As a historical example, the lysyl oxidase (Lox) inhibitor β-aminopropionitrile (BAPN) has been used to improve both hemodynamic and histologic indices of PAH in experimental rodent models, thus reinforcing the therapeutic feasibility of targeting collagen cross-linking and ECM reprogramming in PH (33, 57–59). The gene discussed is LOX; the disease is pulmonary arterial hypertension.