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1 Section of Pediatric and Adult Endocrinology, Diabetes and Metabolism, Kovler Diabetes Center and Comer Children's Hospital, University of Chicago Medicine, Chicago, Illinois, USA.
2 Hôpital Universitaire Necker-Enfants Malades, Université de Paris Cité, INSERM U1016, Institut IMAGINE, Paris, France.
3 Department of Clinical Science, University of Bergen, and Children and Youth Clinic, Hauk eland University Hospital, Bergen, Norway.
5 National Severe Insulin Resistance Service, Cambridge University Hospitals NHS Trust, Cambridge, UK.
6 Endocrinology and Diabetes Research Group, Biocruces Bizkaia Health Research Institute, Cruces University Hospital, CIBERDEM, CIBERER, Endo-ERN, UPV/EHU, Barakaldo, Spain.
7 Department of Paediatric Endocrinology and Diabetology, Charité - Universitätsmedizin, Berlin, Germany.
8 Center for Endocrinology, Metabolism, Genetics and Molecular Therapy, Departement of Pediatric Endocrinology and Diabetes, Vietnam National Children's Hospital, Hanoi, Vietnam.
9 Department of Pediatrics and Department of Biology and Medical Genetics, Hanoi Medical University, Hanoi, Vietnam.
10 Department of Pediatrics, Prince Mohamed bin Abdulaziz Hopsital, National Guard Health Affairs, Madinah, Saudi Arabia.
11 Institute of Biomedical and Clinical Sciences, University of Exeter Medical School, Exeter, UK.
12 Institute of Maternal and Child Research, School of Medicine, University of Chile, Santiago, Chile.
1 Section of Pediatric and Adult Endocrinology, Diabetes and Metabolism, Kovler Diabetes Center and Comer Children's Hospital, University of Chicago Medicine, Chicago, Illinois, USA.
2 Hôpital Universitaire Necker-Enfants Malades, Université de Paris Cité, INSERM U1016, Institut IMAGINE, Paris, France.
3 Department of Clinical Science, University of Bergen, and Children and Youth Clinic, Hauk eland University Hospital, Bergen, Norway.
5 National Severe Insulin Resistance Service, Cambridge University Hospitals NHS Trust, Cambridge, UK.
6 Endocrinology and Diabetes Research Group, Biocruces Bizkaia Health Research Institute, Cruces University Hospital, CIBERDEM, CIBERER, Endo-ERN, UPV/EHU, Barakaldo, Spain.
7 Department of Paediatric Endocrinology and Diabetology, Charité - Universitätsmedizin, Berlin, Germany.
8 Center for Endocrinology, Metabolism, Genetics and Molecular Therapy, Departement of Pediatric Endocrinology and Diabetes, Vietnam National Children's Hospital, Hanoi, Vietnam.
9 Department of Pediatrics and Department of Biology and Medical Genetics, Hanoi Medical University, Hanoi, Vietnam.
10 Department of Pediatrics, Prince Mohamed bin Abdulaziz Hopsital, National Guard Health Affairs, Madinah, Saudi Arabia.
11 Institute of Biomedical and Clinical Sciences, University of Exeter Medical School, Exeter, UK.
12 Institute of Maternal and Child Research, School of Medicine, University of Chile, Santiago, Chile.
Dr Michel Polak MD, PhD has acted as scientific advisor for the development of the glibenclamide‐glyburide suspension named AMGLIDIA in the European Union. The other authors have declared no conflicts of interest.
Figures
FIGURE 1
Insulin secretion from the pancreatic…
FIGURE 1
Insulin secretion from the pancreatic beta cell in (A) normal cell in a…
FIGURE 1
Insulin secretion from the pancreatic beta cell in (A) normal cell in a high plasma glucose environment and (B) in a cell with a K‐ATP channel mutation.
Source: adapted from Reference
. (A) Glucose enters the cell and is metabolized, causing an increase in ATP, K‐ATP channel closure is induced via ATP binding, the membrane is depolarized, and calcium influx is triggered resulting in the release of insulin from its storage vesicles. (B) A gain of function mutation in the K‐ATP channel results in the failure of ATP to bind to the channel, causing the channel to remain open, the membrane stays hyperpolarized and no insulin is released
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