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. Author manuscript; available in PMC: 2021 Jul 1.
Published in final edited form as: Hum Mutat. 2020 Apr 1;41(7):1298–1307. doi: 10.1002/humu.24014

Figure 4. Qualitative vesicle ultrastructure of A549 cells transduced with wild-type or mutant ABCA3 constructs.

Figure 4.

A549 cells transduced with adenovirus expressing WT-ABCA3 or ABCA3 variant constructs encoding p.Asn140His, p.Phe1203del, p.Ser1262Gly, p.Val1399Met, or p.Leu101Pro were fixed for assessment of lamellar body-like vesicle ultrastructure. White arrows indicate well-organized lamellar body-like vesicles, black arrows indicate abnormal appearing lamellar body-like vesicles. A549 cells expressing variants p.Asn140His, p.Phe1203del, p.Ser1262Gly, and p.Val1399Met had multiple well-organized lamellar body-like vesicles similar to WT-ABCA3. Similar to previous results, A549 cells expressing p.Leu101Pro had frequent abnormal appearing lamellar body-like vesicles with dense inclusions, whereas A549 cells expressing p.Glu292Val had well organized appearing lamellar body-like vesicles. Black arrowheads (untransduced A549 cells) indicate mitochondria. Scale bar = 0.8 mm