The endocytic function and signaling properties confer a major role to LRP1 in the pathophysiology of numerous diseases such as hepatic steatosis, pulmonary hypertension, kidney fibrosis, acute respiratory distress syndrome, Alzheimer’s disease, atherosclerosis, and left ventricular modulation after acute myocardial infarction [31]. Here, LRP1 is linked to pulmonary arterial hypertension.