Introduction ATP + 1 1 2 ATP 2+ 3 ATP 2+ ATP 4 6 7 9 10 13 ATP KCNJ11 ABCC8 14 16 17 18 14 19 21 14 20 22 ATP 23 ATP 15 24 ATP ATP ATP 15 KCNJ11 ATP P o P o Methods Participants Molecular genetic analysis KCNJ11 25 Oocyte preparation Xenopus laevis Electrophysiology Xenopus 26 Xenopus 21 2 2 2 2 2 4 2 \documentclass[12pt]{minimal}
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\begin{document}$$\frac{G}{{G_{\text{c}} }} = \frac{1}{{1 + \left( {{{\left[ {{\text{ATP}}} \right]} \mathord{\left/{\vphantom {{\left[ {{\text{ATP}}} \right]} {{\text{IC}}_{50} }}} \right.\kern-\nulldelimiterspace} {{\text{IC}}_{50} }}} \right)^h }}$$\end{document} G K ATP G c K ATP 50 h P o t p Results Patient characteristics and genetics KCNJ11 27 28 1c −1 −1 −1 −1 −1 −1 1c −1 −1 −1 −1 28 1c 27 −1 −1 1c ATP Xenopus i 1 i 29 1 1 Fig. 1 ATP G ATP G c ATP  ATP 1 ATP ATP 21 ATP 2+ 2+ 11 50 2 1 Fig. 2 a c Xenopus a c 2+ b d K ATP G G c n n n b d 2+ 50 b 2+ 50 d 2+  Table 1 ATP sensitivity of wild-type and mutant channels   50 h 50 2+ h 2+ A 2+ %I max Wild-type 9.6 ± 1.6 1.30 ± 0.08 15.8 ± 3.0 0.99 ± 0.05 n.a. 0.01 ± 0.01 hetL164P 100 ± 8* 1.10 ± 0.05 118 ± 29* 1.16 ± 0.34 0.29 ± 0.07* 33.6 ± 3.4* homL164P n.a. n.a. n.a. n.a. n.a. 98 ± 1* Values are means ± SEM. The number of patches was four to nine in each case. A h 50 I max n.a. p Molecular mechanism of the reduced ATP sensitivity ATP 15 30 31 32 3 2 P o n n 4 2 P o Fig. 3 32  Fig. 4 ATP 2+  Table 2 Single-channel parameters for wild-type and homL164P channels   P o i Wild-type 0.39 ± 0.05 4.0 ± 0.1 homL164P 0.86 ± 0.01* 4.1 ± 0.1 P o i The number of patches was five to six in each case p ATP 2+ 24 2+ 2+ 2 2+ 33 34 50 2+ 1 1 Functional analysis: effects on MgADP sensitivity 5 n n n n Fig. 5 a I Xenopus 2+ b  5 Discussion KCNJ11 ATP K ATP Structural considerations and molecular basis for reduced ATP sensitivity 32 3 + 35 ATP 36 37 2+ 38 P o P o 30 36 37 39 P o 2+ 24 P o P o 16 15 2+ ATP P o 40 P o Clinical implications KCNJ11 6 Fig. 6 K ATP 21 14 26 15 24 16 34  P o Xenopus ATP P o