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Enhanced Notch3 signaling contributes to pulmonary emphysema in a Murine Model of Marfan syndrome
Marfan syndrome (MFS) is a heritable disorder of connective tissue, caused by mutations in the fibrillin-1 gene. Pulmonary functional abnormalities, such as emphysema and restrictive lung diseases, are frequently observed in patients with MFS. However, the pathogenesis and molecular mechanism of pul...
Autores principales: | , , , , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Nature Publishing Group UK
2020
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7331498/ https://www.ncbi.nlm.nih.gov/pubmed/32616814 http://dx.doi.org/10.1038/s41598-020-67941-3 |