Dystrophin-related protein

Dystrobrevins are a small family of dystrophin-related proteins encoded by two genes, one of which—Q-dystrobrevin—encodes at least three proteins all expressed in cardiac and skeletal muscle. o-Dystrobrevin is a key component of the DPC, and its loss results in neuromuscular junction defects and muscular dystrophy. It is a cytoplasmic protein indirectly linked with the transmembrane sarcoglycan components via dystrophin and other components of the DPC (Fig. 4) (Blake and Martin-Rendon, 2002). Recently, two novel type IV IF proteins—syncoilin (Newey et al.,... 

Following the discovery of the dystrophin gene, another cDNA was identified that showed considerable homology to that of dystrophin (Love et al., 1989). Initially this protein was referred to as dystrophin-related protein (DRP), but once cloned and sequenced (Tinsley et al., 1992) itwas subsequently renamed utrophin due to a ubiquitous expression pattern compared to that of dystrophin. In muscle cells, utrophin shares a high degree of functional similarity with dystrophin (Glaudepierre et al, 1999 Earnest et al, 1995 Loh et al., 2000 Matsumura et al, 1993 Nguyen et al, 1991 Pons et al., 1994 Raats et al, 2000) and has been proposed as a potential therapeutic replacement for dystrophin in the treatment of DMD (Matsumura et al, 1992 Pearce et al, 1993 Tinsley et al, 1992). 

Earnest, J. P., Santos, G. F., Zuerbig, S., and Fox, J. E. (1995). Dystrophin-related protein in the platelet membrane skeleton. Integrin-induced change in detergent-insolubility and cleavage by calpain in aggregating platelets. J. Biol. Chem. 270, 27259-27265. 

Matsumura, K., Ervasti, J. M., Ohlendieck, K., Kahl, S. D., and Campbell, K. P. (1992). Association of dystrophin-related protein with dystrophin-associated proteins in mdx mouse muscle. Nature 360, 588-591. 

Nguyen, T. M., Ellis, J. M., Love, D. R., Davies, K. E., Gatter, K. C., Dickson, G., and Morris, G. E. (1991). Localization of the DMDL gene-encoded dystrophin-related protein using a panel of nineteen monoclonal antibodies Presence at neuromuscular junctions, in the sarcolemma of dystrophic skeletal muscle, in vascular and other smooth muscles, and in proliferating brain cell lines. J. Cell Biol. 115, 1695-1700. 

Blake DJ, Nawrotzki R, Loh NY, Gorecki DC, Davies KE. 1998. B-dystrobrevin, a member of the dystrophin-related protein family. Proc Natl Acad Sci USA 95 241-246. 

Blake DJ, Weir A, Newey SE, Davies KE. 2002. Function and genetics of dystrophin and dystrophin-related proteins in muscle. Physiol Rev 82 291-329. 

Dystrophin, shorter isoforms, and related proteins are found in many tissues including the brain.s One related protein, utrophin (dystrophin-related... 

Blake, D.J., Tinsley,J. M., Davies, K. E., Knight, A. E., Winder, S. J., and Kendrick-Jones, J. (1995). Coiled-coil regions in the carboxy-terminal domains of dystrophin and related proteins Potentials for protein-protein interactions. Trends Biochem. Sci. 20, 133-135. 

Apel, E.D., Lewis, R.M., Grady, R.M. and Sanes, J.R. (2000) Syne-1, a dystrophin- and Klarsicht-related protein associated with synaptic nuclei at the neuromuscular junction. J. Biol. Chem. 275, 31986-31995. 

Spectrin is a much more elongated protein compared to a-actinin due to the additional number of repeats. The additional repeats are more closely related to one another than repeats common to both a-actinin and spectrin. The spectrin repeat sequences are the most divergent in dystrophin and its homologue utrophin (Winder et al, 1995a), most likely reflecting an earlier divergent event when compared to spectrin (Pascual et al, 1997). 

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