Abstract
The Duchenne muscular dystrophy gene gives rise to transcripts of several lengths. These mRNAs differ in their coding content and tissue distribution. The 14 kb mRNA encodes dystrophin, a 427 kDa protein found in muscle and brain, and the short transcripts described encode DP71, a 77 kDa protein found in various organs. These short transcripts have many features common to the deduced primary structure of dystrophin, especially in the cysteine-rich specific C-terminal domains. The dystrophin C-terminal domain could be involved in membrane anchorage via the glycoprotein complex, but such a functional role for these short transcript products has yet to be demonstrated. Here we report the first isolation of a short transcript product from saponin-solubilized cardiac muscle membranes using alkaline buffer and affinity chromatography procedures. This molecule was found to be glycosylated and could be easily dissociated from cardiac muscle and other non-muscle tissues such as brain and liver. DP71-specific monoclonal antibody helped to identify this molecule as being related to the dystrophin gene family. Immunofluorescence analysis of bovine or chicken cardiac muscle showed a periodic distribution of DP71 in transverse T tubules and this protein was co-localized with the dystrophin glycoprotein complex in the Z-disk area.
Dates
Type | When |
---|---|
Created | 10 years ago (Aug. 10, 2015, 5:37 p.m.) |
Deposited | 3 years, 9 months ago (Nov. 23, 2021, 5:46 p.m.) |
Indexed | 1 month, 3 weeks ago (July 8, 2025, 7:31 a.m.) |
Issued | 31 years, 4 months ago (April 15, 1994) |
Published | 31 years, 4 months ago (April 15, 1994) |
Published Print | 31 years, 4 months ago (April 15, 1994) |
@article{Fabbrizio_1994, title={Characterization and localization of a 77 kDa protein related to the dystrophin gene family}, volume={299}, ISSN={1470-8728}, url={http://dx.doi.org/10.1042/bj2990359}, DOI={10.1042/bj2990359}, number={2}, journal={Biochemical Journal}, publisher={Portland Press Ltd.}, author={Fabbrizio, E and Nudel, U and Hugon, G and Robert, A and Pons, F and Mornet, D}, year={1994}, month=apr, pages={359–365} }