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A dominant negative variant of RAB5B disrupts maturation of surfactant protein B and surfactant protein C

Pathogenic variants in surfactant proteins SP-B and SP-C cause surfactant deficiency and interstitial lung disease. Surfactant proteins are synthesized as precursors (proSP-B, proSP-C), trafficked, and processed via a vesicular-regulated secretion pathway; however, control of vesicular trafficking e...

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Detalles Bibliográficos
Autores principales: Huang, Huiyan, Pan, Jiehong, Spielberg, David R., Hanchard, Neil A., Scott, Daryl A., Burrage, Lindsay C., Dai, Hongzheng, Murdock, David, Rosenfeld, Jill A., Mohammad, Ariz, Huang, Tao, Lindsey, Anika G., Kim, Hyori, Chen, Jian, Ramu, Avinash, Morrison, Stephanie A., Dawson, Zachary D., Hu, Alex Z., Tycksen, Eric, Silverman, Gary A., Baldridge, Dustin, Wambach, Jennifer A., Pak, Stephen C., Brody, Steven L., Schedl, Tim
Formato: Online Artículo Texto
Lenguaje:English
Publicado: National Academy of Sciences 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8832968/
https://www.ncbi.nlm.nih.gov/pubmed/35121658
http://dx.doi.org/10.1073/pnas.2105228119