tiprelestat

Tiprelestat is a recombinant form of the human protein elafin and acts as a potent reversible inhibitor of human neutrophil elastase and proteinase 3. It is being developed primarily for the treatment of pulmonary arterial hypertension (PAH) and other indications such as postoperative inflammatory complications and myocardial reperfusion injury. Tiprelestat exerts anti-inflammatory and tissue-protective effects by inhibiting neutrophil exosomes and extracellular traps, suppressing NFkB signaling, restoring bone morphogenic protein receptor type II (BMPR2) signaling, reducing vascular smooth muscle cell proliferation, promoting alveolar formation, and suppressing reperfusion injury in various tissues. The drug has demonstrated an excellent safety profile in multiple clinical trials and has received orphan drug designation in both the US and EU for PAH[1][4][5][6][7].

Brand names

tiprelestat

Other names

elafinhuman elafinrecombinant elafinelastase-specific inhibitorskin-derived antileukoproteinasepeptidase inhibitor 3