Intestinal epithelial cell polarity defects in disease: lessons from microvillus inclusion disease

Disease Models & Mechanisms

Kerstin Schneeberger¹  Edward E. S. Nieuwenhuis¹  Sabine Middendorp¹  Sabrina Roth¹ 

[1] Division of Paediatrics, Department of Paediatric Gastroenterology, Wilhelmina Children's Hospital, 3584 CT, Utrecht, The Netherlands;

关键词: Intestine;    Polarity;    Epithelial cells;    Microvillus inclusion disease;   

DOI  :  10.1242/dmm.031088

来源: DOAJ

【 摘 要 】

The intestinal epithelium is a highly organized tissue. The establishment of epithelial cell polarity, with distinct apical and basolateral plasma membrane domains, is pivotal for both barrier formation and for the uptake and vectorial transport of nutrients. The establishment of cell polarity requires a specialized subcellular machinery to transport and recycle proteins to their appropriate location. In order to understand and treat polarity-associated diseases, it is necessary to understand epithelial cell-specific trafficking mechanisms. In this Review, we focus on cell polarity in the adult mammalian intestine. We discuss how intestinal epithelial polarity is established and maintained, and how disturbances in the trafficking machinery can lead to a polarity-associated disorder, microvillus inclusion disease (MVID). Furthermore, we discuss the recent developments in studying MVID, including the creation of genetically manipulated cell lines, mouse models and intestinal organoids, and their uses in basic and applied research.

【 授权许可】

Unknown