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BBB seminar: Ulrike Mayer

Genetic analysis of α7β1 integrin function in skeletal muscle


Ulrike Mayer
Wellcome Trust Centre for Cell-Matrix Research, University of Manchester, Faculty of Life Sciences and School of Biological Sciences, University of East Anglia, Norwich, UK

Skeletal muscle accounts for 40% of the human body and is the major consumer of body fuels. Contraction within muscle fibers by ATP consumption generates the force required for our body movement, but the coordinated transmission of this force through the muscle cell membrane to the surrounding basement membrane and through the tendon and into the bone is as important as the contraction itself. Cell adhesion molecules are thought to provide the link between the muscle cell interior and the environment, both as a means of transferring signals from the outside into the cell as well as maintaining the structural integrity of skeletal muscle upon repeated contraction.

Both the laminin-binding integrin α7β1 and the dystrophin glycoprotein complex (DGC) have critical roles in the maintenance of muscle integrity via providing mechanical links between muscle fibers and the basement membrane. Absence of either dystrophin or the integrin α7 subunit results in muscular dystrophies. To clarify the inter-relationship between α7β1 integrin and dystrophin, we have generated integrin α7/dystrophin double-mutant (DKO) mice. The DKO mice develop a severe muscular dystrophy, which becomes evident in early postnatal life and leads to premature death post puberty. The integrin α7/dystrophin-deficient mouse model therefore closely resembles the phenotype of Duchenne muscular dystrophy and suggests an essential role for α7β1 integrin in the protection of dystrophin-deficient myofibers against early necrosis.

Host: Donald Gullberg, Department of Biomedicine

The research areas of Dr. Ulrike Mayer, Chair in Cell Biology at the University of East Anglia in Norwich, UK, are in cell adhesion and in tissue pathologies and regeneration. The work of her group mainly focuses on the analysis of the basement membrane proteins laminin and nidogen in vivo and the receptor-mediated interaction of basement membranes with cells by gene targeting approaches. The combination of the analysis of the supramolecular organisation of basement membranes and the function of their cellular interactions will lead to a better understanding of a variety of biological processes.