Dystrophin–Glycoprotein Complex in Blood Cells

Abstract

The Dystrophin-Associated Protein Complex (DAPC), known as the DystrophinGlycoprotein Complex (DGC), comprises an array of glycoproteins that are essential for the normal function of striated muscle, in which they were irst described, and for many other tissues, including blood. Understanding the role that these molecules play in muscle function has increased over the last decade, and some of the knowledge derived can be applied to other biological systems. However, there is no doubt that to date, some progress has been achieved in blood cells. Multiple interactions have been described among the proteins comprising the DGC, it is now well established that the DGC possesses a crucial role for numerous signaling pathways, recruiting and regulating various signaling proteins into a macromolecular complex. The aim of this chapter is to summarize the current state of knowledge regarding DGC processing and assembly, mainly in muscle tissue and in blood cells, with a primary focus on the dystroglycan heterodimer and associated proteins, including ion channels and membrane lipids. In addition, and due to increasing evidence involving dystroglycan proteins in the pathophysiology of solid tissue cancer, Duchenne muscular dystrophy, and leukemia, current information on these topics will be included.