Role of Cilia in Renal Fibrosis
Role of Cilia in Renal Fibrosis
批准号:
10153778
负责人:
ZHAOXIA SUN
金额:
$37.69万
依托单位:
依托单位国家:
美国
项目类别:
财政年份:
2017
资助国家:
美国
项目状态:
已结题
起止时间:
2017-09-15 至 2023-05-31
关键词:
AffectBackCell surfaceCellsCiliaCommunicationComplexCystCystic kidneyDefectDiseaseDisease ProgressionDistalEpithelialEpithelial CellsErinaceidaeFeedsFibroblastsFibrosisGenesKidneyKnockout MiceLeadLigandsMental RetardationModelingMonomeric GTP-Binding ProteinsMusMutant Strains MiceMyofibroblastNephronophthisisNephronsObesityOrganellesPathway interactionsPatternPhenotypePolycystic Kidney DiseasesRetinal DegenerationRoleSignal PathwaySignal TransductionSmooth Muscle Actin Staining MethodTestingTissuesbasecell typeciliopathycilium biogenesishuman diseaseinsightinterstitialinterstitial cellkidney fibrosismutantparacrinerenal epitheliumresponsesmoothened signaling pathway
中文摘要
点击翻译按钮获取中文摘要
英文摘要
Summary
The cilium is a widely distributed cell surface organelle that functions as a signaling hub for the vertebrate cell.
Ciliary defects leads to a wide range of human diseases collectively referred to as ciliopathies. Multiple
ciliopathies, including nephronophthisis, show renal fibrosis. However, the role of cilia in renal fibrosis has not
been studied extensively. In this project, we use Arl13b, a gene essential for cilia biogenesis, as an entry point
to dissect the role of cilia in renal fibrosis. We generated conditional Arl13b knockout mice. Our results show
that deletion of Arl13b in renal epithelial cells leads to renal fibrosis and cysts. Moreover, multiple signaling
pathways, including the Wnt, HH and Hippo pathway, are mis-regulated in the mutant kidney. This project
focuses on dissecting tissue specific functions of cilia and communications between different cell types in renal
fibrosis through both candidate and unbiased approaches. Aim 1 is focused on signaling in renal epithelial cells
regulated by ciliary defects and the relationship between different pathways. Aim 2 studies targets in interstitial
cells that are affected by ciliary defects and the functional significance of interstitial cilia. Through this study, we
will elucidate how ciliary defects in epithelial cells triggers a signaling cascade that eventually lead to renal
fibrosis and whether and how cilia in interstitial cells modify this response. These results not only will provide
insight to the mechanism of renal fibrosis in ciliopathies, but will also be informative to fibrosis in general.
期刊论文(0)
专著(0)
科研奖励(0)
会议论文
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批准号:10930194
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批准号:10251032
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资助金额:$38.34万
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财政年份:2018
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负责人:ZHAOXIA SUN
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依托单位:
Genetic Analysis of Organ Patterning Defects in Ciliopathies
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财政年份:2018
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负责人:ZHAOXIA SUN
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Genetic Analysis of Organ Patterning Defects in Ciliopathies
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批准号:10477030
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项目类别:
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资助金额:$38.34万
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财政年份:2018
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负责人:ZHAOXIA SUN
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依托单位:
NPHP2 in ciliary function, renal fibrosis and cyst formation
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项目类别:
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资助金额:$58.7万
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财政年份:2017
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负责人:ZHAOXIA SUN
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Investigate kidney cyst formation and a cilia-mediated signaling network
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负责人:ZHAOXIA SUN
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依托单位:
Investigate kidney cyst formation and a cilia-mediated signaling network
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项目类别:
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负责人:ZHAOXIA SUN
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Investigate kidney cyst formation and a cilia-mediated signaling network
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项目类别:
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财政年份:2012
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负责人:ZHAOXIA SUN
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依托单位:
Sco, A Zebrafish Model Links Cilia and Kidney Cysts
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批准号:7069661
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项目类别:
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资助金额:$37.52万
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财政年份:2005
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负责人:ZHAOXIA SUN
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依托单位:
Sco, A Zebrafish Model Links Cilia and Kidney Cysts
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批准号:7617568
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项目类别:
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资助金额:$35.7万
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财政年份:2005
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负责人:ZHAOXIA SUN
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依托单位:
Sco, A Zebrafish Model Links Cilia and Kidney Cysts
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批准号:7242605
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项目类别:
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资助金额:$36.43万
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财政年份:2005
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负责人:ZHAOXIA SUN
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依托单位:
Sco, A Zebrafish Model Links Cilia and Kidney Cysts
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批准号:7421079
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项目类别:
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资助金额:$35.7万
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财政年份:2005
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负责人:ZHAOXIA SUN
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依托单位:
GENETIC ANALYSIS /EARLY DEVELOPMENT /DISEASES IN ZEBRAFI
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批准号:7070253
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项目类别:
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资助金额:$16.35万
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财政年份:2005
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负责人:ZHAOXIA SUN
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依托单位:
Sco, A Zebrafish Model Links Cilia and Kidney Cysts
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批准号:6966729
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项目类别:
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资助金额:$38.42万
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财政年份:2005
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负责人:ZHAOXIA SUN
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依托单位:
GENETIC ANALYSIS /EARLY DEVELOPMENT /DISEASES IN ZEBRAFI
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批准号:7311600
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项目类别:
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资助金额:$16.35万
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财政年份:--
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负责人:ZHAOXIA SUN
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依托单位:
GENETIC ANALYSIS OF EARLY DEVELOPMENT AND DISEASES IN ZEBRAFISH
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项目类别:
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资助金额:$17.48万
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财政年份:--
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负责人:ZHAOXIA SUN
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依托单位:
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资助金额:$17.48万
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财政年份:--
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