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Characterization and localization of a 77 kDa protein related to the dystrophin gene family.

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.

SUBMITTER: Fabbrizio E

PROVIDER: S-EPMC1138280 | biostudies-other | 1994 Apr

REPOSITORIES: biostudies-other

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