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Systematic review of drug effects in humans and models with surfactant-processing disease
Fibrotic interstitial pneumonias are a group of rare diseases characterised by distortion of lung interstitium. Patients with mutations in surfactant-processing genes, such as surfactant protein C (SFTPC), surfactant protein A1 and A2 (SFTPA1 and A2), ATP binding cassette A3 (ABCA3) and Hermansky–Pu...
Autores principales: | Klay, Dymph, Hoffman, Thijs W., Harmsze, Ankie M., Grutters, Jan C., van Moorsel, Coline H.M. |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
European Respiratory Society
2018
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9489115/ https://www.ncbi.nlm.nih.gov/pubmed/29997245 http://dx.doi.org/10.1183/16000617.0135-2017 |
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