Muscle satellite cells and impaired late stage regeneration in different murine models for muscular dystrophies
| Autor: | Antonio Fernando Ribeiro, Stephanie A. Fernandes, Mariz Vainzof, A.L.F. Santos, Lucas Santos Souza, Camila F. Almeida, P.C.G. Onofre-Oliveira, Danielle A. Guerrieri, Renata Ishiba |
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| Rok vydání: | 2019 |
| Předmět: |
0301 basic medicine
Weakness Satellite Cells Skeletal Muscle Muscle Fibers Skeletal lcsh:Medicine Degeneration (medical) Muscle Development Article Muscular Dystrophies 03 medical and health sciences 0302 clinical medicine Muscle stem cells MODELOS ANIMAIS DE DOENÇAS Genetics medicine Animals Regeneration lcsh:Science Regulation of gene expression Multidisciplinary biology Regeneration (biology) lcsh:R PAX7 Transcription Factor Regenerative process biology.organism_classification Cell biology Mice Inbred C57BL Disease Models Animal Mechanisms of disease Ki-67 Antigen 030104 developmental biology Gene Expression Regulation Mice Inbred mdx lcsh:Q Satellite (biology) PAX7 medicine.symptom Stem cell tissues 030217 neurology & neurosurgery |
| Zdroj: | Scientific Reports Scientific Reports, Vol 9, Iss 1, Pp 1-11 (2019) Repositório Institucional da USP (Biblioteca Digital da Produção Intelectual) Universidade de São Paulo (USP) instacron:USP |
| ISSN: | 2045-2322 |
| Popis: | Satellite cells (SCs) are the main muscle stem cells responsible for its regenerative capacity. In muscular dystrophies, however, a failure of the regenerative process results in muscle degeneration and weakness. To analyze the effect of different degrees of muscle degeneration in SCs behavior, we studied adult muscle of the dystrophic strains: DMDmdx, Largemyd, DMDmdx/Largemyd, with variable histopathological alterations. Similar results were observed in the dystrophic models, which maintained normal levels of PAX7 expression, retained the Pax7-positive SCs pool, and their proliferation capacity. Moreover, elevated expression of MYOG, an important myogenic factor, was also observed. The ability to form new fibers was verified by the presence of dMyHC positive regenerating fibers. However, those fibers had incomplete maturation characteristics, such as small and homogenous fiber caliber, which could contribute to their dysfunction. We concluded that dystrophic muscles, independently of their degeneration degree, retain their SCs pool with proliferating and regenerative capacities. Nonetheless, the maturation of these new fibers is incomplete and do not prevent muscle degeneration. Taken together, these results suggest that the improvement of late muscle regeneration should better contribute to therapeutic approaches. |
| Databáze: | OpenAIRE |
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