Different from the well-established roles of TNFα-NFκB and iNOS pathways in the pathogenesis of severe HPS in cirrhosis, only role of TNFα rather than whole TNFα-NFκB and iNOS cascades has been explored in experimental cirrhotic models with muscle wasting10, 11, 12. This evidence concerns the gene NFKB1 and Hermansky-Pudlak syndrome.