Figure 6.

Several patient mutations affect Merlin phosphorylation state and/or the binding to α-tubulin. A, the binding of α-tubulin and the phosphorylation state of Ser-518 and Thr-581 of Merlin isoform 1 and 2, truncation mutants as well as several patient missense mutants, were evaluated using HEK293 cells transfected with GFP-Merlin constructs. The expression level of the constructs is presented in the upper panel (probed with anti-GFP antibody). The list of constructs used is indicated below the Western blots. GFP was used as immunoprecipitation control throughout. B, Merlin Q324L expression in HeLa cells leads to a limited shortening of mitosis duration. In contrast, L64P mutant strongly delays NEB to telophase progression in a way very similar to Merlin S518A/T581A mutant (Fig. 3, A and B). Mann-Whitney test on R Software.