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. Author manuscript; available in PMC: 2011 Jun 11.
Published in final edited form as: Cell. 2010 Jun 11;141(6):982–993. doi: 10.1016/j.cell.2010.05.018

Figure 6. Pathogenic DNA mutations in the mitochondrial termination sequence.

Figure 6

A. Scheme indicating the location of the mutations in the termination sequence and binding constants for titrations of a 22 bp oligonucleotide containing the leu-tRNA MTERF1 binding sequence carrying each of the pathogenic mutations into wt MTERF1. The DNA residues involved in arginine guanine interactions with MTERF1 are indicated by an asterisk. B. Interaction of R387 with G3249. Hydrogen-bonding distances are shown in orange. A simulated annealing fo-fc electron density map is shown (blue) contoured at 4σ. C. In vitro termination activity of wt MTERF1 on substrates containing each of the nine pathogenic mutations. FL, full-length run-off transcription. T, termination product. C, control lane without MTERF1. D. Quantification of termination activity on the different mutant sequences. The bar graph shows the percent termination observed in in vitro termination experiments with the termination sequence in the reverse orientation. Values correspond to the mean ± SD of at least three independent experiments.