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

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...

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Detalles Bibliográficos
Autores principales: Abudi-Sinreich, Shachar, Bodine, Steven P., Yokoyama, Tadafumi, Tolman, Nathanial J., Tyrlik, Michal, Testa, Lauren C., Han, Chen G., Dorward, Heidi M., Wincovitch, Stephen M., Anikster, Yair, Gahl, William A., Cinar, Resat, Gochuico, Bernadette R., Malicdan, May Christine V.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2022
Materias:
Research
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9066931/
https://www.ncbi.nlm.nih.gov/pubmed/35509004
http://dx.doi.org/10.1186/s12931-022-02002-z

Internet

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9066931/
https://www.ncbi.nlm.nih.gov/pubmed/35509004
http://dx.doi.org/10.1186/s12931-022-02002-z

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