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학술연구/단체지원/교육 등 연구자 활동을 지속하도록 DBpia가 지원하고 있어요.
커뮤니티
연구자들이 자신의 연구와 전문성을 널리 알리고, 새로운 협력의 기회를 만들 수 있는 네트워킹 공간이에요.
논문 기본 정보
- 자료유형
- 학술저널
- 저자정보
- 발행연도
- 2018.3
- 수록면
- 224 - 233 (10page)
- DOI
- 10.14348/molcells.2018.2307
이용수
초록· 키워드
오류제보하기
Primary cilia are solitary, non-motile, axonemal microtubule-based antenna-like organelles that project from the plasma membrane of most mammalian cells and are implicated in transducing hedgehog signals during development. It was recently proposed that aberrant SHH signaling may be implicated in the progression of idiopathic pulmonary fibrosis (IPF). However, the distribution and role of primary cilia in IPF remains unclear. Here, we clearly observed the primary cilia in alveolar epithelial cells, fibroblasts, and endothelial cells of human normal lung tissue. Then, we investigated the distribution of primary cilia in human IPF tissue samples using immu-nofluorescence. Tissues from six IPF cases showed an increase in the number of primary cilia in alveolar cells and fibroblasts. In addition, we observed an increase in ciliogenesis related genes such as IFT20 and IFT88 in IPF. Since major components of the SHH signaling pathway are known to be localized in primary cilia, we quantified the mRNA expression of the SHH signaling components using qRT-PCR in both IPF and control lung. mRNA levels of SHH, the coreceptor SMO, and the transcription factors GLI1 and GLI2 were upregulated in IPF compared with control. Furthermore, the nuclear localization of GLI1 was observed mainly in alveolar epithelia and fibroblasts. In addition, we showed that defective KIF3A-mediated ciliary loss in human type II alveolar epithelial cell lines leads to disruption of SHH signaling. These results indicate that a significant increase in the number of primary cilia in IPF contributes to the upregulation of SHH signals.
목차
등록된 정보가 없습니다.
참고문헌 (36)
참고문헌 신청
Adams, M. / 2008 / Recent advances in the molecular pathology, cell biology and genetics of ciliopathies / J. Med. Genet. 45:257~267
Ashizawa, N. / 1999 / The morphological changes of exocrine pancreas in chronic pancreatitis / Histol. Histopathol. 14:539~552
Bolanos, A.L. / 2012 / Role of Sonic Hedgehog in idiopathic pulmonary fibrosis / Am. J. Physiol. Lung Cell. Mol. Physiol. 303:L978~L990
Cardoso, W.V. / 2006 / Regulation of early lung morphogenesis: questions, facts and controversies / Development 133:1611~1624
Chang, M.Y. / 2006 / Haploinsufficiency of Pkd2 is associated with increased tubular cell proliferation and interstitial fibrosis in two murine Pkd2 models / Nephrol. Dial. Transplant. 21:2078~2084
Ashizawa, N. / 1999 / The morphological changes of exocrine pancreas in chronic pancreatitis / Histol. Histopathol. 14:539~552
Bolanos, A.L. / 2012 / Role of Sonic Hedgehog in idiopathic pulmonary fibrosis / Am. J. Physiol. Lung Cell. Mol. Physiol. 303:L978~L990
Cardoso, W.V. / 2006 / Regulation of early lung morphogenesis: questions, facts and controversies / Development 133:1611~1624
Chang, M.Y. / 2006 / Haploinsufficiency of Pkd2 is associated with increased tubular cell proliferation and interstitial fibrosis in two murine Pkd2 models / Nephrol. Dial. Transplant. 21:2078~2084
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