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

Shachar Abudi-SinreichSteven P BodineTadafumi YokoyamaNathanial J TolmanMichal TyrlikLauren C TestaChen G HanHeidi M DorwardStephen M WincovitchYair AniksterWilliam A GahlResat CinarBernadette R GochuicoMay Christine V Malicdan

Published in: Respiratory research (2022)

This model provides a preclinical tool that will allow researchers to study the mechanism of pulmonary fibrosis in HPS and provide a platform for the development of therapeutics to treat HPSPF. This method can be applied on studies of IPF or other monogenic disorders that lead to pulmonary fibrosis.

Keyphrases

pulmonary fibrosis
multiple sclerosis
idiopathic pulmonary fibrosis
small molecule
case control
bone marrow