title: Expression of dystrophin-associated proteins during neuronal differentiation of P19 embryonal carcinoma cells.
abstract: The dystrophin gene that is defective in Duchenne muscular dystrophy shows a complex transcriptional control based on several promoters driving independent cell-type-specific expression of different isoforms. Dystrophin isoforms together with dystroglycan, a transmembrane protein which in turn binds to extracellular matrix, are the core of a complex of proteins, the dystrophin-associated protein (DAP) complex, which also comprises cytoplasmic elements like dystrobrevin. Whereas the molecular organization of DAP complex in muscle is well documented, the composition of a similar complex in the nervous system remains largely unknown. We followed by competitive PCR the expression of DAP complex components during retinoic acid (RA)-induced neuronal differentiation of P19 cells. Transcripts for the full-length dystrophin, Dp427, and the short isoform, Dp71, as well as for alpha-dystrobrevin 2 increased in parallel with days in culture after RA stimulation, while dystroglycan, alpha-dystrobrevin 1 and 3, and beta-dystrobrevin were constitutively expressed. The upregulation of some of the components of the dystrophin complex during neuronal maturation suggests functional flexibility of the complex in the nervous system, where specific associations between different isoforms of DAP complex components could possibly organize distinct DAP complex-like complexes.
authors.affiliation: Laboratory of Cell Biology, Istituto Superiore di Sanità, Viale Regina Elena, 299-00161 Rome, Italy. mceccarini@iss.it
authors.forename: Marina
authors.initials: M
authors.lastname: Ceccarini
authors.forename: Pompeo
authors.initials: P
authors.lastname: Macioce
authors.forename: Barbara
authors.initials: B
authors.lastname: Panetta
authors.forename: Tamara C
authors.initials: TC
authors.lastname: Petrucci
mesh_terms: D000818:Animals; D000970:Antineoplastic Agents / Q000494:pharmacology; D015153:Blotting, Western; D002454:Cell Differentiation / Q000187:drug effects / Q000502:physiology; D003598:Cytoskeletal Proteins / Q000032:analysis / Q000235:genetics; D049030:Dystroglycans; D016189:Dystrophin / Q000032:analysis / Q000235:genetics*; D049029:Dystrophin-Associated Proteins; D054278:Embryonal Carcinoma Stem Cells; D015870:Gene Expression / Q000502:physiology; D008562:Membrane Glycoproteins / Q000032:analysis / Q000235:genetics; D008565:Membrane Proteins / Q000032:analysis / Q000235:genetics; D051379:Mice; D014411:Neoplastic Stem Cells; D009474:Neurons / Q000737:chemistry / Q000166:cytology* / Q000502:physiology*; D012333:RNA, Messenger / Q000032:analysis; D020133:Reverse Transcriptase Polymerase Chain Reaction; D014212:Tretinoin / Q000494:pharmacology; D014407:Tumor Cells, Cultured
chemical_list: D000970:Antineoplastic Agents; D003598:Cytoskeletal Proteins; D016189:Dystrophin; D049029:Dystrophin-Associated Proteins; D008562:Membrane Glycoproteins; D008565:Membrane Proteins; D012333:RNA, Messenger; C098885:dystrobrevin; D049030:Dystroglycans; D014212:Tretinoin