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Surfactant Protein A2 Mutations Associated with Pulmonary Fibrosis Lead to Protein Instability and Endoplasmic Reticulum Stress

Rare heterozygous mutations in the gene encoding surfactant protein A2 (SP-A2, SFTPA2) are associated with adult-onset pulmonary fibrosis and adenocarcinoma of the lung. We have previously shown that two recombinant SP-A2 mutant proteins (G231V and F198S) remain within the endoplasmic reticulum (ER)...

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Detalhes bibliográficos
Main Authors: Maitra, Meenakshi, Wang, Yongyu, Gerard, Robert D., Mendelson, Carole R., Garcia, Christine Kim
Formato: Artigo
Idioma:Inglês
Publicado em: American Society for Biochemistry and Molecular Biology 2010
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Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC2903395/
https://ncbi.nlm.nih.gov/pubmed/20466729
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1074/jbc.M110.121467
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