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Fig. 6 GATA6 mutant variants exhibit anomalous interaction with HHEX. (A) Coimmunoprecipitation assays of GATA6 and HHEX in HEK293T cells. (B?D) Analysis of the effect of HHEX on GATA6 protein stability. (E) Schematic drawing showing the positions of 15 mutated GATA6 variants associated with hepatobiliary malformations. GATA-N, transcriptional activation domain (light blue); ZnF, zinc finger domain (deep yellow). (F) Luciferase activity of a ?3 kb human LRH-1 promoter with wild type and 15 mutated GATA6 variants in HepG2 cells. (G) Coimmunoprecipitation assays of HHEX and five representative mutated GATA6 variants. (H) Luciferase activity of a ?3 kb human LRH-1 promoter with HHEX and five representative mutated GATA6 variants in HepG2 cells. (I) Luciferase assays of the ?3 kb zebrafish lrh-1 promoter with human GATA6 and HHEX in HepG2 cells. Data are mean ± SD, *P < 0.05, **P < 0.01, ****P < 0.0001, ns, nonsignificant, two-tailed unpaired t test.