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D. R., C. L. Na, and T. E. Weaver. Reserve autophagic capacity in alveolar epithelia provides a replicative niche for influenza A virus. Am J Respir Cell
Mol Biol. epub ahead of print. 2014.
J. M., M. Dong, K. S. Apsley, E. P. Martin, C. L. Na, S. Sitaraman, and T. E.
Weaver. Deficiency of the BiP cochaperone ERdj4 causes constitutive endoplasmic reticulum stress and metabolic defects. Mol Biol Cell. 25: 431-440. 2014.
Dong M, Bridges JP, Apsley K, Xu Y, Weaver TE. ERdj4 and ERdj5 Are Required for Endoplasmic Reticulum-associated Protein Degradation of Misfolded Surfactant Protein C. Mol. Biol. Cell. 19:2620-2630. 2008.
Nerelius C, Martin E, Peng S, Gustafsson M, Nordling K, Weaver TE, Johansson J. Mutations linked to interstitial lung disease can abrogate anti-amyloid function of prosurfactant protein C. Biochem. J. 416:201-209. 2008.
H., K. Nordling, T. E. Weaver, and J. Johansson. The Brichos domain-containing C-terminal part of pro-surfactant protein C binds to an unfolded poly-val transmembrane segment.J Biol Chem. 281: 21032-21039. 2006.
J. P., Y. Xu, C. L. Na, H. R. Wong, and T. E. Weaver. Adaptation and increased susceptibility to infection associated with constitutive expression of misfolded SP-C. J. Cell Biol. 172: 395-407. 2006.
J. P., S. E. Wert, L. M. Nogee, and T. E. Weaver. Expression of a human surfactant protein C mutation associated with interstitial lung disease disrupts lung development in transgenic mice. J. Biol. Chem. 278:
click to enlarge
SP-C proproteinThe N-terminal propeptide (green) and the C-terminal peptide (blue) are cleaved to generate the mature peptide (red) that is secreted into the alveolar airspaces with surfactant lipids. Three disease-associated mutations under study in the Weaver lab are shown: I73T, Δexon 4 (results in deletion of amino acids 109-145) and L188Q.
High expression of the SP-CΔexon4 mutant profoundly disrupts lung development in transgenic mice (TG1). Lower expression of the transgene results in a less severe phenotype (TG2 and TG3). For comparison, a WT lung is shown in the lower right panel.
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