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. 2019 Jul 8;9:9808. doi: 10.1038/s41598-019-46161-4

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© The Author(s) 2019

Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The images or other third party material in this article are included in the article’s Creative Commons license, unless indicated otherwise in a credit line to the material. If material is not included in the article’s Creative Commons license and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this license, visit http://creativecommons.org/licenses/by/4.0/.

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DNAJB9 may be a limiting factor for ERAD of ΔF508-CFTR. (A) DNAJB9 heterozygosity rescued CFTR-dependent fluid secretion in ΔF508-CFTR intestinal organoid. Intestinal organoid fluid secretion as described in “Material and Method” was performed to compare DNAJB9^+/+CFTR^{ΔF508/ΔF508} with DNAJB9^+/−CFTR^{ΔF508/ΔF508}. Representative images before and after FSK stimulation were shown on the top. Quantification of organoid fluid secretion was shown at the bottom. Each symbol accounts for one organoid. (B) DNAJB9 heterozygosity enhanced CFTR-dependent cholera toxin (CTX)-induced fluid secretion in in vivo closed loop experiments. In vivo closed loop experiment was performed as described in “Materials and Methods” to compare DNAJB9^+/+CFTR^{ΔF508/ΔF508} with DNAJB9^+/−CFTR^{ΔF508/ΔF508}. Representative images of intestinal loop were shown on the top upon two different-doses of CTX treatment. Quantification of secreted fluid were shown at the bottom. Student t-test was performed to determine the statistical significance. (*P < 0.05; **P < 0.01).