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Dystroglycan: a possible mediator for reducing congenital muscular dystrophy?

Paper ID Volume ID Publish Year Pages File Format Full-Text
37759 45407 2007 7 PDF Available
Title
Dystroglycan: a possible mediator for reducing congenital muscular dystrophy?
Abstract

α-Dystroglycan is a highly glycosylated peripheral protein forming a complex with the membrane-spanning β-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 α-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.

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Publisher
Database: Elsevier - ScienceDirect
Journal: - Volume 25, Issue 6, June 2007, Pages 262–268
Authors
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Subjects
Physical Sciences and Engineering Chemical Engineering Bioengineering
Get Full-Text Now
Don't Miss Today's Special Offer
Price was $35.95
You save - $31
Price after discount Only $4.95
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