Dystroglycan: a possible mediator for reducing congenital muscular dystrophy?
Author
Summary, in English
alpha-Dystroglycan is a highly glycosylated peripheral protein forming a complex with the membrane-spanning beta-dystroglycan and establishing a connection between the extracellular matrix and the cytoskeleton. In skeletal muscle, as part of the larger dystrophin-glycoprotein complex, dystroglycan is believed to be essential for maintaining the structural and functional stability of muscle fibers. Recent work highlights the role of abnormal dystroglycan glycosylation at the basis of glycosyltransferase-deficient congenital muscular dystrophies. Notably, modulation of glycosyltransferase activity can restore alpha-dystroglycan receptor function in these disorders. Moreover, transgenic approaches favoring the interaction between dystroglycan and the extracellular matrix molecules also represent an innovative way to restore skeletal muscle structure. These pioneering approaches might comprise an important first step towards the design of gene-transfer-based strategies for the rescue of congenital muscular dystrophies involving dystroglycan.
Department/s
Publishing year
2007
Language
English
Pages
262-268
Publication/Series
Trends in Biotechnology
Volume
25
Issue
6
Document type
Journal article review
Publisher
Elsevier
Topic
- Cell and Molecular Biology
Status
Published
Research group
- Muscle Biology
ISBN/ISSN/Other
- ISSN: 0167-7799