SERPINH1 and idiopathic pulmonary fibrosis: Heat shock protein 47 (HSP47) is a collagen-specific chaperone that is essential for the correct folding of pro-collagen fibrils in the ER.172HSP47 also plays a role in collagen turnover through collagenase, MMPs, and integrin signaling.172A phase II RCT with siRNA targeted HSP47 gene silencing (ND-L02-s0201) did not demonstrate efficacy in patients with IPF (identifier: NCT03538301).