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. Author manuscript; available in PMC: 2014 Jun 26.
Published in final edited form as: J Pathol. 2013 Oct;231(2):199–209. doi: 10.1002/path.4207

Figure 7.

Figure 7

Membrane breaches in dysferlin-deficient skeletal muscle release damage-associated molecular pattern molecules (DAMPs), which include endogenous TLR ligands such as ssRNA and ATP. These ligands bind to their respective receptors (ssRNA to TLR-7/8 and ATP to P2x7 receptor; step 1). Signalling through these receptors activates the inflammasome pathway in skeletal muscle as well as immune cells, leading to the production of pro-inflammatory cytokines such as IL-1, TNF OPN (step 2). These cytokines activate immune cells and exacerbate the inflammatory response and damage to skeletal muscle, leading to a self-sustaining disease.