Mice treated with lipopolysaccharide (LPS; bacterial endotoxin which induces a strong immune response and leads to increase in granzyme B) and monocrotaline-induced PAH mouse and rat models results in loss of full-length ITSN-1s expression and the presence of a 28-kDa fragment corresponding to the molecular weight of EHITSN [15]. This evidence concerns the gene GZMB and pulmonary arterial hypertension.