Notably, the negative allosteric modulator of the CaSR, NPS2143, attenuated the upregulation of all the above pathways, targeting the key pathological hallmarks of IPF: transition to myofibroblast and contractility, collagen synthesis, ECM regulation, and altered cell morphology, suggesting the receptor plays a role in IPF pathogenesis. This evidence concerns the gene CASR and idiopathic pulmonary fibrosis.