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.
Original language | English |
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Journal | Int J Mol Sci |
Volume | 21 |
Issue number | 17 |
DOIs | |
Publication status | Published - Aug 19 2020 |
Keywords
- Adenosine Triphosphate/metabolism
- Animals
- Humans
- Inflammasomes/metabolism
- Muscle, Skeletal/metabolism
- Muscular Dystrophies/metabolism
- Receptors, Purinergic P2X7/metabolism
- Signal Transduction