Data supporting phenotype of a transient neonatal diabetes point mutation (SUR1-R1183W) in mice

This data record contains 13 MS Excel files (.xls) underlying figures 1-2 and 5-8 in the associated article. The related study investigates the mechanism of neonatal diabetes remission and relapse (transient neonatal diabetes mellitus or TNDM) via mutations in mice of the KATP channel, a key controller of glucose homeostasis..BACKGROUND: The KATP channel plays a key role in glucose homeostasis by coupling metabolically generated changes in ATP to insulin secretion from pancreatic beta-cells. Gain-of-function mutations in either the pore-forming (Kir6.2) or regulatory (SUR1) subunit of this channel are a common cause of transient neonatal diabetes mellitus, in which diabetes presents shortly after birth but remits within the first few years of life, only to return in later life. The reasons behind this time dependence are unclear. METHODS: In an attempt to understand the mechanism behind diabetes remission and relapse, we generated mice expressing the common TNDM mutation SUR1-R1183W. We employed Cre/LoxP technology for both inducible and constitutive expression of SUR1-R1183W specifically in mouse beta-cells, followed by investigation of their phenotype using glucose tolerance tests and insulin secretion from isolated islets. This data record contains the following files:figure1A.xls and figure1B.xls: A: age at diagnosis, remission and relapse in the Exeter cohort of 92 TNDM patients with activating KATP channel mutations. B: The same variables for the 22 patients that had reached relapse stage at the time of study.figure 2.xls: electrophysiology results showing A: relative current I/I0 vs intracellular MgATP concentration [MgATP] in µM: for Kir6.2/SUR1 (wild-type), Kir6.2/SUR1-R1183W, Kir6.2/SUR1-FLAG and Kir6.2/SUR1-R1183W-FLAG KATP channels. and B: combined effects of 30 µM gliclazide (SU) and 100 µM MgATP (ATP).figure 5A.xls and figure 5B.xls: A: Fed blood glucose levels (mM) for β-R1183W mice (n=11) and littermate controls (Cre-only, n=4) vs age in days. B: Intra-peritoneal glucose tolerance test: Blood glucose levels (mM) for control (Cre-only, n=4) and iβ-R1183W (R1183W, n=8) mice at 12 weeks of age, vs time in minutes.figure 6A.xls and figure 6BC.xls: A: Body weight (g), sex, genotype and identifier of control (n=5) and β-R1183W (n=10) mice on a standard and high fat diet (HFD). HFD started at 8 weeks of age. Weights reported 8 days from 15-Mar-17 to 02-May-17. B: Fed blood glucose levels (mM), sex, date of birth, ear punch identifier, cage number,