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. Author manuscript; available in PMC: 2020 Apr 29.
Published in final edited form as: Hum Mutat. 2019 Jun 18;40(10):1731–1748. doi: 10.1002/humu.23777

Figure 2. Kinetic parameters of the pathogenic ELAC2 variants.

Figure 2

(A) Kinetic parameters of the previously reported ELAC2 variants (Haack et al., 2013) with mt-tRNALeu(UUR) substrate. The graph shows kcat/KM value relative to WT (e.g. the quotient [kcat mutant]/[kcat WT]). The bars indicate standard error. *, ** above the bars indicate p-values with a significance of 0.05 and 0.01, respectively, calculated using unpaired t-test. (B) Kinetic parameters of the novel ELAC2 variants with mt-tRNALeu(UUR) substrate analysed as per (A). (C) Kinetic parameters of selected novel ELAC2 variants with mt-tRNAIle substrate analysed as per (A).