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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-...
Autores principales: | , , , , , , , , , , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
BioMed Central
2022
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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 |