{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,12]],"date-time":"2026-03-12T09:14:30Z","timestamp":1773306870141,"version":"3.50.1"},"reference-count":50,"publisher":"American Diabetes Association","issue":"2","content-domain":{"domain":["diabetesjournals.org"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2007,2,1]]},"abstract":"<jats:p>Mutations in the pancreatic ATP-sensitive K+ channel (KATP channel) cause permanent neonatal diabetes mellitus (PNDM) in humans. All of the KATP channel mutations examined result in decreased ATP inhibition, which in turn is predicted to suppress insulin secretion. Here we describe a patient with severe PNDM, which includes developmental delay and epilepsy, in addition to neonatal diabetes (developmental delay, epilepsy, and neonatal diabetes [DEND]), due to a G334D mutation in the Kir6.2 subunit of KATP channel. The patient was wholly unresponsive to sulfonylurea therapy (up to 1.14 mg \u00b7 kg\u22121 \u00b7 day\u22121) and remained insulin dependent. Consistent with the putative role of G334 as an ATP-binding residue, reconstituted homomeric and mixed WT+G334D channels exhibit absent or reduced ATP sensitivity but normal gating behavior in the absence of ATP. In disagreement with the sulfonylurea insensitivity of the affected patient, the G334D mutation has no effect on the sulfonylurea inhibition of reconstituted channels in excised patches. However, in macroscopic rubidium-efflux assays in intact cells, reconstituted mutant channels do exhibit a decreased, but still present, sulfonylurea response. The results demonstrate that ATP-binding site mutations can indeed cause DEND and suggest the possibility that sulfonylurea insensitivity of such patients may be a secondary reflection of the presence of DEND rather than a simple reflection of the underlying molecular basis.<\/jats:p>","DOI":"10.2337\/db06-1275","type":"journal-article","created":{"date-parts":[[2007,1,26]],"date-time":"2007-01-26T20:11:02Z","timestamp":1169842262000},"page":"328-336","update-policy":"https:\/\/doi.org\/10.2337\/ada-journal-policies","source":"Crossref","is-referenced-by-count":76,"title":["An ATP-Binding Mutation (G334D) in <i>KCNJ11<\/i> Is Associated With a Sulfonylurea-Insensitive Form of Developmental Delay, Epilepsy, and Neonatal Diabetes"],"prefix":"10.2337","volume":"56","author":[{"given":"Ricard","family":"Masia","sequence":"first","affiliation":[{"name":"Department of Cell Biology and Physiology, Washington University School of Medicine, St. Louis, Missouri"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Joseph C.","family":"Koster","sequence":"additional","affiliation":[{"name":"Department of Cell Biology and Physiology, Washington University School of Medicine, St. Louis, Missouri"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Stefano","family":"Tumini","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, University of Chieti, Chieti, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Francesco","family":"Chiarelli","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, University of Chieti, Chieti, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Carlo","family":"Colombo","sequence":"additional","affiliation":[{"name":"Bambino Gesu\u0300 Children\u2019s Hospital IRCCS, Piazza S Onofrio 4, 00164 Rome, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Colin G.","family":"Nichols","sequence":"additional","affiliation":[{"name":"Department of Cell Biology and Physiology, Washington University School of Medicine, St. Louis, Missouri"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Fabrizio","family":"Barbetti","sequence":"additional","affiliation":[{"name":"Bambino Gesu\u0300 Children\u2019s Hospital IRCCS, Piazza S Onofrio 4, 00164 Rome, Italy"},{"name":"S. Raffaele Scientific Park Foundation, Rome, Italy"},{"name":"Department of Internal Medicine, University of Tor Vergata, Rome, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1167","reference":[{"key":"2022031208564581100_R1","doi-asserted-by":"crossref","unstructured":"Gloyn AL, Pearson ER, Antcliff JF, Proks P, Bruining GJ, Slingerland AS, Howard N, Srinivasan S, Silva JM, Molnes J, Edghill EL, Frayling TM, Temple IK, Mackay D, Shield JP, Sumnik Z, van Rhijn A, Wales JK, Clark P, Gorman S, Aisenberg J, Ellard S, Njolstad PR, Ashcroft FM, Hattersley AT: Activating mutations in the gene encoding the ATP-sensitive potassium-channel subunit Kir6.2 and permanent neonatal diabetes. 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