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

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 lysosome-...

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Detalles Bibliográficos
Autores principales: Imani, Jewel, Bodine, Steven P. M., Lamattina, Anthony M., Ma, Diane D., Shrestha, Shikshya, Maynard, Dawn M., Bishop, Kevin, Nwokeji, Arinze, Malicdan, May Christine V., Testa, Lauren C., Sood, Raman, Stump, Benjamin, Rosas, Ivan O., Perrella, Mark A., Handin, Robert, Young, Lisa R., Gochuico, Bernadette R., El-Chemaly, Souheil
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9229912/
https://www.ncbi.nlm.nih.gov/pubmed/35739508
http://dx.doi.org/10.1186/s12931-022-02083-w