title: Dystrophin isoforms DP71 and DP427 have distinct roles in myogenic cells.
abstract: Duchenne muscular dystrophy is caused by mutations in the dystrophin gene, a complex gene that generates a family of distinct isoforms. In immature muscle cells, two dystrophin isoforms are expressed, Dp427 and Dp71. To characterize the function of Dp71 in myogenesis, we have examined the expression of Dp71 in myogenic cells. The localization of Dp71 in these cells is distinct from the localization of Dp427. Whereas Dp427 localizes to focal adhesions and surface membrane during myogenesis, Dp71 localizes to stress fiberlike structures in myogenic cells. Biochemical fractionation of myogenic cells demonstrates that Dp71 cosediments with the actin bundles thus confirming this interaction. Furthermore, transfection of C2C12 myoblasts with constructs encoding Dp71 fused to green fluorescent protein targeted the protein to the actin microfilament bundles. These results demonstrate involvement of Dp71 with the actin cytoskeleton during myogenesis and suggest a role for Dp71 that is distinct from Dp427.
authors.affiliation: Department of Molecular and Medical Genetics, University of Toronto, Canada.
authors.forename: P L
authors.initials: PL
authors.lastname: Howard
authors.forename: G Y
authors.initials: GY
authors.lastname: Dally
authors.forename: S D
authors.initials: SD
authors.lastname: Ditta
authors.forename: R C
authors.initials: RC
authors.lastname: Austin
authors.forename: R G
authors.initials: RG
authors.lastname: Worton
authors.forename: H J
authors.initials: HJ
authors.lastname: Klamut
authors.forename: P N
authors.initials: PN
authors.lastname: Ray
mesh_terms: D000199:Actins / Q000378:metabolism; D000818:Animals; D015153:Blotting, Western; D002460:Cell Line; D002478:Cells, Cultured; D016189:Dystrophin / Q000031:analogs & derivatives* / Q000032:analysis / Q000235:genetics / Q000502:physiology*; D005333:Fetus; D018507:Gene Expression Regulation, Developmental; D049452:Green Fluorescent Proteins; D006801:Humans; D008164:Luminescent Proteins / Q000032:analysis / Q000235:genetics; D051379:Mice; D008856:Microscopy, Fluorescence; D018482:Muscle, Skeletal / Q000737:chemistry / Q000378:metabolism*; D020033:Protein Isoforms; D011993:Recombinant Fusion Proteins; D020133:Reverse Transcriptase Polymerase Chain Reaction; D013347:Subcellular Fractions; D014162:Transfection
chemical_list: D000199:Actins; D016189:Dystrophin; D008164:Luminescent Proteins; D020033:Protein Isoforms; D011993:Recombinant Fusion Proteins; C085339:apo-dystrophin 1; D049452:Green Fluorescent Proteins