Idiopathic pulmonary fibrosis (IPF) is a chronic and fatal lung disease characterized by the
overgrowth, hardening, and scarring of lung tissue. The exact mechanisms of how IPF
develops and progresses are unknown. IPF is characterized by extracellular matrix
remodeling and accumulation of active TGFβ, which promotes collagen expression and the
differentiation of smooth muscle α-actin (SMA)-positive myofibroblasts. Aortic
carboxypeptidase-like protein (ACLP) is an extracellular matrix protein secreted by …