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(American Journal of Pathology. 2007;170:609-619.)
© 2007 American Society for Investigative Pathology
DOI: 10.2353/ajpath.2007.060686

{alpha}7ß1 Integrin Does Not Alleviate Disease in a Mouse Model of Limb Girdle Muscular Dystrophy Type 2F

Derek J. Milner and Stephen J. Kaufman

From the Department of Cell and Developmental Biology, University of Illinois, Urbana, Illinois

Transgenic expression of the {alpha}7ß1 integrin in the dystrophic mdx/utr–/– mouse decreases development of muscular dystrophy and enhances longevity. To explore the possibility that elevating {alpha}7ß1 integrin expression could also ameliorate different forms of muscular dystrophy, we used transgenic technology to enhance integrin expression in mice lacking {delta}-sarcoglycan ({delta} sgc), a mouse model for human limb girdle muscular dystrophy type 2F. Unlike {alpha}7 transgenic mdx/utr–/– mice, enhanced {alpha}7ß1 integrin expression in the {delta} sgc-null mouse did not alleviate muscular dystrophy in these animals. Expression of the transgene in the {delta} sgc-null did not alleviate dystrophic histopathology, nor did it decrease cardiomyopathy or restore exercise tolerance. One hallmark of integrin-mediated alleviation of muscular dystrophy in the mdx/utr–/– background is the restoration of myotendinous junction integrity. As assessed by atomic force microscopy, myotendinous junctions from normal and {delta} sgc-null mice were indistinguishable, thus suggesting the important influence of myotendinous junction integrity on the severity of muscular dystrophy and providing a possible explanation for the inability of enhanced integrin expression to alleviate dystrophy in the {delta} sgc-null mouse. These results suggest that distinct mechanisms underlie the development of the diseases that arise from deficiencies in dystrophin and sarcoglycan.






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Copyright © 2007 by the American Society for Investigative Pathology.