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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Authors | Chiara Panicucci, Lizzia Raffaghello, Santina Bruzzone, Serena Baratto, Elisa Principi, Carlo Minetti, Elisabetta Gazzerro, Claudio Bruno |
Journal | International journal of molecular sciences
(Int J Mol Sci)
Vol. 21
Issue 17
(Aug 19 2020)
ISSN: 1422-0067 [Electronic] Switzerland |
PMID | 32825102
(Publication Type: Journal Article, Review)
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Chemical References |
- Inflammasomes
- Receptors, Purinergic P2X7
- Adenosine Triphosphate
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Topics |
- Adenosine Triphosphate
(metabolism)
- Animals
- Humans
- Inflammasomes
(metabolism)
- Muscle, Skeletal
(metabolism)
- Muscular Dystrophies
(metabolism)
- Receptors, Purinergic P2X7
(metabolism)
- Signal Transduction
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