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Dysregulated myosin in Hermansky-Pudlak syndrome lung fibroblasts is associated with increased cell motility

Abstract Hermansky-Pudlak syndrome (HPS) is an autosomal recessive disorder characterized by improper biogenesis of lysosome-related organelles (LROs). Lung fibrosis is the leading cause of death among adults with HPS-1 and HPS-4 genetic types, which are associated with defects in the biogenesis of...

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Principais autores: Jewel Imani, Steven P. M. Bodine, Anthony M. Lamattina, Diane D. Ma, Shikshya Shrestha, Dawn M. Maynard, Kevin Bishop, Arinze Nwokeji, May Christine V. Malicdan, Lauren C. Testa, Raman Sood, Benjamin Stump, Ivan O. Rosas, Mark A. Perrella, Robert Handin, Lisa R. Young, Bernadette R. Gochuico, Souheil El-Chemaly
Formato: Artigo
Idioma:Inglês
Publicado em: BMC 2022-06-01
coleção:Respiratory Research
Assuntos:
Hermansky-Pudlak syndrome
Pulmonary fibrosis
Myosin IIB
Cell migration
Acesso em linha:https://doi.org/10.1186/s12931-022-02083-w
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