Progressive pulmonary fibrosis in a murine model of Hermansky-Pudlak syndrome.

ABSTRACT:

Background

HPS-1 is a genetic type of Hermansky-Pudlak syndrome (HPS) with highly penetrant pulmonary fibrosis (HPSPF), a restrictive lung disease that is similar to idiopathic pulmonary fibrosis (IPF). Hps1^ep/ep (pale ear) is a naturally occurring HPS-1 mouse model that exhibits high sensitivity to bleomycin-induced pulmonary fibrosis (PF). Traditional methods of administering bleomycin as an intratracheal (IT) route to induce PF in this model often lead to severe acute lung injury and high mortality rates, complicating studies focusing on pathobiological mechanisms or exploration of therapeutic options for HPSPF.

Methods

To develop a murine model of HPSPF that closely mimics the progression of human pulmonary fibrosis, we investigated the pulmonary effects of sy

SUBMITTER: Abudi-Sinreich S 

PROVIDER: S-EPMC9066931 | biostudies-literature | 2022 May

REPOSITORIES: biostudies-literature