The dystroglycan complex: from biology to cancer

Abstract

Dystroglycan (DG), a non-integrin adhesion molecule, is a pivotal component of the dystrophin-glycoprotein complex, that is expressed in skeletal muscle and in a wide variety of tissues at the interface between the basement membrane (BM) and the cell membrane. DG has been mainly studied for its role in skeletal muscle cell stability and its alterations in muscular diseases, such as dystrophies. However, accumulating evidence have implicated DG in a variety of other biological functions, such as maturation of post-synaptic elements in the central and peripheral nervous system, early morphogenesis, and infective pathogens targeting. Moreover, DG has been reported to play a role in regulating cytoskeletal organization, cell polarization, and cell growth in epithelial cells. Recent studies also indicate that abnormalities in the expression of DG frequently occur in human cancers and may play a role in both the process of tumor progression and in the maintenance of the malignant phenotype. This paper reviews the available information on the biology of DG, the abnormalities found in human cancers, and the implications of these findings with respect to our understanding of cancer pathogenesis and to the development of novel strategies for a better management of cancer patients.