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

Biochem J. 1994 Apr 15;299 ( Pt 2)(Pt 2):359-65. doi: 10.1042/bj2990359.

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.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Amino Acid Sequence
  • Animals
  • Antibodies, Monoclonal
  • Base Sequence
  • Blotting, Western
  • Brain / metabolism
  • Cattle
  • Cell Membrane / metabolism
  • Chickens
  • DNA Primers
  • Dystrophin / analysis
  • Dystrophin / biosynthesis*
  • Dystrophin / genetics*
  • Humans
  • Liver / metabolism
  • Membrane Glycoproteins / analysis
  • Membrane Glycoproteins / biosynthesis*
  • Molecular Sequence Data
  • Molecular Weight
  • Muscular Dystrophies / genetics
  • Myocardium / cytology
  • Myocardium / metabolism*
  • Organ Specificity
  • Polymerase Chain Reaction
  • RNA, Messenger / metabolism
  • Rats
  • Restriction Mapping
  • Transcription, Genetic

Substances

  • Antibodies, Monoclonal
  • DNA Primers
  • Dystrophin
  • Membrane Glycoproteins
  • RNA, Messenger