InflammX Technology
The NLRP3 inflammasome is a multiprotein complex that forms inside cells. It is essential for function of the innate immune system, the body's first response to any insult such as pathogenic organisms, foreign substances, or sterile stressors. The inflammasome pathway of inflammation is relatively new discovery, first described in 2002. Since then it has become a major target for therapeutic intervention since inflammasome mediated autoinflammation has been shown to be a common pathway of pathology in multiple disease states. The inflammasome has two triggering signals, a priming signal (the initial insult) which is unpredictable and beyond an organism’s control. This leads to an activation signal that enables assembly of inflammasome complexes, resulting in the activation of multiple pro-inflammatory cytokines and downstream pathology to affected tissues. If not terminated, these dynamic immune responses contribute to long lasting autoinflammatory disease. The InflammX drugs in development, regulate upstream activation signaling, breaking the persistent autoinflammation that is a feature of those diseases.
InflammX has identified that ATP (Adenosine Triphosphate- a compound that provides energy to drive processes in living cells) released through pathologically open Connexin43 (Cx43) hemichannels forming in the cell membrane, is the key inflammasome signal activator. Under normal physiological conditions, forming hemichannels migrate around the cell membrane until its extracellular protein loops “dock” to an analogous hemichannel structure on a neighboring cell. This forms the Gap Junction Channel between cells and is the portal for cell to cell communication.
In the presence of pathological stimuli, hemichannels open prematurely before docking with hemichannels on adjoining cells, creating what is known as a pathological pore. Prematurely open Cx43 hemichannels, facilitate the release of ATP into the extracellular space. The released ATP activates an inflammatory response mediated by the assembly of the NLRP3 innate inflammasome pathway of inflammation. Simultaneously there is an upregulation of the most ubiquitous connexin protein, Connexin43, which is then over expressed, further adding to the number of open Cx43 hemichannels, creating a perpetuated cycle of inflammation. This cycle continues to cause further release of ATP and inflammatory cytokines leading to perpetuated autoinflammation, resulting in significant tissue damage including microvascular leak, edema, microvascular dropout, ischemia and ultimately fibrosis.
InflammX product cadidates such as Xiflam™, break the cycle of activated inflammasome mediated autoinflammation by closing prematurely open Cx43 hemichannels, reducing and ultimately preventing the release of the inflammasome signal 2 activator (extracellular ATP). This provides a non inflammatory environment for cells to return to a state of physiological homeostasis. Once the cycle of inflammation is eliminated the damaged tissue has the opportunity to begin a regenerative process.