Article
Aberrant lung remodeling in a mouse model of surfactant dysregulation induced by modulation of the Abca3 gene.
Annals of anatomy = Anatomischer Anzeiger : official organ of the Anatomische Gesellschaft - 1 Mar 2017
Beers Michael F, Knudsen Lars, Tomer Yaniv, Maronn Julian, Zhao Ming, Ochs Matthias, Mulugeta Surafel
Abstract excerpt
The lipid transporter, ATP binding cassette class A3 (ABCA3), plays a critical role in the biogenesis of alveolar type 2 (AT2) cell lamellar bodies (LBs). A relatively large number of mutations in the ABCA3 gene have been identified in association with diffuse parenchymal lung disease (DPLD), the most common of which is a missense mutation (valine substitution for lysine at residue 292 (ABCA3E292V)) that leads to...
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