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Select item 99055901.

Lymphedema as first clinical presentation of Cantu Syndrome: reversed phenotyping after identification of gain-of-function variant in ABCC9

Jian Gao, Conor McClenaghan, Imke Christiaans, Marielle Alders, Kirsten van Duinen, Mieke M. van Haelst, Gijs van Haaften, Colin G. Nichols

Eur J Hum Genet. 2023 Feb; 31(2): 188–194. Published online 2022 Nov 7. doi: 10.1038/s41431-022-01210-x

PMCID:: PMC9905590

Abstract Article PDF–2.4M

Select item 58908822.

The ATPase activities of SUR2A and SUR2B are influenced by the C-terminal 42 amino acids

Heidi de Wet, Constantina Fotinou, Nawaz Amad, Matthias Dreger, Frances M. Ashcroft

FEBS J. Author manuscript; available in PMC 2018 Apr 9.

Published in final edited form as: FEBS J. 2010 Jun; 277(12): 2654–2662. doi: 10.1111/j.1742-464X.2010.07675.x

PMCID:: PMC5890882

Abstract Article PDF–1.1M

Select item 58087653.

Cantu syndrome–associated SUR2 (ABCC9) mutations in distinct structural domains result in K_ATP channel gain-of-function by differential mechanisms

Conor McClenaghan, Alex Hanson, Monica Sala-Rabanal, Helen I. Roessler, Dragana Josifova, Dorothy K. Grange, Gijs van Haaften, Colin G. Nichols

J Biol Chem. 2018 Feb 9; 293(6): 2041–2052. Published online 2017 Dec 22. doi: 10.1074/jbc.RA117.000351

PMCID:: PMC5808765

Abstract Article PDF–2.8M

Select item 45662424.

Phosphorylation-dependent Changes in Nucleotide Binding, Conformation, and Dynamics of the First Nucleotide Binding Domain (NBD1) of the Sulfonylurea Receptor 2B (SUR2B)

Elvin D. de Araujo, Claudia P. Alvarez, Jorge P. López-Alonso, Clarissa R. Sooklal, Marijana Stagljar, Voula Kanelis

J Biol Chem. 2015 Sep 11; 290(37): 22699–22714. Published online 2015 Jul 21. doi: 10.1074/jbc.M114.636233

PMCID:: PMC4566242

Abstract Article PDF–9.8M

Select item 35994795.

Tetrameric structure of SUR2B revealed by electron microscopy of oriented single particles

Constantina Fotinou, Jussi Aittoniemi, Heidi Wet, Ange Polidori, Bernard Pucci, Mark S P Sansom, Catherine Vénien-Bryan, Frances M Ashcroft

FEBS J. 2013 Feb; 280(4): 1051–1063. Published online 2013 Jan 27. doi: 10.1111/febs.12097

PMCID:: PMC3599479

Abstract Article PDF–882K

Select item 35732296.

Testosterone protects female embryonic heart H9c2 cells against severe metabolic stress by activating estrogen receptors and up-regulating IES SUR2B

Thomas Ballantyne, Qingyou Du, Sofija Jovanović, Andrew Neemo, Robert Holmes, Sharabh Sinha, Aleksandar Jovanović

Int J Biochem Cell Biol. 2013 Feb; 45(2): 283–291. doi: 10.1016/j.biocel.2012.10.005

PMCID:: PMC3573229

Abstract Article

Select item 22763267.

cAMP-dependent Protein Kinase Phosphorylation Produces Interdomain Movement in SUR2B Leading to Activation of the Vascular K_ATP Channel

Yun Shi, Xianfeng Chen, Zhongying Wu, Weiwei Shi, Yang Yang, Ningren Cui, Chun Jiang, Robert W. Harrison

J Biol Chem. 2008 Mar 21; 283(12): 7523–7530. doi: 10.1074/jbc.M709941200

PMCID:: PMC2276326

Abstract Article PDF–614K

Select item 22582218.

PKA phosphorylation of SUR2B subunit underscores vascular K_ATP channel activation by beta-adrenergic receptors

Yun Shi, Zhongying Wu, Ningren Cui, Weiwei Shi, Yang Yang, Xiaoli Zhang, Asheebo Rojas, Binh T. Ha, Chun Jiang

Am J Physiol Regul Integr Comp Physiol. Author manuscript; available in PMC 2008 Sep 1.

Published in final edited form as: Am J Physiol Regul Integr Comp Physiol. 2007 Sep; 293(3): R1205–R1214. Published online 2007 Jun 27. doi: 10.1152/ajpregu.00337.2007.

PMCID:: PMC2258221

Abstract Article PDF–726K

Select item 49796259.

Altered K_ATP Channel Subunits Expression and Vascular Reactivity in Spontaneously Hypertensive Rats With Age

Xiaojing Liu, Peng Duan, Xingxing Hu, Ruisheng Li, Qinglei Zhu

J Cardiovasc Pharmacol. 2016 Aug; 68(2): 143–149. Published online 2016 Apr 1. doi: 10.1097/FJC.0000000000000394

PMCID:: PMC4979625

Abstract Article PDF–388K

Select item 466482710.

Differential mechanisms of Cantú syndrome–associated gain of function mutations in the ABCC9 (SUR2) subunit of the K_ATP channel

Paige E. Cooper, Monica Sala-Rabanal, Sun Joo Lee, Colin G. Nichols

J Gen Physiol. 2015 Dec; 146(6): 527–540. doi: 10.1085/jgp.201511495

PMCID:: PMC4664827

Abstract Article PDF–3.0M

Select item 394786911.

ABCC9 is a Novel Brugada and Early Repolarization Syndrome Susceptibility Gene

Dan Hu, Hector Barajas-Martínez, Andre Terzic, Sungjo Park, Ryan Pfeiffer, Elena Burashnikov, Yuesheng Wu, Martin Borggrefe, Christian Veltmann, Rainer Schimpf, John J. Cai, Gi-Byong Nam, Pramod Deshmukh, Melvin Scheinman, Mark Preminger, Jonathan Steinberg, Angélica López-Izquierdo, Daniela Ponce-Balbuena, Christian Wolpert, Michel Haïssaguerre, José Antonio Sánchez-Chapula, Charles Antzelevitch

Int J Cardiol. Author manuscript; available in PMC 2015 Feb 15.

Published in final edited form as: Int J Cardiol. 2014 Feb 15; 171(3): 431–442. Published online 2014 Jan 4. doi: 10.1016/j.ijcard.2013.12.084

PMCID:: PMC3947869

Abstract Article PDF–4.7M

Select item 327442412.

Increase of ATP-sensitive potassium (K_ATP) channels in the heart of type-1 diabetic rats

Zhih-Cherng Chen, Yung-Ze Cheng, Li-Jen Chen, Kai-Chun Cheng, Yin- Xiao Li, Juei- Tang Cheng

Cardiovasc Diabetol. 2012; 11: 8. Published online 2012 Jan 18. doi: 10.1186/1475-2840-11-8

PMCID:: PMC3274424

Abstract Article PDF–1.2M

Select item 274228713.

Exchange protein activated by cAMP (Epac) mediates cAMP-dependent but protein kinase A-insensitive modulation of vascular ATP-sensitive potassium channels

Gregor I Purves, Tomoko Kamishima, Lowri M Davies, John M Quayle, Caroline Dart

J Physiol. 2009 Jul 15; 587(Pt 14): 3639–3650. Published online 2009 Jun 2. doi: 10.1113/jphysiol.2009.173534

PMCID:: PMC2742287

Abstract Article PDF–592K

Select item 227700214.

A mutation in the ATP-binding site of the Kir6.2 subunit of the K_ATP channel alters coupling with the SUR2A subunit

Paolo Tammaro, Frances M Ashcroft

J Physiol. 2007 Nov 1; 584(Pt 3): 743–753. Published online 2007 Sep 13. doi: 10.1113/jphysiol.2007.143149

PMCID:: PMC2277002

Abstract Article PDF–519K

Select item 207528615.

Remodelling of the SUR–Kir6.2 interface of the K_ATP channel upon ATP binding revealed by the conformational blocker rhodamine 123

Eric Hosy, Renaud Dérand, Jean Revilloud, Michel Vivaudou

J Physiol. 2007 Jul 1; 582(Pt 1): 27–39. Published online 2007 May 17. doi: 10.1113/jphysiol.2007.134288

PMCID:: PMC2075286

Abstract Article PDF–705K

Select item 199543816.

ABCC9 mutations identified in human dilated cardiomyopathy disrupt catalytic K_ATP channel gating

Martin Bienengraeber, Timothy M Olson, Vitaliy A Selivanov, Eva C Kathmann, Fearghas O’Cochlain, Fan Gao, Amy B Karger, Jeffrey D Ballew, Denice M Hodgson, Leonid V Zingman, Yuan-Ping Pang, Alexey E Alekseev, Andre Terzic

Nat Genet. Author manuscript; available in PMC 2007 Oct 1.

Published in final edited form as: Nat Genet. 2004 Apr; 36(4): 382–387. Published online 2004 Mar 21. doi: 10.1038/ng1329

PMCID:: PMC1995438

Abstract Article PDF–2.3M

Select item 122404117.

Proximal C-terminal domain of sulphonylurea receptor 2A interacts with pore-forming Kir6 subunits in KATP channels.

Richard D Rainbow, Marian James, Diane Hudman, Mohammed Al Johi, Harprit Singh, Peter J Watson, Ian Ashmole, Noel W Davies, David Lodwick, Robert I Norman

Biochem J. 2004 Apr 1; 379(Pt 1): 173–181. doi: 10.1042/BJ20031087

PMCID:: PMC1224041

Summary PDF–253K

Select item 295734618.

Cardiomyocyte sulfonylurea receptor 2-K_ATP channel mediates cardioprotection and ST segment elevation

Douglas A. Stoller, John P. Fahrenbach, Karel Chalupsky, Bi-Hua Tan, Nitin Aggarwal, Jamie Metcalfe, Michele Hadhazy, Nian-Qing Shi, Jonathan C. Makielski, Elizabeth M. McNally

Am J Physiol Heart Circ Physiol. 2010 Oct; 299(4): H1100–H1108. Published online 2010 Jul 23. doi: 10.1152/ajpheart.00084.2010

PMCID:: PMC2957346

Abstract Article

Select item 288153419.

Infection with AV-SUR2A protects H9C2 cells against metabolic stress: A mechanism of SUR2A-mediated cytoprotection independent from the K_ATP channel activity

Qingyou Du, Sofija Jovanović, Andriy Sukhodub, Aleksandar Jovanović

Biochim Biophys Acta. 2010 Mar; 1803(3): 405–415. doi: 10.1016/j.bbamcr.2010.01.018

PMCID:: PMC2881534

Abstract Article

Select item 275806720.

M-LDH physically associated with sarcolemmal K_ATP channels mediates cytoprotection in heart embryonic H9C2 cells

Sofija Jovanović, Qingyou Du, Andriy Sukhodub, Aleksandar Jovanović

Int J Biochem Cell Biol. 2009 Nov; 41(11): 2295–2301. doi: 10.1016/j.biocel.2009.05.012

PMCID:: PMC2758067

Abstract Article

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