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胰腺发育与疾病中的初级纤毛

Primary cilia in pancreatic development and disease.

作者信息

Lodh Sukanya, O'Hare Elizabeth A, Zaghloul Norann A

机构信息

Division of Endocrinology, Diabetes and Nutrition, Department of Medicine, University of Maryland School of Medicine, Baltimore, Maryland.

出版信息

Birth Defects Res C Embryo Today. 2014 Jun;102(2):139-58. doi: 10.1002/bdrc.21063. Epub 2014 May 26.

Abstract

Primary cilia and their anchoring basal bodies are important regulators of a growing list of signaling pathways. Consequently, dysfunction in proteins associated with these structures results in perturbation of the development and function of a spectrum of tissue and cell types. Here, we review the role of cilia in mediating the development and function of the pancreas. We focus on ciliary regulation of major pathways involved in pancreatic development, including Shh, Wnt, TGF-β, Notch, and fibroblast growth factor. We also discuss pancreatic phenotypes associated with ciliary dysfunction, including pancreatic cysts and defects in glucose homeostasis, and explore the potential role of cilia in such defects.

摘要

初级纤毛及其锚定基体是越来越多信号通路的重要调节因子。因此,与这些结构相关的蛋白质功能障碍会导致一系列组织和细胞类型的发育和功能受到干扰。在此,我们综述纤毛在介导胰腺发育和功能中的作用。我们重点关注参与胰腺发育的主要信号通路的纤毛调节,包括 Sonic Hedgehog(Shh)、Wnt、转化生长因子-β(TGF-β)、Notch 和成纤维细胞生长因子。我们还讨论了与纤毛功能障碍相关的胰腺表型,包括胰腺囊肿和葡萄糖稳态缺陷,并探讨了纤毛在这些缺陷中的潜在作用。

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