title: Characterization of DRP2, a novel human dystrophin homologue.
abstract: 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.
authors.affiliation: Division of Medical and Molecular Genetics, UMDS, London, UK.
authors.forename: R G
authors.initials: RG
authors.lastname: Roberts
authors.forename: T C
authors.initials: TC
authors.lastname: Freeman
authors.forename: E
authors.initials: E
authors.lastname: Kendall
authors.forename: D L
authors.initials: DL
authors.lastname: Vetrie
authors.forename: A K
authors.initials: AK
authors.lastname: Dixon
authors.forename: C
authors.initials: C
authors.lastname: Shaw-Smith
authors.forename: Q
authors.initials: Q
authors.lastname: Bone
authors.forename: M
authors.initials: M
authors.lastname: Bobrow
mesh_terms: D000595:Amino Acid Sequence; D000818:Animals; D001483:Base Sequence; D002874:Chromosome Mapping; D003598:Cytoskeletal Proteins / Q000737:chemistry* / Q000145:classification / Q000235:genetics*; D004284:Dogfish / Q000235:genetics; D016189:Dystrophin / Q000096:biosynthesis / Q000737:chemistry* / Q000235:genetics*; D005399:Fishes / Q000235:genetics; D006801:Humans; D008565:Membrane Proteins; D051379:Mice; D008969:Molecular Sequence Data; D009124:Muscle Proteins; D010802:Phylogeny; D017422:Sequence Analysis, DNA; D017386:Sequence Homology, Amino Acid; D014018:Tissue Distribution; D049411:Utrophin; D014960:X Chromosome
chemical_list: D003598:Cytoskeletal Proteins; D016189:Dystrophin; D008565:Membrane Proteins; D009124:Muscle Proteins; C483600:Utrn protein, mouse; D049411:Utrophin; C099589:dystrophin-related protein 2