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eATP/P2X7R Axis: An Orchestrated Pathway Triggering Inflammasome Activation in Muscle Diseases.

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.
AuthorsChiara Panicucci, Lizzia Raffaghello, Santina Bruzzone, Serena Baratto, Elisa Principi, Carlo Minetti, Elisabetta Gazzerro, Claudio Bruno
JournalInternational journal of molecular sciences (Int J Mol Sci) Vol. 21 Issue 17 (Aug 19 2020) ISSN: 1422-0067 [Electronic] Switzerland
PMID32825102 (Publication Type: Journal Article, Review)
Chemical References
  • Inflammasomes
  • Receptors, Purinergic P2X7
  • Adenosine Triphosphate
Topics
  • Adenosine Triphosphate (metabolism)
  • Animals
  • Humans
  • Inflammasomes (metabolism)
  • Muscle, Skeletal (metabolism)
  • Muscular Dystrophies (metabolism)
  • Receptors, Purinergic P2X7 (metabolism)
  • Signal Transduction

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