Figure EV4. Selected ELAC2 mutations involved in hypertrophic cardiomyopathy do not impair the 3’-CCA antidetermination (related to Fig. 5).

(A) Interactions around the tRNA 3′-end or the 3′-CCA tail involve the same ELAC2 residues. Two parallel black lines indicate π-stacking. Selected electrostatic interactions are indicated with gray dotted lines. (B) Selected clinical mutations near the ELAC2 active site that only mildly impact the catalytic activity, namely T520I (Haack et al, 2013), F154L (Haack et al, 2013), and G132R (Paucar et al, 2018). Mutations are shown in dark and light purple for the NTD and the CTD of ELAC2, respectively. The rest of ELAC2 is in gray, and the tRNA is in red. (C) Cleavage activity of the ELAC2 mutants on tRNA^His-CCA assessed by TBE-UREA PAGE. None of the mutations significantly affects the 3′-CCA antideterminant effect. The WT panel is the same as in Fig. 5A, shown here for an easier side-to-side comparison. Representative gel images of technical triplicates and biological duplicates for WT. Source data are available online for this figure.