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| Item Type: | Review |
|---|---|
| Title: | eATP/P2X7R axis: an orchestrated pathway triggering inflammasome activation in muscle diseases |
| Creators Name: | Panicucci, C., Raffaghello, L., Bruzzone, S., Baratto, S., Principi, E., Minetti, C., Gazzerro, E. and Bruno, C. |
| Abstract: | In muscle ATP is primarily known for its function as an energy source and as a mediator of the “excitation-transcription” process, which guarantees muscle plasticity in response to environmental stimuli. When quickly released in massive concentrations in the extracellular space as in presence of muscle membrane damage, ATP acts as a damage-associated molecular pattern molecule (DAMP). In experimental murine models of muscular dystrophies characterized by membrane instability, blockade of eATP/P2X7 receptor (R) purinergic signaling delayed the progression of the dystrophic phenotype dampening the local inflammatory response and inducing Foxp3(+) T Regulatory lymphocytes. These discoveries highlighted the relevance of ATP as a harbinger of immune-tissue damage in muscular genetic diseases. Given the interactions between the immune system and muscle regeneration, the comprehension of ATP/purinerigic pathway articulated organization in muscle cells has become of extreme interest. This review explores ATP release, metabolism, feedback control and cross-talk with members of muscle inflammasome in the context of muscular dystrophies. |
| Keywords: | eATP, P2X7R, Inflammasome, Muscular Dystrophies, Duchenne Muscular Dystrophy, Sarcoglycanopathies, Animals |
| Source: | International Journal of Molecular Sciences |
| ISSN: | 1422-0067 |
| Publisher: | MDPI |
| Volume: | 21 |
| Number: | 17 |
| Page Range: | 5963 |
| Date: | 19 August 2020 |
| Official Publication: | https://doi.org/10.3390/ijms21175963 |
| PubMed: | View item in PubMed |
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