Loss of functional K(ATP) channels in pancreatic β-cells causes persistent hyperinsulinemic hypoglycemia of infancy

Nature Medicine

Volumn 2, Issue 12, 1996, Pages 1344-1347

  Kane, Charlotte ^a   Shepherd, Ruth M ^a   Squires, Paul E ^a   Johnson, Paul R V ^b   James, Roger F L ^b   Milla, Peter J ^c   Aynsley Green, Albert ^c   Lindley, Keith J ^c   Dunne, Mark J ^a  

^a UNIVERSITY OF SHEFFIELD   (United Kingdom)

^b UNIVERSITY OF LEICESTER   (United Kingdom)

^c UNIVERSITY COLLEGE LONDON   (United Kingdom)

Author keywords

[No Author keywords available]

Indexed keywords

ADENOSINE TRIPHOSPHATASE (POTASSIUM); POTASSIUM CHANNEL; ABC TRANSPORTER; ADENOSINE TRIPHOSPHATE; CALCIUM CHANNEL BLOCKING AGENT; DRUG RECEPTOR; INWARDLY RECTIFYING POTASSIUM CHANNEL; SULFONYLUREA RECEPTOR; VERAPAMIL;

ARTICLE; CONTROLLED STUDY; HUMAN; HUMAN TISSUE; HYPERINSULINEMIA; HYPOGLYCEMIA; PANCREAS ISLET BETA CELL; PATHOGENESIS; PRIORITY JOURNAL; ACTION POTENTIAL; BLOOD; CELL CULTURE; HYPERINSULINISM; METABOLISM; NEWBORN; PANCREAS ISLET; PHYSIOLOGY;

ACTION POTENTIALS; ADENOSINE TRIPHOSPHATE; ATP-BINDING CASSETTE TRANSPORTERS; CALCIUM CHANNEL BLOCKERS; CELLS, CULTURED; HUMANS; HYPERINSULINISM; HYPOGLYCEMIA; INFANT, NEWBORN; ISLETS OF LANGERHANS; POTASSIUM CHANNELS; POTASSIUM CHANNELS, INWARDLY RECTIFYING; RECEPTORS, DRUG; VERAPAMIL;

EID: 0029658788     PISSN: 10788956     EISSN: None     Source Type: Journal    
DOI: 10.1038/nm1296-1344     Document Type: Article

References (26)

1. Morris, A.A.A. et al. Evaluation of fasts for investigating hypoglycaemia or suspected metabolic disease. Arch. Dis. Child. 75, 115-119 (1996).
2. Aynsley-Green, A. Nesidioblastosis of the pancreas in infancy. Dev. Med. Child. Neurol. 23, 372-9 (1981).
3. Aynsley-Green, A. et al. Nesidioblastosis of the pancreas: Definition of the syndrome and the management of the severe neonatal hyperinsulinaemic hypoglycaemia. Arch. Dis. Child. 56, 496-508 (1981).
4. Woolf, D.A., Leonard, J.V., Trembath, R.C., Pembury, M., & Grant, D.B. Nesidioblastosis: Evidence for an autosomal recessive variant. Arch. Dis. Child. 66, 529-530 (1991).
5. Thornton, P.S. et al. Familial hyperinsulinism (HI) inherited in an autosomal dominant (AD) form differs clinically and genetically from the more common autosomal recessive (AR) form. Pediatr. Res. 37, 100A (1995).
6. Spitz, L., Bhargav, R.K., Grant, D.B. & Leonard, J.V. Surgical treatment of hyperinsulinaemic hypoglycaemia in infancy and childhood. Arch. Dis. Child. 67, 201-205 (1992).
7. Kaiser, N. et al. Regulation of insulin release in persistent hyperinsulinaemic hypoglycaemia of infancy studied in long-term culture of pancreatic tissue. Diabetologia 33, 482-488 (1990).
8. Glaser, B. et al. Familial hyperinsulinism maps to chromosome 11p14-15.1, 30 cM centromeric to the insulin gene. Nature Genet. 7, 185-188 (1994).
9. Thomas, P.M. et al. Mutations in the sulfonylurea receptor gene in familial hyperinsulinemic hypoglycemia of infancy. Science 268, 426-429 (1995).
10. Aguilar-Bryan, L. et al. Cloning of the beta cell high-affinity sulfonylurea receptor: A regulator of insulin secretion. Science 268, 423-426 (1995).
11. Inagaki, N. et al. Reconstitution of IKATP: An inward rectifier subunit plus the sulfonylurea receptor. Science 270, 1166-1170 (1995).
12. Ashcroft, F.M., Harrison, D.E. & Ashcroft, S.J.H. Glucose induces closure of single potassium channels in isolated rat pancreatic beta-cells. Nature 312, 446-448 (1984).
13. Dunne, M.J. & Petersen, O.H. Potassium selective ion channels in insulin-secreting cells: Physiology, pharmacology and their role in stimulus secretion coupling. Biochim. Biophys. Acta (Rev. Biomembr.) 1071, 67-82 (1991).
14. Mathew, P.M. et al. Persistent neonatal hyperinsulinism. Clin. Pediatr. (USA) 27, 148-151 (1988).
15. Sempoux, C. et al. Nesidioblastosis and persistent neonatal hyperinsulinism. Diabete Metab. 21, 402-407 (1995).
16. Sakura, H., Ämmälä, C., Smith, P.A., Gribble, F.M. & Ashcroft, F.M. Cloning and functional expression of the cDNA encoding a novel ATP-sensitive potassium channel expressed in pancreatic B-cells, brain, heart and skeletal muscle. FEBS Lett. 377, 338-344 (1995).
17. Nichols, C.G. et al. Adenosine diphosphate as an intracellular regulator of insulin secretion. Science 272, 1785-1787 (1996).
18. Welsh, M.J. & Smith, H.E. (1993) Molecular mechanisms of CFTR chloride channel dysfunction in cystic fibrosis. Cell 73,-1251-1254.
19. Kristidis, P. et al. Genetic determination of exocrine pancreatic function in cystic fibrosis. Am. J. Hum. Genet. 50, 1178-1184 (1992).
20. - channels with altered pore properties. Nature 362, 160-164 (1993).
21. Higgins, C.F. The ABC of channel regulation. Cell 82, 693-618 (1995).
22. Lindley, K.J. et al. Ionic control of β-cell function in nesidioblastosis: A possible therapeutic role for calcium channel blockade Arch. Dis. Child. 74, 373-378 (1996).
23. Brandhorst, H., Klitcsher, D., Hering, B.J., Ferderlin, R. & Bretzel, R.G. Influence of organ procurement on human islet isolation. Honn. Metab. Res. 25, 51-52 (1993).
24. Hamill, O.P., Marty, A., Neher, E., Sakmann, B. & Sigworth, F.J. Improved patch-clamp techniques for high-resolution current recording from cells and cell-free membrane patches. Pfluegers Arch., 391, 85-100 (1981).
25. + channels and inhibition of insulin release; effect of BPDZ-62. J. Pharmacol. Exp. Ther. 277, 156-162 (1996).
26. 2+ signals in isolated human insulin-secreting cells. Pfluegers Arch. 427, 181-183 (1994).