Hyperinsulinism in Infancy: From Basic Science to Clinical Disease

Type 2 (non-insulin-dependent) diabetes mellitus: the thrifty phenotype hypothesis

Hales, C. N., & Barker, D. J. P. (1992) · Diabetologia · doi:10.1007/bf00400248 · Cited by 3,309

Gestational diabetes mellitus

Buchanan, T. A., & Xiang, A. H. (2005) · Journal of Clinical Investigation · doi:10.1172/jci200524531 · Cited by 2,123

Insulin-promoter-factor 1 is required for pancreas development in mice

Jonsson, J., Carlsson, L., Edlund, T., & Edlund, H. (1994) · Nature · doi:10.1038/371606a0 · Cited by 1,785

Reconstitution of I KATP : An Inward Rectifier Subunit Plus the Sulfonylurea Receptor

Inagaki, N., Gonoi, T., Clement, J. P., Namba, N., Inazawa, J., Gonzalez, G., Aguilar-Bryan, L., Seino, S., & Bryan, J. (1995) · Science · doi:10.1126/science.270.5239.1166 · Cited by 1,709

Mutations in the hepatocyte nuclear factor-1α gene in maturity-onset diabetes of the young (MODY3)

Yamagata, K., Oda, N., Kaisaki, P. J., Menzel, S., Furuta, H., Vaxillaire, M., Southam, L., Cox, R. D., Lathrop, G. M., Boriraj, V. V., et al. (1996) · Nature · doi:10.1038/384455a0 · Cited by 1,614

Cloning of the β Cell High-Affinity Sulfonylurea Receptor: a Regulator of Insulin Secretion

Aguilar-Bryan, L., Nichols, C. G., Wechsler, S. W., Clement, J. P., Boyd, A. E., González, G., Herrera-Sosa, H., Nguy, K., Bryan, J., & Nelson, D. A. (1995) · Science · doi:10.1126/science.7716547 · Cited by 1,315

Triggering and amplifying pathways of regulation of insulin secretion by glucose.

Henquin, J. C. (2000) · Diabetes · doi:10.2337/diabetes.49.11.1751 · Cited by 1,201

Pancreatic agenesis attributable to a single nucleotide deletion in the human IPF1 gene coding sequence

Stoffers, D. A., Zinkin, N. T., Stanojevic, V., Clarke, W. L., & Habener, J. F. (1997) · Nature Genetics · doi:10.1038/ng0197-106 · Cited by 1,106

A New ER Trafficking Signal Regulates the Subunit Stoichiometry of Plasma Membrane KATP Channels

Zerangue, N., Schwappach, B., Jan, Y. N., & Jan, L. Y. (1999) · Neuron · doi:10.1016/s0896-6273(00)80708-4 · Cited by 1,025

Mutations in the hepatocyte nuclear factor-4α gene in maturity-onset diabetes of the young (MODY1)

Yamagata, K., Furuta, H., Oda, N., Kaisaki, P. J., Menzel, S., Cox, N. J., Fajans, S. S., Signorini, S., Stoffel, M., & Bell, G. I. (1996) · Nature · doi:10.1038/384458a0 · Cited by 976

A Family of Sulfonylurea Receptors Determines the Pharmacological Properties of ATP-Sensitive K+ Channels

Inagaki, N., Gonoi, T., Iv, J. P. C., Wang, C.-Z., Aguilar-Bryan, L., Bryan, J., & Seino, S. (1996) · Neuron · doi:10.1016/s0896-6273(00)80124-5 · Cited by 974

Secretory Granule Exocytosis

Burgoyne, R. D., & Morgan, A. (2003) · Physiological Reviews · doi:10.1152/physrev.00031.2002 · Cited by 934

Association of In Vitro Fertilization with Beckwith-Wiedemann Syndrome and Epigenetic Alterations of LIT1 and H19

DeBaun, M. R., Niemitz, E. L., & Feinberg, A. P. (2003) · The American Journal of Human Genetics · doi:10.1086/346031 · Cited by 916

IPF1, a homeodomain-containing transactivator of the insulin gene.

Ohlsson, H., Karlsson, K., & Edlund, T. (1993) · The EMBO Journal · doi:10.1002/j.1460-2075.1993.tb06109.x · Cited by 888

Early-onset type-ll diabetes mellitus (MODY4) linked to IPF1

Staffers, D. A., Ferrer, J., Clarke, W. L., & Habener, J. F. (1997) · Nature Genetics · doi:10.1038/ng1097-138 · Cited by 882

Mutations in the Sulfonylurea Receptor Gene in Familial Persistent Hyperinsulinemic Hypoglycemia of Infancy

Thomas, P. M., Cote, G. J., Wohllk, N., Haddad, B., Mathew, P. M., Rabl, W., Aguilar-Bryan, L., Gagel, R. F., & Bryan, J. (1995) · Science · doi:10.1126/science.7716548 · Cited by 806

Glucose intolerance but normal satiety in mice with a null mutation in the glucagon–like peptide 1 receptor gene

Scrocchi, L. A., Brown, T. J., Maclusky, N., Brubaker, P. L., Auerbach, A. B., Joyner, A. L., & Drucker, D. J. (1996) · Nature Medicine · doi:10.1038/nm1196-1254 · Cited by 793

Truncation of Kir6.2 produces ATP-sensitive K+ channels in the absence of the sulphonylurea receptor

Tucker, S. J., Gribble, F. M., Zhao, C., Trapp, S., & Ashcroft, F. M. (1997) · Nature · doi:10.1038/387179a0 · Cited by 753

Association and Stoichiometry of KATP Channel Subunits

Clement, J. P., Kunjilwar, K., Gonzalez, G., Schwanstecher, M., Panten, U., Aguilar-Bryan, L., & Bryan, J. (1997) · Neuron · doi:10.1016/s0896-6273(00)80321-9 · Cited by 692

Hyperinsulinism and Hyperammonemia in Infants with Regulatory Mutations of the Glutamate Dehydrogenase Gene

Stanley, C. A., Lieu, Y. K., Hsu, B. Y. L., Burlina, A. B., Greenberg, C. R., Hopwood, N. J., Perlman, K., Rich, B. H., Zammarchi, E., & Poncz, M. (1998) · New England Journal of Medicine · doi:10.1056/nejm199805073381904 · Cited by 677

Gestational Diabetes Mellitus

(1986) · Annals of Internal Medicine · doi:10.7326/0003-4819-105-3-461 · Cited by 663

Close linkage of glucokinase locus on chromosome 7p to early-onset non-insulin-dependent diabetes mellitus

Froguel, P., Vaxillaire, M., Sun, F., Velho, G., Zouali, H., Butel, M. O., Lesage, S., Vionnet, N., Clément, K., Fougerousse, F., et al. (1992) · Nature · doi:10.1038/356162a0 · Cited by 630

Mutations in NEUROD1 are associated with the development of type 2 diabetes mellitus

Malecki, M. T., Jhala, U. S., Antonellis, A., Fields, L., Doria, A., Orban, T., Saad, M., Warram, J. H., Montminy, M., & Krolewski, A. S. (1999) · Nature Genetics · doi:10.1038/15500 · Cited by 619

Nonsense mutation in the glucokinase gene causes early-onset non-insulin-dependent diabetes mellitus

Vionnet, N., Stoffel, M., Takeda, J., Yasuda, K., Bell, G. I., Zouali, H., Lesage, S., Velho, G., Iris, F., Passa, P., et al. (1992) · Nature · doi:10.1038/356721a0 · Cited by 604

Familial Hyperinsulinism Caused by an Activating Glucokinase Mutation

Glaser, B., Kesavan, P., Heyman, M., Davis, E., Cuesta, A., Buchs, A., Stanley, C. A., Thornton, P. S., Permutt, M. A., Matschinsky, F. M., et al. (1998) · New England Journal of Medicine · doi:10.1056/nejm199801223380404 · Cited by 564

Membrane Phospholipid Control of Nucleotide Sensitivity of K ATP Channels

Shyng, S.-L., & Nichols, C. G. (1998) · Science · doi:10.1126/science.282.5391.1138 · Cited by 559

Toward Understanding the Assembly and Structure of KATPChannels

AGUILAR-BRYAN, L., CLEMENT, J. P., GONZALEZ, G., KUNJILWAR, K., BABENKO, A., & BRYAN, J. (1998) · Physiological Reviews · doi:10.1152/physrev.1998.78.1.227 · Cited by 540

Adenosine Diphosphate as an Intracellular Regulator of Insulin Secretion

Nichols, C. G., Shyng, S.-L., Nestorowicz, A., Glaser, B., Clement, J. P., Gonzalez, G., Aguilar-Bryan, L., Permutt, M. A., & Bryan, J. (1996) · Science · doi:10.1126/science.272.5269.1785 · Cited by 524

Mitochondrial function in normal and diabetic β-cells

Maechler, P., & Wollheim, C. B. (2001) · Nature · doi:10.1038/414807a · Cited by 519

PIP 2 and PIP as Determinants for ATP Inhibition of K ATP Channels

Baukrowitz, T., Schulte, U., Oliver, D., Herlitze, S., Krauter, T., Tucker, S. J., Ruppersberg, J. P., & Fakler, B. (1998) · Science · doi:10.1126/science.282.5391.1141 · Cited by 513

Physiological and pathophysiological roles of ATP-sensitive K+ channels

Seino, S., & Miki, T. (2003) · Progress in Biophysics and Molecular Biology · doi:10.1016/s0079-6107(02)00053-6 · Cited by 505

Defective insulin secretion and enhanced insulin action in K ATP channel-deficient mice

Miki, T., Nagashima, K., Tashiro, F., Kotake, K., Yoshitomi, H., Tamamoto, A., Gonoi, T., Iwanaga, T., Miyazaki, J.-I., & Seino, S. (1998) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.95.18.10402 · Cited by 502

THE SULPHONYLUREA RECEPTOR MAY BE AN ATP-SENSITIVE POTASSIUM CHANNEL

Sturgess, N., Cook, D., Ashford, M. J., & Hales, C. N. (1985) · The Lancet · doi:10.1016/s0140-6736(85)90403-9 · Cited by 500

Pancreatic islet glucose metabolism and regulation of insulin secretion

Meglasson, M. D., & Matschinsky, F. M. (1986) · Diabetes/Metabolism Reviews · doi:10.1002/dmr.5610020301 · Cited by 499

Octameric Stoichiometry of the KATP Channel Complex

Shyng, S.-L., & Nichols, C. G. (1997) · The Journal of General Physiology · doi:10.1085/jgp.110.6.655 · Cited by 495

Evidence that glucose can control insulin release independently from its action on ATP-sensitive K+ channels in mouse B cells.

Gembal, M., Gilon, P., & Henquin, J. C. (1992) · Journal of Clinical Investigation · doi:10.1172/jci115714 · Cited by 494

cAMP-GEFII is a direct target of cAMP in regulated exocytosis

Ozaki, N., Shibasaki, T., Kashima, Y., Miki, T., Takahashi, K., Ueno, H., Sunaga, Y., Yano, H., Matsuura, Y., Iwanaga, T., et al. (2000) · Nature Cell Biology · doi:10.1038/35041046 · Cited by 481

-to: Hales CN, Barker DJP (1992) Type 2 (non-insulin-dependent) diabetes mellitus: the thrifty phenotype hypothesis. Diabetologia 35:595–601

Hockaday, T. D. R., & Yajnik, C. S. (2003) · Diabetologia · doi:10.1007/s00125-002-1024-1 · Cited by 469

Are the β-Cell Signaling Molecules Malonyl-CoA and Cystolic Long-Chain Acyl-CoA Implicated in Multiple Tissue Defects of Obesity and NIDDM?

Prentki, M., & Corkey, B. E. (1996) · Diabetes · doi:10.2337/diab.45.3.273 · Cited by 469

Loss of imprinting of IGF2 is linked to reduced expression and abnormal methylation of H19 in Wilms' tumour

Steenman, M. J. C., Rainier, S., Dobry, C. J., Grundy, P., Horon, I. L., & Feinberg, A. P. (1994) · Nature Genetics · doi:10.1038/ng0794-433 · Cited by 460

Glucose intolerance caused by a defect in the entero-insular axis: A study in gastric inhibitory polypeptide receptor knockout mice

Miyawaki, K., Yamada, Y., Yano, H., Niwa, H., Ban, N., Ihara, Y., Kubota, A., Fujimoto, S., Kajikawa, M., Kuroe, A., et al. (1999) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.96.26.14843 · Cited by 454

Low lactate dehydrogenase and high mitochondrial glycerol phosphate dehydrogenase in pancreatic beta-cells. Potential role in nutrient sensing.

Sekine, N., Cirulli, V., Regazzi, R., Brown, L. J., Gine, E., Tamarit-Rodriguez, J., Girotti, M., Marie, S., MacDonald, M. J., & Wollheim, C. B. (1994) · Journal of Biological Chemistry · doi:10.1016/s0021-9258(17)37629-9 · Cited by 446

Malonyl-CoA and long chain acyl-CoA esters as metabolic coupling factors in nutrient-induced insulin secretion.

Prentki, M., Vischer, S., Glennon, M. C., Regazzi, R., Deeney, J. T., & Corkey, B. E. (1992) · Journal of Biological Chemistry · doi:10.1016/s0021-9258(18)42624-5 · Cited by 442

Mutation of the pancreatic islet inward rectifier Kir6.2 also leads to familial persistent hyperinsulinemic hypoglycemia of infancy

Thomas, P. (1996) · Human Molecular Genetics · doi:10.1093/hmg/5.11.1809 · Cited by 442

Malonyl-CoA Signaling, Lipid Partitioning, and Glucolipotoxicity

Prentki, M., Joly, E., El-Assaad, W., & Roduit, R. (2002) · Diabetes · doi:10.2337/diabetes.51.2007.s405 · Cited by 431

Human and rat beta cells differ in glucose transporter but not in glucokinase gene expression.

De Vos, A., Heimberg, H., Quartier, E., Huypens, P., Bouwens, L., Pipeleers, D., & Schuit, F. (1995) · Journal of Clinical Investigation · doi:10.1172/jci118308 · Cited by 425

Neonatal Diabetes Mellitus Due to Complete Glucokinase Deficiency

Njølstad, P. R., Søvik, O., Cuesta-Muñoz, A., Bjørkhaug, L., Massa, O., Barbetti, F., Undlien, D. E., Shiota, C., Magnuson, M. A., Molven, A., et al. (2001) · New England Journal of Medicine · doi:10.1056/nejm200105243442104 · Cited by 424

Clinical features and natural history of Beckwith‐Wiedemann syndrome: presentation of 74 new cases

Elliott, M., Bayly, R., Cole, T., Temple, I. K., & Maher, E. R. (1994) · Clinical Genetics · doi:10.1111/j.1399-0004.1994.tb04219.x · Cited by 423

Glucose‐stimulated signaling pathways in biphasic insulin secretion

Straub, S. G., & Sharp, G. W. G. (2002) · Diabetes/Metabolism Research and Reviews · doi:10.1002/dmrr.329 · Cited by 421

Cloning and functional expression of the cDNA encoding a novel ATP‐sensitive potassium channel subunit expressed in pancreatic β‐cells, brain, heart and skeletal muscle

(1995) · FEBS Letters · doi:10.1016/0014-5793(95)01369-5 · Cited by 411

Gestational Diabetes Mellitus

Jovanovic, L. (2001) · JAMA · doi:10.1001/jama.286.20.2516 · Cited by 399

Critical Role of cAMP-GEFII·Rim2 Complex in Incretin-potentiated Insulin Secretion

Kashima, Y., Miki, T., Shibasaki, T., Ozaki, N., Miyazaki, M., Yano, H., & Seino, S. (2001) · Journal of Biological Chemistry · doi:10.1074/jbc.m108378200 · Cited by 381

A novel syndrome of diabetes mellitus, renal dysfunction and genital malformation associated with a partial deletion of the pseudo-POU domain of hepatocyte nuclear factor-1beta

Lindner, T. (1999) · Human Molecular Genetics · doi:10.1093/hmg/8.11.2001 · Cited by 367

Sur1 Knockout Mice

Seghers, V., Nakazaki, M., DeMayo, F., Aguilar-Bryan, L., & Bryan, J. (2000) · Journal of Biological Chemistry · doi:10.1074/jbc.275.13.9270 · Cited by 365

Beta-cell proliferation and apoptosis in the developing normal human pancreas and in hyperinsulinism of infancy.

Kassem, S. A., Ariel, I., Thornton, P. S., Scheimberg, I., & Glaser, B. (2000) · Diabetes · doi:10.2337/diabetes.49.8.1325 · Cited by 361

Pidd, a new death-domain–containing protein, is induced by p53 and promotes apoptosis

Lin, Y., Ma, W., & Benchimol, S. (2000) · Nature Genetics · doi:10.1038/79102 · Cited by 353

Transcriptional activation of the GLUT2 gene by the IPF-1/STF-1/IDX-1 homeobox factor

Waeber, G. (1996) · Molecular Endocrinology · doi:10.1210/me.10.11.1327 · Cited by 348

Neurologic outcomes of 90 neonates and infants with persistent hyperinsulinemic hypoglycemia

Menni, F., de Lonlay, P., Sevin, C., Touati, G., Peigné, C., Barbier, V., Nihoul-Fékété, C., Saudubray, J.-M., & Robert, J.-J. (2001) · PEDIATRICS · doi:10.1542/peds.107.3.476 · Cited by 347

A recessive contiguous gene deletion causing infantile hyperinsulinism, enteropathy and deafness identifies the Usher type 1C gene

Bitner-Glindzicz, M., Lindley, K. J., Rutland, P., Blaydon, D., Smith, V. V., Milla, P. J., Hussain, K., Furth-Lavi, J., Cosgrove, K. E., Shepherd, R. M., et al. (2000) · Nature Genetics · doi:10.1038/79178 · Cited by 324

Epigenetic lesions at the H19 locus in Wilms' tumour patients

Moulton, T., Crenshaw, T., Hao, Y., Moosikasuwan, J., Lin, N., Dembitzer, F., Hensle, T., Weiss, L., McMorrow, L., Loew, T., et al. (1994) · Nature Genetics · doi:10.1038/ng0794-440 · Cited by 316

Hyperinsulinism in short-chain L-3-hydroxyacyl-CoA dehydrogenase deficiency reveals the importance of β-oxidation in insulin secretion

Clayton, P. T., Eaton, S., Aynsley-Green, A., Edginton, M., Hussain, K., Krywawych, S., Datta, V., Malingré, H. E. M., Berger, R., & van den Berg, I. E. T. (2001) · Journal of Clinical Investigation · doi:10.1172/jci11294 · Cited by 312

Somatic deletion of the imprinted 11p15 region in sporadic persistent hyperinsulinemic hypoglycemia of infancy is specific of focal adenomatous hyperplasia and endorses partial pancreatectomy.

de Lonlay, P., Fournet, J. C., Rahier, J., Gross-Morand, M. S., Poggi-Travert, F., Foussier, V, Bonnefont, J. P., Brusset, M. C., Brunelle, F., Robert, J. J., et al. (1997) · Journal of Clinical Investigation · doi:10.1172/jci119594 · Cited by 306

Practical management of hyperinsulinism in infancy

Aynsley-Green, A., Hussain, K., Hall, J., Saudubray, J. M., Nihoul-Fékété, C., De Lonlay-Debeney, P., Brunelle, F., Otonkoski, T., Thornton, P., & Lindley, K. J. (2000) · Archives of Disease in Childhood - Fetal and Neonatal Edition · doi:10.1136/fn.82.2.f98 · Cited by 298

Clinical Features of 52 Neonates with Hyperinsulinism

de Lonlay-Debeney, P., Poggi-Travert, F., Fournet, J.-C., Sempoux, C., Vici, C. D., Brunelle, F., Touati, G., Rahier, J., Junien, C., Nihoul-Fékété, C., et al. (1999) · New England Journal of Medicine · doi:10.1056/nejm199904153401505 · Cited by 294

Molecular cloning and functional expression of a brain-specific somatostatin receptor.

Bruno, J. F., Xu, Y., Song, J., & Berelowitz, M. (1992) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.89.23.11151 · Cited by 294

Triggering and Augmentation Mechanisms, Granule Pools, and Biphasic Insulin Secretion

Bratanova-Tochkova, T. K., Cheng, H., Daniel, S., Gunawardana, S., Liu, Y.-J., Mulvaney-Musa, J., Schermerhorn, T., Straub, S. G., Yajima, H., & Sharp, G. W. G. (2002) · Diabetes · doi:10.2337/diabetes.51.2007.s83 · Cited by 291

Paternal mutation of the sulfonylurea receptor (SUR1) gene and maternal loss of 11p15 imprinted genes lead to persistent hyperinsulinism in focal adenomatous hyperplasia.

Verkarre, V, Fournet, J. C., de Lonlay, P., Gross-Morand, M. S., Devillers, M., Rahier, J., Brunelle, F., Robert, J. J., Nihoul-Fékété, C., Saudubray, J. M., et al. (1998) · Journal of Clinical Investigation · doi:10.1172/jci4495 · Cited by 290

Age as Independent Determinant of Glucose Tolerance

Shimokata, H., Muller, D. C., Fleg, J. L., Sorkin, J., Ziemba, A. W., & Andres, R. (1991) · Diabetes · doi:10.2337/diab.40.1.44 · Cited by 289

Targeted Overactivity of β Cell KATP Channels Induces Profound Neonatal Diabetes

Koster, J. C., Marshall, B. A., Ensor, N., Corbett, J. A., & Nichols, C. G. (2000) · Cell · doi:10.1016/s0092-8674(00)80701-1 · Cited by 287

Subtype-selective expression of the five somatostatin receptors (hSSTR1-5) in human pancreatic islet cells: a quantitative double-label immunohistochemical analysis.

Kumar, U., Sasi, R., Suresh, S., Patel, A., Thangaraju, M., Metrakos, P., Patel, S. C., & Patel, Y. C. (1999) · Diabetes · doi:10.2337/diabetes.48.1.77 · Cited by 284

Dominantly inherited hyperinsulinism caused by a mutation in the sulfonylurea receptor type 1

Huopio, H., Reimann, F., Ashfield, R., Komulainen, J., Lenko, H.-L., Rahier, J., Vauhkonen, I., Kere, J., Laakso, M., Ashcroft, F., et al. (2000) · Journal of Clinical Investigation · doi:10.1172/jci9804 · Cited by 276

Nesidioblastoma, the islet tumor of the pancreas.

(1938) · PubMed Central · Cited by 272

Identification of 14 new glucokinase mutations and description of the clinical profile of 42 MODY-2 families

Velho, G., Blanché, H., Vaxillaire, M., Bellanné-Chantelot, C., Pardini, V. C., Timsit, J., Passa, P., Deschamps, I., Robert, J.-J., Weber, I. T., et al. (1997) · Diabetologia · doi:10.1007/s001250050666 · Cited by 266

A Role for Malonyl-CoA in Glucose-Stimulated Insulin Secretion from Clonal Pancreatic β-Cells

Corkey, B. E., Glennon, M. C., Chen, K. S., Deeney, J. T., Matschinsky, F. M., & Prentki, M. (1989) · Journal of Biological Chemistry · doi:10.1016/s0021-9258(20)88227-1 · Cited by 265

Macroglossia, omphalocele, adrenal cytomegaly, gigantism, and hyperplastic visceromegaly

Beckwith, J. (1969) · Birth Defects · Cited by 262

Electrophysiology of the pancreas.

Petersen, O. H., & Findlay, I. (1987) · Physiological Reviews · doi:10.1152/physrev.1987.67.3.1054 · Cited by 259

Phosphorylation of 25-kDa Synaptosome-associated Protein

Shimazaki, Y., Nishiki, T.-I., Omori, A., Sekiguchi, M., Kamata, Y., Kozaki, S., & Takahashi, M. (1996) · Journal of Biological Chemistry · doi:10.1074/jbc.271.24.14548 · Cited by 246

FAMILIAL HYPOGLYCEMIA PRECIPITATED BY AMINO ACIDS

Cochrane, W. A., Payne, W. W., Simpkiss, M. J., & Woolf, L. I. (1956) · Journal of Clinical Investigation · doi:10.1172/jci103292 · Cited by 245

Mutations in the sulonylurea receptor gene are associated with familial hyperinsulinism in Ashkenazi Jews

Nestorowicz, A. (1996) · Human Molecular Genetics · doi:10.1093/hmg/5.11.1813 · Cited by 245

Membrane topology distinguishes a subfamily of the ATP‐binding cassette (ABC) transporters

Tusnády, G. E., Bakos, É., Váradi, A., & Sarkadi, B. (1997) · FEBS Letters · doi:10.1016/s0014-5793(96)01478-0 · Cited by 244

KIR6.2 Polymorphism Predisposes to Type 2 Diabetes by Inducing Overactivity of Pancreatic β-Cell ATP-Sensitive K+ Channels

Schwanstecher, C., Meyer, U., & Schwanstecher, M. (2002) · Diabetes · doi:10.2337/diabetes.51.3.875 · Cited by 243

The ATP‐sensitivity of K+ channels in rat pancreatic B‐cells is modulated by ADP

Kakei, M., Kelly, R. P., Ashcroft, S. J. H., & Ashcroft, F. M. (1986) · FEBS Letters · doi:10.1016/0014-5793(86)81533-2 · Cited by 241

All Five Cloned Human Somatostatin Receptors (hSSTR1-5) Are Functionally Coupled to Adenylyl Cyclase

Patel, Y. C., Greenwood, M. T., Warszynska, A., Panetta, R., & Srikant, C. B. (1994) · Biochemical and Biophysical Research Communications · doi:10.1006/bbrc.1994.1088 · Cited by 239

A Nonsense Mutation in the Inward Rectifier Potassium Channel Gene, Kir6.2, Is Associated With Familial Hyperinsulinism

Nestorowicz, A., Inagaki, N., Gonoi, T., Schoor, K. P., Wilson, B. A., Glaser, B., Landau, H., Stanley, C. A., Thornton, P. S., Seino, S., et al. (1997) · Diabetes · doi:10.2337/diab.46.11.1743 · Cited by 237

Intracellular ADP activates K+ channels that are inhibited by ATP in an insulin‐secreting cell line

Dunne, M. J., & Petersen, O. H. (1986) · FEBS Letters · doi:10.1016/0014-5793(86)81532-0 · Cited by 229

A Subset of 50 Secretory Granules in Close Contact With l-Type Ca2+ Channels Accounts for First-Phase Insulin Secretion in Mouse β-Cells

Barg, S., Eliasson, L., Renström, E., & Rorsman, P. (2002) · Diabetes · doi:10.2337/diabetes.51.2007.s74 · Cited by 227

Loss of functional KATP channels in pancreatic β–cells causes persistent hyperinsulinemic hypoglycemia of infancy

Kane, C., Shepherd, R. M., Squires, P. E., Johnson, P. R. V., James, R. F. L., Milla, P. J., Aynsley-Green, A., Lindley, K. J., & Dunne, M. J. (1996) · Nature Medicine · doi:10.1038/nm1296-1344 · Cited by 225

Molecular determinants of presynaptic active zones

Garner, C. C., Kindler, S., & Gundelfinger, E. D. (2000) · Current Opinion in Neurobiology · doi:10.1016/s0959-4388(00)00093-3 · Cited by 223

Molecular determinants of KATP channel inhibition by ATP

Tucker, S. J. (1998) · The EMBO Journal · doi:10.1093/emboj/17.12.3290 · Cited by 222

The Human Glucokinase Gene β-Cell-Type Promoter: An Essential Role of Insulin Promoter Factor 1/PDX-1 in Its Activation in HIT-T15 Cells

Watada, H., Kajimoto, Y., Umayahara, Y., Matsuoka, T.-A., Kaneto, H., Fujitani, Y., Kamada, T., Kawamori, R., & Yamasaki, Y. (1996) · Diabetes · doi:10.2337/diab.45.11.1478 · Cited by 221

Familial Persistent Hyperinsulinemic Hypoglycemia of Infancy and Mutations in the Sulfonylurea Receptor

Dunne, M. J., Kane, C., Shepherd, R. M., Sanchez, J. A., James, R. F. L., Johnson, P. R. V., Aynsley-Green, A., Lu, S., Clement, J. P., Lindley, K. J., et al. (1997) · New England Journal of Medicine · doi:10.1056/nejm199703063361005 · Cited by 221

Genetics of neonatal hyperinsulinism

Glaser, B., Thornton, P., Otonkoski, T., & Junien, C. (2000) · Archives of Disease in Childhood - Fetal and Neonatal Edition · doi:10.1136/fn.82.2.f79 · Cited by 221

Impaired hepatic glycogen synthesis in glucokinase-deficient (MODY-2) subjects.

Velho, G., Petersen, K. F., Perseghin, G., Hwang, J. H., Rothman, D. L., Pueyo, M. E., Cline, G. W., Froguel, P., & Shulman, G. I. (1996) · Journal of Clinical Investigation · doi:10.1172/jci118974 · Cited by 217

Maternally inherited diabetes and deafness is a distinct subtype of diabetes and associates with a single point mutation in the mitochondrial tRNA Leu(UUR) gene

van den Ouweland, J. M. W., Lemkes, H. H. P. J., Trembath, R. C., Ross, R., Velho, G., Cohen, D., Froguel, P., & Maassen, J. A. (1994) · Diabetes · doi:10.2337/diab.43.6.746 · Cited by 217

Targeted Expression of Placental Lactogen in the Beta Cells of Transgenic Mice Results in Beta Cell Proliferation, Islet Mass Augmentation, and Hypoglycemia

Vasavada, R. C., Garcia-Ocaña, A., Zawalich, W. S., Sorenson, R. L., Dann, P., Syed, M., Ogren, L., Talamantes, F., & Stewart, A. F. (2000) · Journal of Biological Chemistry · doi:10.1074/jbc.275.20.15399 · Cited by 212

The N-Ethylmaleimide-sensitive Fusion Protein and α-SNAP Induce a Conformational Change in Syntaxin

Hanson, P. I., Otto, H., Barton, N., & Jahn, R. (1995) · Journal of Biological Chemistry · doi:10.1074/jbc.270.28.16955 · Cited by 209

Transcriptional regulation of the human insulin gene is dependent on the homeodomain protein STF1/IPF1 acting through the CT boxes.

Petersen, H. V., Serup, P., Leonard, J., Michelsen, B. K., & Madsen, O. D. (1994) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.91.22.10465 · Cited by 207

Nesidioblastosis of the pancreas: definition of the syndrome and the management of the severe neonatal hyperinsulinaemic hypoglycaemia.

Aynsley-Green, A., Polak, J. M., Bloom, S. R., Gough, M. H., Keeling, J., Ashcroft, S. J., Turner, R. C., & Baum, J. D. (1981) · Archives of Disease in Childhood · doi:10.1136/adc.56.7.496 · Cited by 207

Are Metabolic Oscillations Responsible for Normal Oscillatory Insulin Secretion?

Tornheim, K. (1997) · Diabetes · doi:10.2337/diab.46.9.1375 · Cited by 203

Structural Determinants of Pip2 Regulation of Inward Rectifier KATP Channels

Shyng, S.-L., Cukras, C. A., Harwood, J., & Nichols, C. G. (2000) · The Journal of General Physiology · doi:10.1085/jgp.116.5.599 · Cited by 202

Specificity of activation by phosphoinositides determines lipid regulation of Kir channels

Rohács, T., Lopes, C. M. B., Jin, T., Ramdya, P. P., Molnár, Z., & Logothetis, D. E. (2003) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.0236364100 · Cited by 199

Piccolo, a Ca2+ Sensor in Pancreatic β-Cells

Fujimoto, K., Shibasaki, T., Yokoi, N., Kashima, Y., Matsumoto, M., Sasaki, T., Tajima, N., Iwanaga, T., & Seino, S. (2002) · Journal of Biological Chemistry · doi:10.1074/jbc.m210146200 · Cited by 197

Noninsulinoma Pancreatogenous Hypoglycemia: A Novel Syndrome of Hyperinsulinemic Hypoglycemia in Adults Independent of Mutations in Kir6.2 and SUR1 Genes1

Service, F. J., Natt, N., Thompson, G. B., Grant, C. S., van Heerden, J. A., Andrews, J. C., Lorenz, E., Terzic, A., & Lloyd, R. V. (1999) · The Journal of Clinical Endocrinology & Metabolism · doi:10.1210/jcem.84.5.5645 · Cited by 196

Priming of insulin granules for exocytosis by granular Cl(-) uptake and acidification

Barg, S., Huang, P., Eliasson, L., Nelson, D. J., Obermüller, S., Rorsman, P., Thévenod, F., & Renström, E. (2001) · Journal of Cell Science · doi:10.1242/jcs.114.11.2145 · Cited by 194

K ATP channel inhibition by ATP requires distinct functional domains of the cytoplasmic C terminus of the pore-forming subunit

Drain, P., Li, L., & Wang, J. (1998) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.95.23.13953 · Cited by 193

New insights into pancreatic β-cell metabolic signaling in insulin secretion

Prentki, M. (1996) · European Journal of Endocrinology · doi:10.1530/eje.0.1340272 · Cited by 192

Overexpression of monocarboxylate transporter and lactate dehydrogenase alters insulin secretory responses to pyruvate and lactate in β cells

Ishihara, H., Wang, H., Drewes, L. R., & Wollheim, C. B. (1999) · Journal of Clinical Investigation · doi:10.1172/jci7515 · Cited by 191

Increased Activity of L-Type Ca 2+ Channels Exposed to Serum from Patients with Type I Diabetes

Juntti-Berggren, L., Larsson, O., Rorsman, P., Ämmälä, C., Bokvist, K., Wåhlander, K., Nicotera, P., Dypbukt, J., Orrenius, S., Hallberg, A., et al. (1993) · Science · doi:10.1126/science.7686306 · Cited by 187

Tissue-specific deletion of Foxa2 in pancreatic β cells results in hyperinsulinemic hypoglycemia

Sund, N. J., Vatamaniuk, M. Z., Casey, M., Ang, S.-L., Magnuson, M. A., Stoffers, D. A., Matschinsky, F. M., & Kaestner, K. H. (2001) · Genes & Development · doi:10.1101/gad.901601 · Cited by 186

Transgenic Reexpression of GLUT1 or GLUT2 in Pancreatic β Cells Rescues GLUT2-null Mice from Early Death and Restores Normal Glucose-stimulated Insulin Secretion

Thorens, B., Guillam, M.-T., Beermann, F., Burcelin, R., & Jaquet, M. (2000) · Journal of Biological Chemistry · doi:10.1074/jbc.m002908200 · Cited by 185

Molecular genetics of Wiedemann-Beckwith syndrome

Li, M., Squire, J. A., & Weksberg, R. (1998) · American Journal of Medical Genetics · doi:10.1002/(sici)1096-8628(19981002)79:4<253::aid-ajmg5>3.0.co;2-n · Cited by 185

A new subtype of autosomal dominant diabetes attributable to a mutation in the gene for sulfonylurea receptor 1

Huopio, H., Otonkoski, T., Vauhkonen, I., Reimann, F., Ashcroft, F. M., & Laakso, M. (2003) · The Lancet · doi:10.1016/s0140-6736(03)12325-2 · Cited by 184

Stimulated Ca2+ influx raises mitochondrial free Ca2+ to supramicromolar levels in a pancreatic beta-cell line. Possible role in glucose and agonist-induced insulin secretion.

Rutter, G. A., Theler, J. M., Murgia, M., Wollheim, C. B., Pozzan, T., & Rizzuto, R. (1993) · Journal of Biological Chemistry · doi:10.1016/s0021-9258(18)41540-2 · Cited by 182

Targeted disruption of the human LIT1 locus defines a putative imprinting control element playing an essential role in Beckwith-Wiedemann syndrome

Horike, S.-I. (2000) · Human Molecular Genetics · doi:10.1093/hmg/9.14.2075 · Cited by 181

Abnormalities of pancreatic islets by targeted expression of a dominant-negative K ATP channel

Miki, T., Tashiro, F., Iwanaga, T., Nagashima, K., Yoshitomi, H., Aihara, H., Nitta, Y., Gonoi, T., Inagaki, N., Miyazaki, J.-I., et al. (1997) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.94.22.11969 · Cited by 180

Glucose modulates the binding of an islet-specific factor to a conserved sequence within the rat I and the human insulin promoters.

Melloul, D., Ben-Neriah, Y., & Cerasi, E. (1993) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.90.9.3865 · Cited by 178

Transgenic mice overexpressing insulin-like growth factor-II in β cells develop type 2 diabetes

Devedjian, J.-C., George, M., Casellas, A., Pujol, A., Visa, J., Pelegrín, M., Gros, L., & Bosch, F. (2000) · Journal of Clinical Investigation · doi:10.1172/jci5656 · Cited by 178

Acute Stimulation with Long Chain Acyl-CoA Enhances Exocytosis in Insulin-secreting Cells (HIT T-15 and NMRI β-Cells)

Deeney, J. T., Gromada, J., Høy, M., Olsen, H. L., Rhodes, C. J., Prentki, M., Berggren, P.-O., & Corkey, B. E. (2000) · Journal of Biological Chemistry · doi:10.1074/jbc.275.13.9363 · Cited by 176

Purification of the β-cell glucose-sensitive factor that transactivates the insulin gene differentially in normal and transformed islet cells

Marshak, S., Totary, H., Cerasi, E., & Melloul, D. (1996) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.93.26.15057 · Cited by 173

Molecular basis and characterization of the hyperinsulinism/hyperammonemia syndrome: predominance of mutations in exons 11 and 12 of the glutamate dehydrogenase gene. HI/HA Contributing Investigators.

Stanley, C. A., Fang, J., Kutyna, K., Hsu, B. Y., Ming, J. E., Glaser, B., & Poncz, M. (2000) · Diabetes · doi:10.2337/diabetes.49.4.667 · Cited by 171

The Second Activating Glucokinase Mutation (A456V)

Christesen, H. B. T., Jacobsen, B. B., Odili, S., Buettger, C., Cuesta-Munoz, A., Hansen, T., Brusgaard, K., Massa, O., Magnuson, M. A., Shiota, C., et al. (2002) · Diabetes · doi:10.2337/diabetes.51.4.1240 · Cited by 171

Transgenic tumor necrosis factor (TNF)-alpha production in pancreatic islets leads to insulitis, not diabetes. Distinct patterns of inflammation in TNF-alpha and TNF-beta transgenic mice

Picarella, D. E., Kratz, A., Li, C. B., Ruddle, N. H., & Flavell, R. A. (1993) · The Journal of Immunology · doi:10.4049/jimmunol.150.9.4136 · Cited by 168

Activation of the ATP-sensitive K+ Channel by Long Chain Acyl-CoA

Larsson, O., Deeney, J. T., Bränström, R., Berggren, P.-O., & Corkey, B. E. (1996) · Journal of Biological Chemistry · doi:10.1074/jbc.271.18.10623 · Cited by 166

Functional analyses of novel mutations in the sulfonylurea receptor 1 associated with persistent hyperinsulinemic hypoglycemia of infancy.

Shyng, S. L., Ferrigni, T., Shepard, J. B., Nestorowicz, A., Glaser, B., Permutt, M. A., & Nichols, C. G. (1998) · Diabetes · doi:10.2337/diabetes.47.7.1145 · Cited by 165

Signal transduction mechanisms in nutrient-induced insulin secretion

Prentki, M., Tornheim, K., & Corkey, B. E. (1997) · Diabetologia · doi:10.1007/s001250051395 · Cited by 164

Regulation of ATP-sensitive K+ channels in insulinoma cells: activation by somatostatin and protein kinase C and the role of cAMP.

de Weille, J. R., Schmid-Antomarchi, H., Fosset, M., & Lazdunski, M. (1989) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.86.8.2971 · Cited by 164

Defective trafficking and function of K ATP channels caused by a sulfonylurea receptor 1 mutation associated with persistent hyperinsulinemic hypoglycemia of infancy

Cartier, E. A., Conti, L. R., Vandenberg, C. A., & Shyng, S.-L. (2001) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.051499698 · Cited by 163

Protein kinase C isotypes and signaling in neutrophils. Differential substrate specificities of a translocatable calcium- and phospholipid-dependent beta-protein kinase C and a phospholipid-dependent protein kinase which is inhibited by long chain fatty acyl coenzyme A

Majumdar, S., Rossi, M. W., Fujiki, T., Phillips, W. A., Disa, S., Queen, C. F., Johnston, R. B., Rosen, O. M., Corkey, B. E., & Korchak, H. M. (1991) · Journal of Biological Chemistry · doi:10.1016/s0021-9258(18)31582-5 · Cited by 162

New perspectives on pancreatic islet glucokinase

Meglasson, M. D., & Matschinsky, F. M. (1984) · American Journal of Physiology-Endocrinology and Metabolism · doi:10.1152/ajpendo.1984.246.1.e1 · Cited by 162

Nesidioblastosis: The Pathologic Basis of Persistent Hyperinsulinemic Hypoglycemia in Infants: Morphologic and Quantitative Analysis of Seven Cases Based on Specific Immunostaining and Electron Microscopy

Heitz, P. U., Klöppel, G., Häcki, W. H., Polak, J. M., & Pearse, A. G. E. (1977) · Diabetes · doi:10.2337/diab.26.7.632 · Cited by 162

Pleiotropic effects of fatty acids on pancreatic β‐cells

Haber, E. P., Ximenes, H. M. A., Procópio, J., Carvalho, C. R. O., Curi, R., & Carpinelli, A. R. (2003) · Journal of Cellular Physiology · doi:10.1002/jcp.10187 · Cited by 160

Persistent hyperinsulinemic hypoglycemia of infancy: Long-term octreotide treatment without pancreatectomy

Glaser, B., Hirsch, H. J., & Landau, H. (1993) · The Journal of Pediatrics · doi:10.1016/s0022-3476(05)80970-9 · Cited by 158

The basic structural lesion of persistent neonatal hypoglycaemia with hyperinsulinism: deficiency of pancreatic D cells or hyperactivity of B cells?

Rahier, J., F�lt, K., M�ntefering, H., Becker, K., Gepts, W., & Falkmer, S. (1984) · Diabetologia · doi:10.1007/bf00283651 · Cited by 158

Cooperative binding of ATP and MgADP in the sulfonylurea receptor is modulated by glibenclamide.

Ueda, K., Komine, J., Matsuo, M., Seino, S., & Amachi, T. (1999) · Proceedings of the National Academy of Sciences of the United States of America · doi:10.1073/pnas.96.4.1268 · Cited by 157

Hyperinsulinemic hypoglycemia of infancy (nesidioblastosis) in clinical remission: high incidence of diabetes mellitus and persistent beta-cell dysfunction at long-term follow-up

Leibowitz, G., Glaser, B., Higazi, A. A., Salameh, M., Cerasi, E., & Landau, H. (1995) · The Journal of Clinical Endocrinology & Metabolism · doi:10.1210/jcem.80.2.7852494 · Cited by 156

A point mutation inactivating the sulfonylurea receptor causes the severe form of persistent hyperinsulinemic hypoglycemia of infancy in Finland.

Otonkoski, T., Ämmälä, C., Huopio, H., Cote, G. J., Chapman, J., Cosgrove, K. E., Ashfield, R., Huang, E. S., Komulainen, J., Ashcroft, F. M., et al. (1999) · Diabetes · doi:10.2337/diabetes.48.2.408 · Cited by 155

Glucose uptake, utilization, and signaling in GLUT2-null islets.

Guillam, M., Dupraz, P., & Thorens, B. (2000) · Diabetes · doi:10.2337/diabetes.49.9.1485 · Cited by 155

Nonselective and G βγ -Insensitive weaver K + Channels

Navarro, B., Kennedy, M. E., Velimirovic, B. M., Bhat, D., Peterson, A. S., & Clapham, D. E. (1996) · Science · doi:10.1126/science.272.5270.1950 · Cited by 153

Hyperinsulinism/Hyperammonemia Syndrome in Children with Regulatory Mutations in the Inhibitory Guanosine Triphosphate-Binding Domain of Glutamate Dehydrogenase

MacMullen, C., Fang, J., Hsu, B. Y. L., Kelly, A. T., de Lonlay, P., Saudubray, J., Ganguly, A. A., Smith, T. J., Stanley, C. A., & The Hyperinsulinism/Hyperammonemia Contributing Investigators (2001) · The Journal of Clinical Endocrinology & Metabolism · doi:10.1210/jcem.86.4.7414 · Cited by 153

Potassium selective ion channels in insulin-secreting cells: physiology, pharmacology and their role in stimulus-secretion coupling

Dunne, M. J., & Petersen, O. H. (1991) · Biochimica et Biophysica Acta (BBA) - Reviews on Biomembranes · doi:10.1016/0304-4157(91)90012-l · Cited by 152

Diverse roles of K(ATP) channels learned from Kir6.2 genetically engineered mice.

Seino, S., Iwanaga, T., Nagashima, K., & Miki, T. (2000) · Diabetes · doi:10.2337/diabetes.49.3.311 · Cited by 151

Heterogeneity of persistent hyperinsulinaemic hypoglycaemia. A series of 175 cases

de Lonlay, P., Fournet, J., Touati, G., Groos, M.-S., Martin, D., Sevin, C., Delagne, V., Mayaud, C., Chigot, V., Sempoux, C. L., et al. (2001) · European Journal of Pediatrics · doi:10.1007/s004310100847 · Cited by 151

Glycemic response to glucagon during fasting hypoglycemia: an aid in the diagnosis of hyperinsulinism

Finegold, D. N., Stanley, C. A., & Baker, L. (1980) · The Journal of Pediatrics · doi:10.1016/s0022-3476(80)80817-1 · Cited by 150

Evidence for an Anaplerotic/Malonyl-CoA Pathway in Pancreatic β-Cell Nutrient Signaling

Brun, T., Roche, E., Assimacopoulos-Jeannet, F., Corkey, B., Kim, K.-H., & Prentki, M. (1996) · Diabetes · doi:10.2337/diab.45.2.190 · Cited by 150

Physical Exercise-Induced Hyperinsulinemic Hypoglycemia Is an Autosomal-Dominant Trait Characterized by Abnormal Pyruvate-Induced Insulin Release

Otonkoski, T., Kaminen‐Ahola, N., Ustinov, J., Lapatto, R., Meißner, T., Mayatepek, E., Kere, J., & Sipilä, I. (2003) · Diabetes · doi:10.2337/diabetes.52.1.199 · Cited by 150

Molecular or Pharmacologic Perturbation of the Link between Glucose and Lipid Metabolism Is without Effect on Glucose-stimulated Insulin Secretion

Antinozzi, P. A., Segall, L., Prentki, M., McGarry, J., & Newgard, C. B. (1998) · Journal of Biological Chemistry · doi:10.1074/jbc.273.26.16146 · Cited by 149

Persistent hyperinsulinaemic hypoglycaemia of infancy: a heterogeneous syndrome unrelated to nesidioblastosis

Rahier, J., Guiot, Y., & Sempoux, C. L. (2000) · Archives of Disease in Childhood Fetal & Neonatal · doi:10.1136/fn.82.2.f108 · Cited by 149

Metabolic control of -cell function

Deeney, J. T., Prentki, M., & Corkey, B. E. (2000) · Seminars in Cell and Developmental Biology · doi:10.1006/scdb.2000.0175 · Cited by 147

Diffuse and Focal Nesidioblastosis

Goossens, A., Gepts, W., Saudubray, J., Bonnefont, J., Nihoul‐Fekete, Heitz, & Klöppel, G. (1989) · The American Journal of Surgical Pathology · doi:10.1097/00000478-198909000-00006 · Cited by 147

Different Binding Properties and Affinities for ATP and ADP among Sulfonylurea Receptor Subtypes, SUR1, SUR2A, and SUR2B

Matsuo, M., Tanabe, K., Kioka, N., Amachi, T., & Ueda, K. (2000) · Journal of Biological Chemistry · doi:10.1074/jbc.m004818200 · Cited by 144

Somatostatin inhibits insulin secretion by a G-protein-mediated decrease in Ca2+ entry through voltage-dependent Ca2+ channels in the beta cell.

Hsu, W. H., Xiang, H. D., Rajan, A. S., Kunze, D. L., & BOYD, A. E. (1991) · Journal of Biological Chemistry · doi:10.1016/s0021-9258(17)35249-3 · Cited by 140

Identification of nine novel mutations in the hepatocyte nuclear factor 1 alpha gene associated with maturity-onset diabetes of the young (MODY3)

Vaxillaire, M. (1997) · Human Molecular Genetics · doi:10.1093/hmg/6.4.583 · Cited by 139

The C Terminus of SUR1 Is Required for Trafficking of KATP Channels

Sharma, N., Crane, A., Clement, J. P., González, G., Babenko, A. P., Bryan, J., & Aguilar‐Bryan, L. (1999) · Journal of Biological Chemistry · doi:10.1074/jbc.274.29.20628 · Cited by 138

Evidence for the malate aspartate shuttle in pancreatic islets

MacDonald, M. J. (1982) · Archives of Biochemistry and Biophysics · doi:10.1016/0003-9861(82)90594-x · Cited by 137

Suppression of Insulin Release by Galanin and Somatostatin Is Meditated by a G-protein

Nilsson, T., Arkhammar, P. O. G., Rorsman, P., & Berggren, P. (1989) · Journal of Biological Chemistry · doi:10.1016/s0021-9258(19)85039-1 · Cited by 133

Mechanism of Cloned ATP-sensitive Potassium Channel Activation by Oleoyl-CoA

Gribble, F. M., Proks, P., Corkey, B. E., & Ashcroft, F. M. (1998) · Journal of Biological Chemistry · doi:10.1074/jbc.273.41.26383 · Cited by 133

Control of Rectification and Gating of Cloned KATP Channels by the Kir6.2 Subunit

Shyng, S., Ferrigni, T., & Nichols, C. G. (1997) · The Journal of General Physiology · doi:10.1085/jgp.110.2.141 · Cited by 132

Dehydrogenase activation by Ca2+ in cells and tissues

Hansford, R. G. (1991) · Journal of Bioenergetics and Biomembranes · doi:10.1007/bf00786004 · Cited by 129

Dysregulation of Insulin Secretion in Children With Congenital Hyperinsulinism due to Sulfonylurea Receptor Mutations

Grimberg, A., Ferry, R. J., Kelly, A. T., Koo-McCoy, S., Polonsky, K. S., Gläser, B., Permutt, M. A., Aguilar‐Bryan, L., Stafford, D. E. J., THORNTON, P. S., et al. (2001) · Diabetes · doi:10.2337/diabetes.50.2.322 · Cited by 127

Hyperinsulinism in Infancy: Diagnosis by Demonstration of Abnormal Response to Fasting Hypoglycemia

Stanley, C. A., & Baker, L. (1976) · PEDIATRICS · doi:10.1542/peds.57.5.702 · Cited by 126

Identification of the Potassium Channel Opener Site on Sulfonylurea Receptors

Uhde, I., Toman, A., Gross, I., Schwanstecher, C., & Schwanstecher, M. (1999) · Journal of Biological Chemistry · doi:10.1074/jbc.274.40.28079 · Cited by 124

Identification of a Familial Hyperinsulinism-causing Mutation in the Sulfonylurea Receptor 1 That Prevents Normal Trafficking and Function of KATP Channels

Taschenberger, G., Mougey, A., Shen, S., Lester, L. B., LAFRANCHI, S. H., & Shyng, S. (2002) · Journal of Biological Chemistry · doi:10.1074/jbc.m200363200 · Cited by 123

Insulitis in transgenic mice expressing tumor necrosis factor beta (lymphotoxin) in the pancreas.

Picarella, D., Kratz, A., Li, C. B., Ruddle, N. H., & Flavell, R. A. (1992) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.89.21.10036 · Cited by 122

Genetic Heterogeneity in Familial Hyperinsulinism

Nestorowicz, A., Gläser, B., Wilson, B. A., Shyng, S., Nichols, C. G., Stanley, C. A., THORNTON, P. S., & Permutt, M. A. (1998) · Human Molecular Genetics · doi:10.1093/hmg/7.7.1119 · Cited by 122

Energy metabolism in islets of Langerhans

Erecińska, M., Bryła, J., Michalik, M., Meglasson, M. D., & NELSON, D. S. (1992) · Biochimica et Biophysica Acta (BBA) - Bioenergetics · doi:10.1016/0005-2728(92)90084-f · Cited by 118

The malonyl-CoA‒long-chain acyl-CoA axis in the maintenance of mammalian cell function

Zammit, V. A. (1999) · Biochemical Journal · doi:10.1042/bj3430505 · Cited by 118

Hrs-2 is an ATPase implicated in calcium-regulated secretion

Bean, A. J., Seifert, R., Chen, Y. A., Sacks, R., & Scheller, R. H. (1997) · Nature · doi:10.1038/385826a0 · Cited by 116

Long-Chain Acyl CoA Regulation of Protein Kinase C and Fatty Acid Potentiation of Glucose-Stimulated Insulin Secretion in Clonal -Cells

Yaney, G. C., Korchak, H. M., & Corkey, B. E. (2000) · Endocrinology · doi:10.1210/endo.141.6.7493 · Cited by 115

The role of ATP and free ADP in metabolic coupling during fuel-stimulated insulin release from islet beta-cells in the isolated perfused rat pancreas.

Ghosh, A. K., Ronner, P., Cheong, E., Khalid, P., & Matschinsky, F. M. (1991) · Journal of Biological Chemistry · doi:10.1016/s0021-9258(18)54437-9 · Cited by 114

Unbalanced Expression of 11p15 Imprinted Genes in Focal Forms of Congenital Hyperinsulinism

Fournet, J., Mayaud, C., de Lonlay, P., Gross, M., Verkarre, V., Castanet, M., Devillers, M., Rahier, J., Brunelle, F., Robert, J., et al. (2001) · American Journal Of Pathology · doi:10.1016/s0002-9440(10)64689-5 · Cited by 113

cAMP enhances insulin secretion by an action on the ATP-sensitive K+ channel-independent pathway of glucose signaling in rat pancreatic islets.

Yajima, H., Komatsu, M., Schermerhorn, T., Aizawa, T., Kaneko, T., Nagai, M., Sharp, G. W. G., & Hashizume, K. (1999) · Diabetes · doi:10.2337/diabetes.48.5.1006 · Cited by 112

Familial hyperinsulinism maps to chromosome 11p14–15.1, 30 cM centromeric to the insulin gene

Gläser, B., Chiu, K. C., Anker, R., Nestorowicz, A., Landau, H., Ben‐Bassat, H., Shlomai, Z., Kaiser, Ν., THORNTON, P. S., Stanley, C. A., et al. (1994) · Nature Genetics · doi:10.1038/ng0694-185 · Cited by 111

Molecular Genetics of Maturity-onset Diabetes of the Young

Froguel, P., & Velho, G. (1999) · Trends in Endocrinology and Metabolism · doi:10.1016/s1043-2760(98)00134-9 · Cited by 110

Partial or near‐total pancreatectomy for persistent neonatal hyperinsulinaemic hypoglycaemia: the pathologist's role

Rahier, Sempoux, C. L., Fournet, Poggi§, Brunelle, Nihoul‐Fekete, Saudubray, & Jaubert (1998) · Histopathology · doi:10.1046/j.1365-2559.1998.00326.x · Cited by 109

Is 95% pancreatectomy the procedure of choice for treatment of persistent hyperinsulinemic hypoglycemia of the neonate?

Shilyansky, J., Fisher, S. J., Cutz, E., Perlman, K., & Filler, R. M. (1997) · Journal of Pediatric Surgery · doi:10.1016/s0022-3468(97)90207-4 · Cited by 104

Identification and Pharmacological Correction of a Membrane Trafficking Defect Associated with a Mutation in the Sulfonylurea Receptor Causing Familial Hyperinsulinism

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Kelly, A. T., & Stanley, C. A. (2001) · Mental Retardation and Developmental Disabilities Research Reviews · doi:10.1002/mrdd.1040 · Cited by 100

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Touati, G., Poggi-Travert, F., de Baulny, H. O., Rahier, J., Brunelle, F., Nihoul‐Feketé, C., Czernichow, P., & Saudubray, J. (1998) · European Journal of Pediatrics · doi:10.1007/s004310050900 · Cited by 97

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Wang, H., Gauthier, B. R., Hagenfeldt-Johansson, K. A., Iezzi, M., & Wollheim, C. B. (2002) · Journal of Biological Chemistry · doi:10.1074/jbc.m111037200 · Cited by 96

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Matsuo, M., Kioka, N., Amachi, T., & Ueda, K. (1999) · Journal of Biological Chemistry · doi:10.1074/jbc.274.52.37479 · Cited by 96

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Malaisse, W. J., Best, L. C., Kawazu, S., MALAISSE-LAGAE, F., & Sener, A. (1983) · Archives of Biochemistry and Biophysics · doi:10.1016/0003-9861(83)90193-5 · Cited by 96

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Küster, U., Bohnensack, R., & Kunz, W. S. (1976) · Biochimica et Biophysica Acta (BBA) - Bioenergetics · doi:10.1016/0005-2728(76)90073-6 · Cited by 94

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Fang, J., Hsu, B. Y. L., MacMullen, C., Poncz, M., Smith, T. J., & Stanley, C. A. (2002) · Biochemical Journal · doi:10.1042/0264-6021:3630081 · Cited by 93

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Kalhan, S. C., Savin, S. M., & Adam, P. A. J. (1977) · New England Journal of Medicine · doi:10.1056/nejm197702172960706 · Cited by 88

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Lovvorn, H. N., Nance, M. L., Ferry, R. J., Stolte, L., Baker, L., O’Neill, J. A., Schnaufer, L., Stanley, C. A., & Adzick, N. S. (1999) · Journal of Pediatric Surgery · doi:10.1016/s0022-3468(99)90374-3 · Cited by 88

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Brunelle, F., Negre, V., Barth, M. O., et al. (1989) · Pediatric Radiology · doi:10.1007/bf02387895 · Cited by 88

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Lindley, K. J., Dunne, M. J., Kane, C. A., Shepherd, R. M., Squires, P. E., James, R. F. L., Johnson, P., Eckhardt, S., Wakeling, E. L., Dattani, M., et al. (1996) · Archives of Disease in Childhood · doi:10.1136/adc.74.5.373 · Cited by 88

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Carty, M. D., Lillquist, J. S., Peshavaria, M., Stein, R. W., & Soeller, W. C. (1997) · Journal of Biological Chemistry · doi:10.1074/jbc.272.18.11986 · Cited by 87

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Howell, S. L., & MONTAGUE, W. (1973) · Biochimica et Biophysica Acta (BBA) - General Subjects · doi:10.1016/0304-4165(73)90163-3 · Cited by 86

Glucose activates both K(ATP) channel-dependent and K(ATP) channel-independent signaling pathways in human islets.

Straub, S. G., James, R. F. L., Dunne, M. J., & Sharp, G. W. G. (1998) · Diabetes · doi:10.2337/diabetes.47.5.758 · Cited by 86

Acyl-CoA esters modulate intracellular Ca2+ handling by permeabilized clonal pancreatic beta-cells.

Deeney, J. T., Tornheim, K., Korchak, H. M., et al. (1992) · Journal of Biological Chemistry · doi:10.1016/s0021-9258(19)88631-3 · Cited by 86

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Civelek, V. N., Deeney, J. T., SHALOSKY, N. J., Tornheim, K., Hansford, R. G., Prentki, M., & Corkey, B. E. (1996) · Biochemical Journal · doi:10.1042/bj3180615 · Cited by 85

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Bränström, R., Corkey, B. E., Berggren, P., & Larsson, O. (1997) · Journal of Biological Chemistry · doi:10.1074/jbc.272.28.17390 · Cited by 83

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Zhao, W.-Q., & Townsend, M. (2009) · Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease · doi:10.1016/j.bbadis.2008.10.014 · Cited by 291