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. 2022 Jan 27;59(1):2100267. doi: 10.1183/13993003.00267-2021

FIGURE 1.

FIGURE 1

Surfactant protein C (SFTPC)-I73T is aberrantly located and processed in vivo and in vitro. a) Schematics of SFTPC delineating structure and location of the pathogenic I73T mutation along with domains and approximate reported cleavage sites (dashed lines, numbered 1–4). b) ProSFTPC immunostaining of alveolar tissue from transgenic mice conditionally expressing SFTPC-WT (wild type) or SFTPC-I73T reveal punctate intracellular staining of SFTPC-WT and redistribution of SFTPC-I73T to the apical plasma membrane. c) When green fluorescent protein (GFP)-SFTPC-WT is expressed in HeLa cells it localises in intracellular punctate structures, whereas GFP-SFTPC-I73T relocalises to the plasma membrane and intracellular tubular structures. In cells expressing equal amounts of GFP (upper right panel), there is an excess of cell surface SFTPC-I73T as measured by a BRICHOS domain antibody (lower right panel). d) Immunoblotting of lysates from vector control or GFP-SFTPC expressing cells using an NproSFTPC antibody reveal altered processing of SFTPC-I73T. TM: transmembrane. Scale bars=10 μm or 5 μm (zoomed images).