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. 1999 Dec 21;96(26):15298–15303. doi: 10.1073/pnas.96.26.15298

Figure 5.

Figure 5

Disruption of the Arg-Lys-Arg triad as a result from mutations associated with aBS shifts pH gating off the neutral pH range. (A) Tertiary topology of the Kir1.1 subunit as deduced here together with point mutations identified in patients with aBS to date. Filled circles represent aBS muations, open symbols represent residues of the Arg-Lys-Arg triad. Mutations (referred to the ROMK1 sequence) are C49Y, I51T, V72E, D74Y, W99C, D108H, P110L, V122E, N124K, G167E, L214V, S219R, L220F, R311Q/W, V315G, and F325C. (B) Current-pHi relation obtained from Kir1.1 wild type and the aBS mutations indicated. Lines represent fit of a Boltzmann function to the data (mean ± SD of 5–8 experiments); values for pH0.5 were as follows: C49Y (7.5), I51T (7.3), D74Y (9.0), S219R (7.9), L220F (7.2), R311W (8.9), and V315G (6.9).