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. 2023 Dec 21;134(5):e174508. doi: 10.1172/JCI174508

Figure 7. Human TMEM63A/B rescues respiratory failure in Tmem63a/b-cDKO mice.

Figure 7

(A) Survival curves for Ager-Sftpc-63ab-cDKO mice that received intratracheally delivered AAVs with empty vector (Ctrl), human TMEM63A (h63A), human TMEM63B (h63B), or the h63B-Y572A mutant after tamoxifen induction. The numbers of mice are shown in parentheses. (B) Images show the relatively normal appearance and structure of h63A- and h63B-rescued lungs compared with the lungs transduced with control or h63B-Y572A AAV. Scale bars: 200 μm. d11, day 11; d12, day 12; d60, day 60. (C) Expression of h63A, h63B, and h63B-Y572A in mouse lungs and localization in AT2 cells. Images show immunofluorescence staining for Pro-SPC (AT2), PDPN (AT1), Flag (h63A/B), and LAMP1 (LBs). Scale bars: 100 μm and 10 μm.