eATP/P2X7R axis: an orchestrated pathway triggering inflammasome activation in muscle diseases
Item Type: | Review |
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Title: | eATP/P2X7R axis: an orchestrated pathway triggering inflammasome activation in muscle diseases |
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Creators Name: | Panicucci, C. and Raffaghello, L. and Bruzzone, S. and Baratto, S. and Principi, E. and Minetti, C. and Gazzerro, E. and Bruno, C. |
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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. |
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Keywords: | eATP, P2X7R, Inflammasome, Muscular Dystrophies, Duchenne Muscular Dystrophy, Sarcoglycanopathies, Animals |
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Source: | International Journal of Molecular Sciences |
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ISSN: | 1422-0067 |
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Publisher: | MDPI |
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Volume: | 21 |
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Number: | 17 |
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Page Range: | 5963 |
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Date: | 19 August 2020 |
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Official Publication: | https://doi.org/10.3390/ijms21175963 |
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PubMed: | View item in PubMed |
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