There are four proteins, namely thromboxane A2 receptor (TBXA2R), cannabinoid receptor type 1 (CB1R), inducible nitric oxide synthase (iNOS), and galactose lectin-3 (Gal-3), involved in inflammation and TGF-b signaling and proliferation of fibroblasts that may be therapeutic targets for treatment of HPS-PF (Figure 2). This evidence concerns the gene NOS2 and Hermansky-Pudlak syndrome.