Academic Journal
Trafficking of ATP-sensitive potassium channels in health and disease
| العنوان: | Trafficking of ATP-sensitive potassium channels in health and disease |
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| المؤلفون: | Sivaprasadarao, A., Taneja, T. K., Mankouri, J., Smith, A. J. |
| المصدر: | Sivaprasadarao , A , Taneja , T K , Mankouri , J & Smith , A J 2007 , ' Trafficking of ATP-sensitive potassium channels in health and disease ' , Biochemical Society Transactions , vol. 35 , no. 5 , pp. 1055-1059 . https://doi.org/10.1042/BST0351055 |
| سنة النشر: | 2007 |
| المجموعة: | The University of Manchester: Research Explorer - Publications |
| مصطلحات موضوعية: | ATP-sensitive potassium channel (KATP channel), Coatamer protein I (COPI), Congenital hyperinsulinism, Endocytosis, Inwardly rectifying potassium channel 6.2 (Kir6.2), Neonatal diabetes mellitus |
| الوصف: | KATP channels (ATP-sensitive potassium channels), comprising four subunits each of Kir6.2 (inwardly rectifying potassium channel 6.2) and the SUR1 (sulfonylurea receptor 1), play a central role in glucose-stimulated insulin secretion by the pancreatic β-cell. Changes in the number of channels at the cell surface are associated with genetic diseases of aberrant insulin secretion, including CHI (congenital hyperinsulinism) and NDM (neonatal diabetes mellitus). The present review summarizes advances in our understanding of the vesicular trafficking of normal KATP channels and how genetic mutations in Kir6.2 interfere with such trafficking. A mutation, E282K, causing CHI, was found to disrupt a DXE [di-acidic ER (endoplasmic reticulum)-exit signal], thereby preventing its assembly into COPII (coatamer protein II)-coated vesicles and subsequent ER exit. The resultant decrease in the cell-surface density of the channel could explain the disease phenotype. Two mutations, Y330C and F333I, reported in patients with NDM, disrupted an endocytic traffic signal, thereby impairing CCV (clathrin-coated vesicle) formation and endocytosis. The consequent increase in the density of KATP channels, together with an attenuated sensitivity to ATP reported previously, may account for the severe form of NDM. ©The Authors. |
| نوع الوثيقة: | article in journal/newspaper |
| اللغة: | English |
| DOI: | 10.1042/BST0351055 |
| الاتاحة: | https://research.manchester.ac.uk/en/publications/08746a92-74ef-4ec6-8e59-37cc0e0071d9 https://doi.org/10.1042/BST0351055 |
| Rights: | info:eu-repo/semantics/closedAccess |
| رقم الانضمام: | edsbas.BCD8069B |
| قاعدة البيانات: | BASE |
| DOI: | 10.1042/BST0351055 |
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