Data for: Mitochondrial Ca2+-coupled generation of reactive oxygen species, peroxynitrite formation, and endothelial dysfunction in Cantú syndrome

Cantú syndrome is a multisystem disorder caused by gain-of-function (GOF) mutations in KCNJ8 and ABCC9, the genes encoding the pore-forming inward rectifier Kir6.1 and regulatory sulfonylurea receptor SUR2B subunits, respectively, of vascular ATP-sensitive K+ channels (KATP). In this study, we investigated changes in the vascular endothelium in mice in which Cantú syndrome -associated Kcnj8 or Abcc9 mutations were knocked-in to the endogenous loci. We found that endothelium-dependent dilation was impaired in small mesenteric arteries from Cantú mice. Loss of endothelium-dependent vasodilation led to increased vasoconstriction in response to intraluminal pressure or treatment with the adrenergic receptor agonist phenylephrine. We also found that either KATP GOF or acute activation of KATP channels with pinacidil increased the amplitude and frequency of wave-like Ca2+ events generated in the endothelium in response to the vasodilator agonist carbachol. Increased cytosolic Ca2+ signaling a..., GraphPad Prism software (Version 9.4.1) was used to conduct statistical analyses and generate graphs. The data presented are expressed as means ± SEM, with \"n\" indicating the number of vessels or cells analyzed. One-way analysis of variance (ANOVA) with Tukey's multiple comparisons test and two-way ANOVA with Tukey's or Šídák's multiple comparisons test were used for statistical analyses. P-values < 0.05 were considered statistically significant for all analyses., , # Data for: Mitochondrial Ca2+-coupled generation of reactive oxygen species, peroxynitrite formation, and endothelial dysfunction in Cantú syndrome

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## Description of the data and file structure

### Figure\_1\_Processed\_Data

1D: Summary data of whole-cell current densities from voltage-clamp recordings of WT and Kir6.1wt/VM SMCs with indicated treatments\
1F: Summary data of whole-cell current densities from voltage-clamp recordings of vascular endothelial cells from WT and Kir6.1wt/VM with indicated treatments
1H: Summary of current-clamp recordings of vascular endothelial cells from WT and Kir6.1wt/VM mice.

### Figure\_2\_Processed\_Data

2A: Representative recordings data showing endothelium-dependent vasodilation evoked by the muscarinic receptor agonist carbachol (CCh) in mesenteric arterioles from WT and Kir6.1wt/VM mice
2B: Summary data showing endothelium-dependent vasodilation evoked by the muscarinic receptor agonist carbachol (CCh) in mesenteric arterioles from WT ...