-9567-0
PubMed ID: 22271248
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