Figure 2. Quantitative protein complex analysis of LCA-causative lebercilin mutations.

Detection of protein complex alterations by comparison of the complexes of SF-TAP tagged lebercilin to either lebercilin-p.P493TfsX1 (A) or lebercilin-p.Q279X (B) in HEK293T cells. Plotted are log₁₀ ratios and log₁₀ intensities as quantified by MaxQuant from 3 independent experiments. For each experiment, label switching was done to exclude label-specific effects. Shown are only proteins quantified and identified in at least 2 of 3 experiments. Highly significant (P < 0.001) altered protein interactions are plotted in red, the majority of them being IFT proteins (Supplemental Table 1). Also shown are representations of the respective protein complexes; all proteins of the IFT protein complex, as well as WDR26, KIAA0564, PGAM5, YPEL5, C20orf11, and RANBP9, were lost for both mutants. CSNK2A1/2, CSNK2B, DYNLL1, DYNLL2, and USP9X only lost association to lebercilin-p.Q279X, not to lebercilin-p.P493TfsX1.