Identification and therapeutic modulation of protein targets in dysfunctional innate immune networks associated with hyperinflammation and microbial susceptibility in cystic fibrosis and inflammatory …

Inflammation is a normal biological response initiated by the immune system to help control and contain infections. Inflammatory diseases occur when defects arise in the immune system pathways that co-ordinate either the detection of pathogens, or the subsequent biological response. Single defects in various critical points on these pathways can lead to profoundly abnormal biological outcomes. When defects in critical points in these immune networks are present, two different scenarios with similar outcomes can occur. In some instances, this can result in a response that is insufficient (hypoinflammatory) for clearing foreign microbes from the body, increasing the risk of lethal infections. Alternately, the inflammatory response can be overly robust (hyperinflammatory), leading to chronic inflammation and tissue damage that impairs the immune response. Examples of diseases with hyperinflammatory phenotypes include, cystic fibrosis (CF) and inflammatory bowel disease (IBD). Matthew Mayer is studying the immune systems of children and adults with these diseases. His goal is to identify new proteins in these dysfunctional inflammatory pathways that could serve as potential drug targets. In addition to treating these immune diseases, such drugs could also be used to treat bacterial infections in otherwise healthy individuals by enhancing their immune systems. This research could lead to new therapies for patients who suffer from inherited hyperinflammatory disease. It could also advance the discovery of new, non-antibiotic drugs that could be used to fight off bacterial infections.