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多囊肾病中的Wnt信号通路

Wnt signaling in polycystic kidney disease.

作者信息

Benzing Thomas, Simons Matias, Walz Gerd

机构信息

Renal Division, University Hospital Freiburg, Freiburg, Germany.

出版信息

J Am Soc Nephrol. 2007 May;18(5):1389-98. doi: 10.1681/ASN.2006121355. Epub 2007 Apr 11.

DOI:10.1681/ASN.2006121355
PMID:17429050
Abstract

Wnt signaling cascades activate morphogenetic programs that range from cell migration and proliferation to cell fate determination and stem cell renewal. These pathways enable cells to translate environmental cues into the complex cellular programs that are needed to organize tissues and build organs. Wnt signaling is essential for renal development; however, the specific molecular underpinnings involved are poorly understood. Recent research has revealed an unexpected intersection between Wnt signaling and polycystic kidney disease. Some polycystic kidney disease proteins, such as Inversin and Bardet-Biedl syndrome family members, were found to use components of the Wnt signaling cascade to orient cells along a secondary polarity axis within the plane of the epithelium. These spatial cues may be needed to position nascent tubules with a defined geometry.

摘要

Wnt信号级联激活从细胞迁移、增殖到细胞命运决定和干细胞更新等一系列形态发生程序。这些信号通路使细胞能够将环境信号转化为组织构建和器官形成所需的复杂细胞程序。Wnt信号对于肾脏发育至关重要;然而,其中具体涉及的分子机制却知之甚少。最近的研究揭示了Wnt信号与多囊肾病之间意想不到的关联。一些多囊肾病相关蛋白,如Inversin和巴德-比德尔综合征家族成员,被发现利用Wnt信号级联的组分在上皮平面内沿次级极性轴引导细胞。这些空间线索可能是将新生肾小管定位成特定几何形状所必需的。

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