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. 2011 Nov 25;21(6):1272–1286. doi: 10.1093/hmg/ddr557

Figure 4.

Figure 4.

Loss of meckelin or filamin A causes deregulation of Wnt signalling. (A) TopFlash assays to measure levels of activated β-catenin, and hence canonical Wnt signalling, following co-transfection of immortalized normal control (control fibs, blue bars) and MKS3-mutated dermal fibroblasts (MKS3 fibs, red bars; see Fig. 3C) with reporter constructs and empty vector, wild-type HA-meckelin, p.F919del mutant HA-meckelin constructs and c myc-filamin A, as indicated. The empty vector results combine the data from transfections with pCMV-HA and pCMV-c myc. Activity is expressed as ratios of luciferase reporter construct expression, normalized for loading by measurement of a Renilla construct expression. The responses are shown to 0.5 × L cell control conditioned media (control), and conditioned media containing expressed Wnt3A and/or Wnt5A. Values shown are means of at least four independent replicates, with error bars indicating SEM. Statistical significance of pair-wise comparisons are shown (*P < 0.01 and **P < 0.001, Student's t-test). (B) TopFlash reporter assays as in (A) for control fibroblasts (control fibs, blue bars) and FLNA-mutated fibroblasts (FLNA fibs, green bars). Values shown are means of three independent replicates. Statistical significance of pair-wise comparisons are shown (*P < 0.05, error bars = SEM, **P < 0.01, Student's t-test).