Abstract
Dystroglycan is an integral membrane protein that has been found in a variety of cell types including muscle, epithelial, and neural cells. Dystroglycan helps to link the extracellular basement membrane, cell membrane and cytoskeleton, thereby contributing to cellular membrane integrity. Dystroglycan can also mediate cell adhesion to the extracellular matrix, stimulate extracellular matrix formation, and mediate signal transduction pathways. Made from a single gene product (DAG1), the dystroglycan protein is post-translationally cleaved to comprise two protein chains, α and β; α dystroglycan undergoes further post-translational modification which results in heavy glycosylation of the dystroglycan molecule. The binding of cells via dystroglycan to extracellular matrix proteins has been shown to be dependent on this glycosylation. Abnormalities of dystroglycan glycosylation have been clearly implicated in muscular dystrophy, neuronal migration disorders and ocular malformations. While its importance in development and in disease is well known, it is only recently that dystroglycan function has been considered in tumor biology. Recent studies have shown that altered dystroglycan expression is a feature of certain cancer cell lines and of some adult primary tumors. These alterations have been implicated in both tumor invasiveness and metastatic potential.
To date, no such investigations have been reported for pediatric cancers. Determination of whether dystroglycan glycosylation and expression factor in pediatric tumorigenesis may provide the insight needed to exploit this molecule as a novel diagnostic and therapeutic target. Various tumor samples have been obtained from the Pediatric Division of the Cooperative Human Tissue Network at Children's Hospital, Columbus, OH. The expression pattern and glycosylation of dystroglycan was analyzed using immunohistochemistry and immunoblotting techniques. The results of the investigation, which is funded by the Samuel J. Roessler Memorial Scholarship, will be presented at the conference.
Article PDF
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Dosunmu, E., Martin, P. & Martin, L. 34 Dystroglycan Expression and Glycosylation in Pediatric Solid Tumors.. Pediatr Res 58, 822 (2005). https://doi.org/10.1203/00006450-200510000-00064
Issue Date:
DOI: https://doi.org/10.1203/00006450-200510000-00064