Assembly of the Dystrophin-associated Protein Complex Does Not Require the Dystrophin COOH-terminal Domain

doi:10.1083/jcb.150.6.1399

Published online 18 September 2000. doi:10.1083/jcb.150.6.1399

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© The Rockefeller University Press, 0021-9525/2000/9/1399/ $5.00
The Journal of Cell Biology, Volume 150, Number 6, September 18, 2000 1399-1410

Original Article

Assembly of the Dystrophin-associated Protein Complex Does Not Require the Dystrophin COOH-terminal Domain

Gregory E. Crawford^a, John A. Faulkner^d, Rachelle H. Crosbie^e, Kevin P. Campbell^e, Stanley C. Froehner^f, and Jeffrey S. Chamberlain^a,b,c
^a Program in Cellular and Molecular Biology, University of Michigan, Ann Arbor, Michigan 48109-0618
^b Department of Human Genetics, University of Michigan, Ann Arbor, Michigan 48109-0618
^c Center for Gene Therapy, University of Michigan, Ann Arbor, Michigan 48109-0618
^d Department of Physiology, University of Michigan, Ann Arbor, Michigan 48109-0618
^e Howard Hughes Medical Institute, Department of Physiology and Biophysics, Department of Neurology, University of Iowa College of Medicine, Iowa City, Iowa 52242
^f Department of Cell and Molecular Physiology, University of North Carolina at Chapel Hill, Chapel Hill, North Carolina 27599-7545

Correspondence to: Jeffrey S. Chamberlain, Department of Human Genetics, University of Michigan, Ann Arbor, MI 48109-0618. Tel:(734) 764-4297 Fax:(734) 764-6898

Dystrophin is a multidomain protein that links the actin cytoskeletonto laminin in the extracellular matrix through the dystrophinassociated protein (DAP) complex. The COOH-terminal domain ofdystrophin binds to two components of the DAP complex, syntrophinand dystrobrevin. To understand the role of syntrophin and dystrobrevin,we previously generated a series of transgenic mouse lines expressingdystrophins with deletions throughout the COOH-terminal domain.Each of these mice had normal muscle function and displayednormal localization of syntrophin and dystrobrevin. Since syntrophinand dystrobrevin bind to each other as well as to dystrophin,we have now generated a transgenic mouse deleted for the entiredystrophin COOH-terminal domain. Unexpectedly, this truncateddystrophin supported normal muscle function and assembly ofthe DAP complex. These results demonstrate that syntrophin anddystrobrevin functionally associate with the DAP complex inthe absence of a direct link to dystrophin. We also observedthat the DAP complexes in these different transgenic mouse strainswere not identical. Instead, the DAP complexes contained varyingratios of syntrophin and dystrobrevin isoforms. These resultssuggest that alternative splicing of the dystrophin gene, whichnaturally generates COOH-terminal deletions in dystrophin, mayfunction to regulate the isoform composition of the DAP complex.

Key Words: dystrophin, muscular dystrophy, syntrophin, dystrobrevin, mdxmice

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