PMID-sentid Pub_year Sent_text comp_official_name comp_offsetprotein_name organism prot_offset 26392140-4 2015 Diazoxide blocks insulin release from the pancreas by opening the SUR1/Kir6.2 channels in ss-cells. Diazoxide 0-9 potassium inwardly rectifying channel subfamily J member 11 Homo sapiens 71-77 26392140-5 2015 Diazoxide can also act on two potassium-gated channels in the skin that affect hair growth, namely SUR1/Kir6.2 and SUR2B/Kir6.1, thus causing hypertrichosis. Diazoxide 0-9 potassium inwardly rectifying channel subfamily J member 11 Homo sapiens 104-110 23345197-9 2013 Among diazoxide-unresponsive patients (n=105), mutations in ABCC8/KCNJ11 were identified in 92 (87.6%) patients, of whom 63 patients had recessively inherited mutations while four patients had dominantly inherited mutations. Diazoxide 6-15 potassium inwardly rectifying channel subfamily J member 11 Homo sapiens 66-72 26316440-3 2015 Recessive ABCC8 and KCNJ11 mutations are responsible for most (82%) of the severe diazoxide-unresponsive CHI. Diazoxide 82-91 potassium inwardly rectifying channel subfamily J member 11 Homo sapiens 20-26 24686051-7 2014 Among diazoxide-unresponsive patients, mutations in ABCC8/KCNJ11 were identified in 16 (94%) patients. Diazoxide 6-15 potassium inwardly rectifying channel subfamily J member 11 Homo sapiens 58-64 23345197-10 2013 A paternal mutation in the ABCC8/KCNJ11 genes was identified in 23 diazoxide-unresponsive patients, of whom six had diffuse disease. Diazoxide 67-76 potassium inwardly rectifying channel subfamily J member 11 Homo sapiens 33-39 16837559-7 2006 Furthermore, in human embryonic kidney 293 cells, iptakalim (300-500 microM) closed diazoxide-induced Kir6.2/SUR1 K(ATP) channels, which were heterologously expressed. Diazoxide 84-93 potassium inwardly rectifying channel subfamily J member 11 Homo sapiens 102-108 20573158-3 2011 Dominant inactivating ABCC8 and KCNJ11 mutations are less frequent, but are usually associated with a milder form of hypoglycaemia that is responsive to diazoxide therapy. Diazoxide 153-162 potassium inwardly rectifying channel subfamily J member 11 Homo sapiens 32-38 20589481-3 2010 The authors report a case of familial hyperinsulinemic hypoglycemia with homozygous T294M mutation of the KCNJ11 gene, which responded to diazoxide therapy. Diazoxide 138-147 potassium inwardly rectifying channel subfamily J member 11 Homo sapiens 106-112 21967988-8 2011 Recessive ABCC8 mutations (encoding SUR1, subunit of a potassium channel) and, more rarely, recessive KCNJ11 (encoding Kir6.2, subunit of the same potassium channel) mutations, are responsible for most severe diazoxide-unresponsive HI. Diazoxide 209-218 potassium inwardly rectifying channel subfamily J member 11 Homo sapiens 102-108 21967988-8 2011 Recessive ABCC8 mutations (encoding SUR1, subunit of a potassium channel) and, more rarely, recessive KCNJ11 (encoding Kir6.2, subunit of the same potassium channel) mutations, are responsible for most severe diazoxide-unresponsive HI. Diazoxide 209-218 potassium inwardly rectifying channel subfamily J member 11 Homo sapiens 119-125 21145327-5 2011 However, in cells transfected with Kir6.2 siRNAs, diazoxide-stimulated activity of K(ATP) channels was abolished. Diazoxide 50-59 potassium inwardly rectifying channel subfamily J member 11 Homo sapiens 35-41 21145327-9 2011 Specifically, to mimic the action of diazoxide, the baseline value of open time (tau(bas)) of K(ATP) channels was arbitrarily elevated, while varying number of active channels (N(O)) was set to simulate electrical behavior of Kir6.2 siRNAs-transfected cells. Diazoxide 37-46 potassium inwardly rectifying channel subfamily J member 11 Homo sapiens 226-232 21185999-7 2011 ABCC8/KCNJ11 defects are identified in approximately 80% of patients with CHI refractory to diazoxide. Diazoxide 92-101 potassium inwardly rectifying channel subfamily J member 11 Homo sapiens 6-12 20685672-0 2010 ABCC8 and KCNJ11 molecular spectrum of 109 patients with diazoxide-unresponsive congenital hyperinsulinism. Diazoxide 57-66 potassium inwardly rectifying channel subfamily J member 11 Homo sapiens 10-16 20685672-5 2010 METHODS: ABCC8/KCNJ11 mutational spectrum was established in 109 diazoxide-unresponsive CHI patients for whom an appropriate clinical management is essential to prevent brain damage. Diazoxide 65-74 potassium inwardly rectifying channel subfamily J member 11 Homo sapiens 15-21 17301957-4 2007 Here, we demonstrated for the first time that Kir 6.1, Kir 6.2 and SUR2 subunits of K(ATP) channels are functionally expressed in HaCaT cells and both non-selective K(ATP) channel opener pinacidil and mitoK(ATP) (mitochondrial K(ATP)) channel opener diazoxide attenuated UV-induced keratinocytes cell death. Diazoxide 250-259 potassium inwardly rectifying channel subfamily J member 11 Homo sapiens 55-62 12639916-7 2003 The ATP-sensitive potassium channel opener diazoxide also induced an outward K(+) current (maximum of 18.7 +/- 2.2 pA) in the majority (92%) of POMC neurons with an EC(50) of 61 micro M. The response to diazoxide was blocked by the sulfonylurea tolbutamide, indicating that the POMC neurons express both Kir6.2 and sulfonylurea receptor 1 channel subunits, which was verified using single cell RT-PCR. Diazoxide 43-52 potassium inwardly rectifying channel subfamily J member 11 Homo sapiens 304-310 12961066-4 2003 RESULTS: In HEK 293 cells, NNC 55-0118 and NN414 activated Kir6.2/SUR1 currents with EC(50) values of 0.33 micromol/l and 0.45 micromol/l, respectively, compared with that of 31 micro mol/l for diazoxide. Diazoxide 194-203 potassium inwardly rectifying channel subfamily J member 11 Homo sapiens 59-65 16475928-5 2006 Openers of Kir6.2/SUR1 channels, e.g. diazoxide, have in contrast only found limited clinical use in treatment of hypersecretion of insulin associated with certain tumours (insulinoma) and genetic disorders (persistent hyperinsulinemia and hypoglycemia of infancy, PHHI). Diazoxide 38-47 potassium inwardly rectifying channel subfamily J member 11 Homo sapiens 11-17 12639916-7 2003 The ATP-sensitive potassium channel opener diazoxide also induced an outward K(+) current (maximum of 18.7 +/- 2.2 pA) in the majority (92%) of POMC neurons with an EC(50) of 61 micro M. The response to diazoxide was blocked by the sulfonylurea tolbutamide, indicating that the POMC neurons express both Kir6.2 and sulfonylurea receptor 1 channel subunits, which was verified using single cell RT-PCR. Diazoxide 203-212 potassium inwardly rectifying channel subfamily J member 11 Homo sapiens 304-310 12031979-0 2002 The novel diazoxide analog 3-isopropylamino-7-methoxy-4H-1,2,4-benzothiadiazine 1,1-dioxide is a selective Kir6.2/SUR1 channel opener. Diazoxide 10-19 potassium inwardly rectifying channel subfamily J member 11 Homo sapiens 107-113 10518593-5 1999 Here, in excised patches, diazoxide (300 microM) activated pancreatic SUR1/Kir6.2 currents and had little effect on native or recombinant cardiac SUR2A/Kir6.2 currents. Diazoxide 26-35 potassium inwardly rectifying channel subfamily J member 11 Homo sapiens 75-81 11808879-16 2002 Almost all patients with SUR1 (38/41) or KIR6.2 (5/7) mutations were resistant to diazoxide. Diazoxide 82-91 potassium inwardly rectifying channel subfamily J member 11 Homo sapiens 41-47 10518593-6 1999 However, in the presence of cytoplasmic ADP (100 microM), SUR2A/Kir6.2 channels became as sensitive to diazoxide as SUR1/Kir6. Diazoxide 103-112 potassium inwardly rectifying channel subfamily J member 11 Homo sapiens 64-70 34633981-0 2021 Birth weight and diazoxide unresponsiveness strongly predict the likelihood of congenital hyperinsulinism due to a mutation in ABCC8 or KCNJ11. Diazoxide 17-26 potassium inwardly rectifying channel subfamily J member 11 Homo sapiens 136-142 33770274-14 2021 Consanguinity is a very important factor regarding the genetics and phenotype of CHI, increasing the risk of autosomal recessive genetic disorders, including the severe, diazoxide-unresponsive forms caused by recessive inactivating mutations in ABCC8 and KCNJ11. Diazoxide 170-179 potassium inwardly rectifying channel subfamily J member 11 Homo sapiens 255-261 33502730-16 2021 Spontaneous remission was also possible for a small number of children that carried mutations in the ABCC and KCNJ11 genes and in whom diazoxide treatment failed. Diazoxide 135-144 potassium inwardly rectifying channel subfamily J member 11 Homo sapiens 110-116 20301549-6 1993 Individuals with autosomal recessive familial hyperinsulinism, caused by pathogenic variants in either ABCC8 or KCNJ11 (FHI-KATP), tend to be large for gestational age and usually present with severe refractory hypoglycemia in the first 48 hours of life; affected infants usually respond only partially to diet or medical management (i.e., diazoxide therapy) and thus may require pancreatic resection. Diazoxide 340-349 potassium inwardly rectifying channel subfamily J member 11 Homo sapiens 112-118 31464105-4 2019 One of the three ABCC8 mutations (p.Ala1458Thr) and all three KCNJ11 mutations were associated with responsiveness to diazoxide. Diazoxide 118-127 potassium inwardly rectifying channel subfamily J member 11 Homo sapiens 62-68 27646472-10 2016 Overexpression of Kir6.2/SUR1 resulted in an increase in iron influx and intracellular iron levels, which was markedly increased after diazoxide treatment. Diazoxide 135-144 potassium inwardly rectifying channel subfamily J member 11 Homo sapiens 18-24 30114684-7 2019 Case 2: A 1-month-old boy with diazoxide-unresponsive CHI due to a paternal heterozygous KCNJ11 gene mutation was partially responsive to octreotide. Diazoxide 31-40 potassium inwardly rectifying channel subfamily J member 11 Homo sapiens 89-95 27181099-8 2016 A novel homozygous mutation (p.F315I) in the KCNJ11 gene, leading to diazoxide-unresponsive CHI, was identified. Diazoxide 69-78 potassium inwardly rectifying channel subfamily J member 11 Homo sapiens 45-51 27181099-14 2016 We present a novel homozygous p.F315I mutation in the KCNJ11 gene leading to diazoxide-unresponsive CHI in a neonate. Diazoxide 77-86 potassium inwardly rectifying channel subfamily J member 11 Homo sapiens 54-60 28270372-12 2017 Among the diazoxide-unresponsive cases, gene mutations were detected in 20/36 (55.6%) cases with ABCC8 and in 2 (5.6%) cases with KCNJ11. Diazoxide 10-19 potassium inwardly rectifying channel subfamily J member 11 Homo sapiens 130-136 28270372-13 2017 Among the diazoxide-responsive cases, gene mutations were detected in 8 patients with ABCC8, 4 with KCNJ11, 5 with GLUD1, and 1 with GCK. Diazoxide 10-19 potassium inwardly rectifying channel subfamily J member 11 Homo sapiens 100-106 28270372-16 2017 More than half of the diazoxide-unresponsive CHI detected mutations are in ABCC8 and KCNJ11 genes. Diazoxide 22-31 potassium inwardly rectifying channel subfamily J member 11 Homo sapiens 85-91 27693800-4 2016 ZD0947 reversibly suppressed diazoxide-elicited SUR1/Kir6.2 channels activity and pinacidil-elicited SUR2A/Kir6.2 channel activity. Diazoxide 29-38 potassium inwardly rectifying channel subfamily J member 11 Homo sapiens 53-59