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Surfactant protein C mutation links postnatal type 2 cell dysfunction to adult disease

Mutations in the gene SFTPC, encoding surfactant protein C (SP-C), are associated with interstitial lung disease in children and adults. To assess the natural history of disease, we knocked in a familial, disease-associated SFTPC mutation, L188Q (L184Q [LQ] in mice), into the mouse Sftpc locus. Tran...

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Detalles Bibliográficos
Autores principales: Sitaraman, Sneha, Martin, Emily P., Na, Cheng-Lun, Zhao, Shuyang, Green, Jenna, Deshmukh, Hitesh, Perl, Anne-Karina T., Bridges, James P., Xu, Yan, Weaver, Timothy E.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: American Society for Clinical Investigation 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8410047/
https://www.ncbi.nlm.nih.gov/pubmed/34138759
http://dx.doi.org/10.1172/jci.insight.142501