microRNAs Involved in Molecular Circuitries Relevant for the Duchenne Muscular Dystrophy Pathogenesis Are Controlled by the Dystrophin/nNOS Pathway (Articolo in rivista)

Type
Label
  • microRNAs Involved in Molecular Circuitries Relevant for the Duchenne Muscular Dystrophy Pathogenesis Are Controlled by the Dystrophin/nNOS Pathway (Articolo in rivista) (literal)
Anno
  • 2010-01-01T00:00:00+01:00 (literal)
Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#doi
  • 10.1016/j.cmet.2010.07.008 (literal)
Alternative label
  • Cacchiarelli D., Martone J., Girardi E., Cesana M., Incitti T., Nicoletti C., Santini T., Sthandier O., Auricchio A., Musarò A., Bozzoni I. (2010)
    microRNAs Involved in Molecular Circuitries Relevant for the Duchenne Muscular Dystrophy Pathogenesis Are Controlled by the Dystrophin/nNOS Pathway
    in Cell metabolism
    (literal)
Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#autori
  • Cacchiarelli D., Martone J., Girardi E., Cesana M., Incitti T., Nicoletti C., Santini T., Sthandier O., Auricchio A., Musarò A., Bozzoni I. (literal)
Pagina inizio
  • 341 (literal)
Pagina fine
  • 351 (literal)
Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#numeroVolume
  • 12 (literal)
Rivista
Note
  • ISI Web of Science (WOS) (literal)
Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#affiliazioni
  • Department of Genetics and Molecular Biology and IBPM, Institute Pasteur Cenci-Bolognetti, SAPIENZA University of Rome, Ple A Moro 5, 00185 Rome, Italy. (literal)
Titolo
  • microRNAs Involved in Molecular Circuitries Relevant for the Duchenne Muscular Dystrophy Pathogenesis Are Controlled by the Dystrophin/nNOS Pathway (literal)
Abstract
  • In Duchenne muscular dystrophy (DMD) the absence of dystrophin at the sarcolemma delocalizes and downregulates nitric oxide synthase (nNOS); this alters S-nitrosylation of HDAC2 and its chromatin association. We show that the differential HDAC2 nitrosylation state in Duchenne versus wild-type conditions deregulates the expression of a specific subset of microRNA genes. Several circuitries controlled by the identified microRNAs, such as the one linking miR-1 to the G6PD enzyme and the redox state of cell, or miR-29 to extracellular proteins and the fibrotic process, explain some of the DMD pathoge- netic traits. We also show that, at variance with other myomiRs, miR-206 escapes from the dystrophin- nNOS control being produced in activated satellite cells before dystrophin expression; in these cells, it contributes to muscle regeneration through repres- sion of the satellite specific factor, Pax7. We conclude that the pathway activated by dystrophin/nNOS controls several important circuitries increasing the robustness of the muscle differentiation program. (literal)
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