Characterization of DRP2, a novel human dystrophin homologue

R G Roberts, T C Freeman, E Kendall, D L Vetrie, A K Dixon, C Shaw-Smith, Q Bone, M Bobrow

Research output: Contribution to journalArticlepeer-review


The currently recognised dystrophin protein family comprises the archetype, dystrophin, its close relative, utrophin or dystrophin-related protein (DRP), and a distantly related protein known as the 87K tyrosine kinase substrate. During the course of a phylogenetic study of sequences encoding the characteristic C-terminal domains of dystrophin-related proteins, we identified an unexpected novel class of vertebrate dystrophin-related sequences. We term this class dystrophin-related protein 2 (DRP2), and suggest that utrophin/DRP be renamed DRP1 to simplify future nomenclature. DRP2 is a relatively small protein, encoded in man by a 45 kb gene localized to Xq22. It is expressed principally in the brain and spinal cord, and is similar in overall structure to the Dp116 dystrophin isoform. The discovery of a novel relative of dystrophin substantially broadens the scope for study of this interesting group of proteins and their associated glycoprotein complexes.
Original languageEnglish
Pages (from-to)223-6
Number of pages4
JournalNature Genetics
Issue number2
Publication statusPublished - Jun 1996


  • Amino Acid Sequence
  • Animals
  • Base Sequence
  • Chromosome Mapping
  • Cytoskeletal Proteins
  • Dogfish
  • Dystrophin
  • Fishes
  • Humans
  • Membrane Proteins
  • Mice
  • Molecular Sequence Data
  • Muscle Proteins
  • Phylogeny
  • Sequence Analysis, DNA
  • Sequence Homology, Amino Acid
  • Tissue Distribution
  • Utrophin
  • X Chromosome


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