Aberrant NLRP3 Inflammasome Activation Ignites the Fire of Inflammation in Neuromuscular Diseases
| Autor: | Christine Péladeau, Jagdeep K. Sandhu |
|---|---|
| Rok vydání: | 2021 |
| Předmět: |
muscular dystrophy
amyotrophic lateral sclerosis QH301-705.5 Inflammasomes innate immune system microglia Inflammation Review Muscle disorder Catalysis Proinflammatory cytokine neuroinflammation Inorganic Chemistry immune cells NLR Family Pyrin Domain-Containing 3 Protein medicine Animals Humans Physical and Theoretical Chemistry Biology (General) Molecular Biology QD1-999 Spectroscopy Neuroinflammation Tissue homeostasis therapy Innate immune system Microglia business.industry Organic Chemistry astrocytes Inflammasome General Medicine Neuromuscular Diseases cytokines Computer Science Applications macrophages Chemistry medicine.anatomical_structure Immunology medicine.symptom business medicine.drug |
| Zdroj: | International Journal of Molecular Sciences International Journal of Molecular Sciences, Vol 22, Iss 6068, p 6068 (2021) |
| ISSN: | 1422-0067 |
| Popis: | Inflammasomes are molecular hubs that are assembled and activated by a host in response to various microbial and non-microbial stimuli and play a pivotal role in maintaining tissue homeostasis. The NLRP3 is a highly promiscuous inflammasome that is activated by a wide variety of sterile triggers, including misfolded protein aggregates, and drives chronic inflammation via caspase-1-mediated proteolytic cleavage and secretion of proinflammatory cytokines, interleukin-1β and interleukin-18. These cytokines further amplify inflammatory responses by activating various signaling cascades, leading to the recruitment of immune cells and overproduction of proinflammatory cytokines and chemokines, resulting in a vicious cycle of chronic inflammation and tissue damage. Neuromuscular diseases are a heterogeneous group of muscle disorders that involve injury or dysfunction of peripheral nerves, neuromuscular junctions and muscles. A growing body of evidence suggests that dysregulation, impairment or aberrant NLRP3 inflammasome signaling leads to the initiation and exacerbation of pathological processes associated with neuromuscular diseases. In this review, we summarize the available knowledge about the NLRP3 inflammasome in neuromuscular diseases that affect the peripheral nervous system and amyotrophic lateral sclerosis, which affects the central nervous system. In addition, we also examine whether therapeutic targeting of the NLRP3 inflammasome components is a viable approach to alleviating the detrimental phenotype of neuromuscular diseases and improving clinical outcomes. |
| Databáze: | OpenAIRE |
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