pubmed-article:11272144 | rdf:type | pubmed:Citation | lld:pubmed |
pubmed-article:11272144 | lifeskim:mentions | umls-concept:C0231330 | lld:lifeskim |
pubmed-article:11272144 | lifeskim:mentions | umls-concept:C0021641 | lld:lifeskim |
pubmed-article:11272144 | lifeskim:mentions | umls-concept:C0020459 | lld:lifeskim |
pubmed-article:11272144 | lifeskim:mentions | umls-concept:C1704259 | lld:lifeskim |
pubmed-article:11272144 | lifeskim:mentions | umls-concept:C0030685 | lld:lifeskim |
pubmed-article:11272144 | lifeskim:mentions | umls-concept:C1705987 | lld:lifeskim |
pubmed-article:11272144 | lifeskim:mentions | umls-concept:C0851285 | lld:lifeskim |
pubmed-article:11272144 | lifeskim:mentions | umls-concept:C0680255 | lld:lifeskim |
pubmed-article:11272144 | lifeskim:mentions | umls-concept:C0391871 | lld:lifeskim |
pubmed-article:11272144 | lifeskim:mentions | umls-concept:C1283071 | lld:lifeskim |
pubmed-article:11272144 | lifeskim:mentions | umls-concept:C1963578 | lld:lifeskim |
pubmed-article:11272144 | pubmed:issue | 2 | lld:pubmed |
pubmed-article:11272144 | pubmed:dateCreated | 2001-2-22 | lld:pubmed |
pubmed-article:11272144 | pubmed:databankReference | http://linkedlifedata.com/r... | lld:pubmed |
pubmed-article:11272144 | pubmed:abstractText | Hyperinsulinism of infancy (HI) is a congenital defect in the regulated release of insulin from pancreatic beta-cells. Here we describe stimulus-secretion coupling mechanisms in beta-cells and intact islets of Langerhans isolated from three patients with a novel SUR1 gene defect. 2154+3 A to G SUR1 (GenBank accession number L78207) is the first report of familial HI among nonconsanguineous Caucasians identified in the U.K. Using patch-clamp methodologies, we have shown that this mutation is associated with both a decrease in the number of operational ATP-sensitive K+ channels (KATP channels) in beta-cells and impaired ADP-dependent regulation. There were no apparent defects in the regulation of Ca2+- and voltage-gated K+ channels or delayed rectifier K+ channels. Intact HI beta-cells were spontaneously electrically active and generating Ca2+ action currents that were largely insensitive to diazoxide and somatostatin. As a consequence, when intact HI islets were challenged with glucose and tolbutamide, there was no rise in intracellular free calcium ion concentration ([Ca2+]i) over basal values. Capacitance measurements used to monitor exocytosis in control and HI beta-cells revealed that there were no defects in Ca2+-dependent exocytotic events. Finally, insulin release studies documented that whereas tolbutamide failed to cause insulin secretion as a consequence of impaired [Ca2+]i signaling, glucose readily promoted insulin release. Glucose was also found to augment the actions of protein kinase C- and protein kinase A-dependent agonists in the absence of extracellular Ca2+. These findings document the relationship between SUR1 gene defects and insulin secretion in vivo and in vitro and describe for the first time KATP channel-independent pathways of regulated insulin secretion in diseased human beta-cells. | lld:pubmed |
pubmed-article:11272144 | pubmed:grant | http://linkedlifedata.com/r... | lld:pubmed |
pubmed-article:11272144 | pubmed:grant | http://linkedlifedata.com/r... | lld:pubmed |
pubmed-article:11272144 | pubmed:language | eng | lld:pubmed |
pubmed-article:11272144 | pubmed:journal | http://linkedlifedata.com/r... | lld:pubmed |
pubmed-article:11272144 | pubmed:citationSubset | AIM | lld:pubmed |
pubmed-article:11272144 | pubmed:chemical | http://linkedlifedata.com/r... | lld:pubmed |
pubmed-article:11272144 | pubmed:chemical | http://linkedlifedata.com/r... | lld:pubmed |
pubmed-article:11272144 | pubmed:chemical | http://linkedlifedata.com/r... | lld:pubmed |
pubmed-article:11272144 | pubmed:chemical | http://linkedlifedata.com/r... | lld:pubmed |
pubmed-article:11272144 | pubmed:chemical | http://linkedlifedata.com/r... | lld:pubmed |
pubmed-article:11272144 | pubmed:chemical | http://linkedlifedata.com/r... | lld:pubmed |
pubmed-article:11272144 | pubmed:chemical | http://linkedlifedata.com/r... | lld:pubmed |
pubmed-article:11272144 | pubmed:chemical | http://linkedlifedata.com/r... | lld:pubmed |
pubmed-article:11272144 | pubmed:chemical | http://linkedlifedata.com/r... | lld:pubmed |
pubmed-article:11272144 | pubmed:status | MEDLINE | lld:pubmed |
pubmed-article:11272144 | pubmed:month | Feb | lld:pubmed |
pubmed-article:11272144 | pubmed:issn | 0012-1797 | lld:pubmed |
pubmed-article:11272144 | pubmed:author | pubmed-author:Aynsley-Green... | lld:pubmed |
pubmed-article:11272144 | pubmed:author | pubmed-author:SharpG WGW | lld:pubmed |
pubmed-article:11272144 | pubmed:author | pubmed-author:ChapmanJ CJC | lld:pubmed |
pubmed-article:11272144 | pubmed:author | pubmed-author:DunneM JMJ | lld:pubmed |
pubmed-article:11272144 | pubmed:author | pubmed-author:GlaserBB | lld:pubmed |
pubmed-article:11272144 | pubmed:author | pubmed-author:BarnesP DPD | lld:pubmed |
pubmed-article:11272144 | pubmed:author | pubmed-author:ShepherdR MRM | lld:pubmed |
pubmed-article:11272144 | pubmed:author | pubmed-author:AmmäläCC | lld:pubmed |
pubmed-article:11272144 | pubmed:author | pubmed-author:StraubS GSG | lld:pubmed |
pubmed-article:11272144 | pubmed:author | pubmed-author:LindleyK JKJ | lld:pubmed |
pubmed-article:11272144 | pubmed:author | pubmed-author:O'BrienR ERE | lld:pubmed |
pubmed-article:11272144 | pubmed:author | pubmed-author:CosgroveK EKE | lld:pubmed |
pubmed-article:11272144 | pubmed:author | pubmed-author:KuchinskiNN | lld:pubmed |
pubmed-article:11272144 | pubmed:author | pubmed-author:SchaeppiMM | lld:pubmed |
pubmed-article:11272144 | pubmed:issnType | Print | lld:pubmed |
pubmed-article:11272144 | pubmed:volume | 50 | lld:pubmed |
pubmed-article:11272144 | pubmed:owner | NLM | lld:pubmed |
pubmed-article:11272144 | pubmed:authorsComplete | Y | lld:pubmed |
pubmed-article:11272144 | pubmed:pagination | 329-39 | lld:pubmed |
pubmed-article:11272144 | pubmed:dateRevised | 2011-11-17 | lld:pubmed |
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pubmed-article:11272144 | pubmed:year | 2001 | lld:pubmed |
pubmed-article:11272144 | pubmed:articleTitle | Hyperinsulinism of infancy: the regulated release of insulin by KATP channel-independent pathways. | lld:pubmed |
pubmed-article:11272144 | pubmed:affiliation | Department of Molecular Medicine, College of Veterinary Medicine, Cornell University, Ithaca, New York, USA. | lld:pubmed |
pubmed-article:11272144 | pubmed:publicationType | Journal Article | lld:pubmed |
pubmed-article:11272144 | pubmed:publicationType | In Vitro | lld:pubmed |
pubmed-article:11272144 | pubmed:publicationType | Research Support, U.S. Gov't, P.H.S. | lld:pubmed |
pubmed-article:11272144 | pubmed:publicationType | Research Support, Non-U.S. Gov't | lld:pubmed |
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