Drug Discovery for Idiopathic Pulmonary Fibrosis

Idiopathic pulmonary fibrosisIPFis the most fatal interstitial lung diseasewith high mortality but with few good treatment optionsIPF is suspected to develop from poorly definedrepeated alveolar injuries that cause increased secretion of extracellular matrixECMproteins and collagen production that result in the scarring characteristic of IPFNew FDA approved treatments do not stop disease progression and have considerable side effectsWe have identified matrix and matrix processing enzymes as novel drug targets for IPFOne such proteinFKbinding proteinFKBPhas been shown to play a pivotal role during these processesImportantlyloss of FKBPhas been shown to correlate with attenuated collagen production and diminished collagencross linkingHereinwe propose to make a library of non immunosuppressant inhibitors of FKBPfollowed by in vivo characterization of the efficacy of the new inhibitors Idiopathic pulmonary fibrosisIPFis a fatal lung disease with ayear survival rate oftowith few good treatment options availableIncreased secretion of matrix proteins and collagen production causing thickening of the lung tissues play a significant role in the pathologyWe have developed a strategy to target IPF by inhibiting FKBP