MicroRNAs involved in molecular circuitries relevant for the duchenne muscular dystrophy pathogenesis are controlled by the dystrophin/nNOS pathway
| Autor: | Alberto Auricchio, Laura Barberi, Tiziana Santini, Mariangela Morlando, Olga Sthandier, Carmine Nicoletti, Tania Incitti, Erika Girardi, Julie Martone, Antonio Musarò, Irene Bozzoni, Marcella Cesana, Davide Cacchiarelli |
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| Přispěvatelé: | Cacchiarelli, Davide, Martone, Julie, Girardi, Erika, Cesana, Marcella, Incitti, Tania, Morlando, Mariangela, Nicoletti, Carmine, Santini, Tiziana, Sthandier, Olga, Barberi, Laura, Auricchio, Alberto, Musar, Antonio, Bozzoni, Irene |
| Jazyk: | angličtina |
| Rok vydání: | 2010 |
| Předmět: |
Physiology
Duchenne muscular dystrophy HUMDISEASE Histone Deacetylase 2 DEVBIO Nitric Oxide Synthase Type I Dystrophin Mice 0302 clinical medicine Muscular Dystrophy Muscular dystrophy Regulation of gene expression 0303 health sciences Nitrosylation MicroRNA Skeletal musculoskeletal system Chromatin Cell biology Nitric oxide synthase Satellite Cells Muscle musculoskeletal diseases congenital hereditary and neonatal diseases and abnormalities Skeletal Muscle Satellite Cells Skeletal Muscle DNA Animals Gene Expression Regulation Mice Inbred mdx MicroRNAs Muscle Skeletal Muscular Dystrophy Animal Regeneration Biology 03 medical and health sciences medicine devbio dna humdisease Molecular Biology 030304 developmental biology Sarcolemma Animal Inbred mdx Cell Biology medicine.disease biology.protein Cancer research 030217 neurology & neurosurgery |
| Zdroj: | Cell metabolism 12 (2010): 341–351. doi:10.1016/j.cmet.2010.07.008 info:cnr-pdr/source/autori:Cacchiarelli D., Martone J., Girardi E., Cesana M., Incitti T., Nicoletti C., Santini T., Sthandier O., Auricchio A., Musarò A., Bozzoni I./titolo:microRNAs Involved in Molecular Circuitries Relevant for the Duchenne Muscular Dystrophy Pathogenesis Are Controlled by the Dystrophin%2FnNOS Pathway/doi:10.1016%2Fj.cmet.2010.07.008/rivista:Cell metabolism/anno:2010/pagina_da:341/pagina_a:351/intervallo_pagine:341–351/volume:12 Cell Metabolism Cell Metabolism; Vol 12 |
| Popis: | SummaryIn 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 pathogenetic 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 repression 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. |
| Databáze: | OpenAIRE |
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