Article
Biochemistry & Molecular Biology
Min Woo Sung, Camden M. Driggers, Barmak Mostofian, John D. Russo, Bruce L. Patton, Daniel M. Zuckerman, Show-Ling Shyng
Summary: Regulation of pancreatic KATP channels involves complex interactions between their subunits and ligands. This study investigates the structural changes and ligand interactions associated with channel gating using cryo-EM data. The results show that pharmacological inhibitors and ATP can modulate the channel conformation and affect intra-subunit and inter-subunit interactions.
JOURNAL OF MOLECULAR BIOLOGY
(2022)
Article
Endocrinology & Metabolism
Gregor Sachse, Elizabeth Haythorne, Thomas Hill, Peter Proks, Russell Joynson, Raul Terron-Exposito, Liz Bentley, Stephen J. Tucker, Roger D. Cox, Frances M. Ashcroft
Summary: The K23 variant in the K-ATP channel impairs insulin secretion and increases diabetes risk, especially in combination with a high-fat diet and obesity. This variant causes dysfunction in beta-cell insulin secretion, particularly at threshold glucose levels.
Article
Endocrinology & Metabolism
Yaning Chen, Xiaodong Hu, Jia Cui, Mingwei Zhao, Hebin Yao
Summary: A young female patient diagnosed with KCNJ11-MODY diabetes at the age of 28 was found to carry the R136C mutation through whole exome sequencing, while her daughter does not have this mutation. Bioinformatics software predicted the mutation to be deleterious, affecting the channel port structure of the K-ATP channel.
DIABETOLOGY & METABOLIC SYNDROME
(2021)
Article
Endocrinology & Metabolism
Cheng-Ting Lee, Wen-Hao Tsai, Chien-Ching Chang, Pei-Chun Chen, Cathy Shen-Jang Fann, Hsueh-Kai Chang, Shih-Yao Liu, Mu-Zon Wu, Pao-Chin Chiu, Wen-Ming Hsu, Wei-Shiung Yang, Ling-Ping Lai, Wen-Yu Tsai, Shi-Bing Yang, Pei-Lung Chen
Summary: This study identified the genetic etiologies of Taiwanese children with the most severe diazoxide-unresponsive CHI and found that pathogenic variants in K-ATP channels are the most common cause. The p.T1042QfsX75 variant in the ABCC8 gene may have a founder effect. In addition, a mutation in the GCK gene and three rare variants in the SUR1 channel were also associated with the diazoxide-unresponsive phenotype.
FRONTIERS IN ENDOCRINOLOGY
(2023)
Review
Biochemistry & Molecular Biology
Yevheniia Kravenska, Vanessa Checchetto, Ildiko Szabo
Summary: Potassium ions can enter and exit mitochondria through various channels in the outer and inner membranes, with the ATP-dependent potassium channel being the most studied and playing a crucial role in protecting cells against ischemic and neuronal damage.
Article
Multidisciplinary Sciences
Chen Zhao, Roderick MacKinnon
Summary: K-ATP channels act as metabolic sensors translating ATP/ADP balance to membrane excitability. The structure of human pancreatic K-ATP reveals an open pore, coordinated structural changes within the ATP-binding site and channel gate in Kir, as well as conformational changes in SUR. The pancreatic hK(ATP) exhibits a unique PIP2-independent opening property.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
(2021)
Review
Neurosciences
Sha Zhao, MengZhen Wang, ZeGang Ma
Summary: This review highlights the crucial roles of K-ATP channels in the pathogenesis of PD, and provides possible research directions from different perspectives such as vulnerability of dopamine neurons, neurotransmitter releasing, iron metabolism, α-synuclein secretion, and mitochondrial dysfunction, which are critical for investigation of K-ATP channels-targeted precise therapeutic interventions for PD.
BRAIN RESEARCH BULLETIN
(2021)
Article
Endocrinology & Metabolism
Julie Siersbaek, Annette Ronholt Larsen, Mads Nybo, Henrik Thybo Christesen
Summary: The sensitive insulin assay showed excellent performance in diagnosing CHI, with optimal p-insulin cut-offs at 12.3 pmol/L (2.0 mU/L) and 10.6 pmol/L (1.8 mU/L) at p-glucose <3.2 mmol/L and <3.0 mmol/L, respectively. This sensitive insulin assay may help simplify the diagnosis of CHI.
FRONTIERS IN ENDOCRINOLOGY
(2021)
Article
Biochemistry & Molecular Biology
Plinio Bezerra Palacio, Geovanna Carvalho de Freitas Soares, Gabriella Moreira Bezerra Lima, Pedro Lourenzo Oliveira Cunha, Anna Lidia Nunes Varela, Heberty Tarso Facundo
Summary: This study investigates the molecular and physiological mechanisms of activators (GTP) and inhibitors (ATP) in the activity of mitoKATP channels. The findings demonstrate that ATP inhibits mitoKATP activity, while GTP reverses this inhibition in a dose-dependent manner. Both nucleotides bind to mitoSUR and competitively affect its function. These results are important for further understanding the role of GTP and ATP in cardioprotection.
CHEMICO-BIOLOGICAL INTERACTIONS
(2023)
Article
Multidisciplinary Sciences
Dian Ding, Jing-Xiang Wu, Xinli Duan, Songling Ma, Lipeng Lai, Lei Chen
Summary: ATP-sensitive potassium channels (K-ATP) composed of Kir6 and SUR subunits convert the metabolic status of the cell into electrical signals. The authors present cryo-EM structures of SUR2A and SUR2B subunits in complex with two vasodilators, P1075 and levcromakalim, uncovering the mechanisms of these drugs.
NATURE COMMUNICATIONS
(2022)
Review
Cell Biology
Jirong Lv, Xue Xiao, Mingxia Bi, Tingting Tang, Deao Kong, Meining Diao, Qian Jiao, Xi Chen, Chunling Yan, Xixun Du, Hong Jiang
Summary: ATP-sensitive potassium channels (KATP channels) play a crucial role in linking the electrical activity of the cell membrane with cell metabolism. They have been found to be expressed in various cells and play important roles in neurodegenerative diseases. The KATP channel openers and inhibitors have protective effects on neurons by regulating neuronal excitability and neurotransmitter release, reducing abnormal protein aggregation and Ca2+ overload, and decreasing reactive oxygen species (ROS) production and microglia activation. However, KATP channels also exhibit dual effects in some cases.
AGEING RESEARCH REVIEWS
(2022)
Article
Pharmacology & Pharmacy
Plinio Bezerra Palacio, Aline Maria Brito Lucas, Joana Varlla de Lacerda Alexandre, Pedro Lourenzo Oliveira Cunha, Yuana Ivia Ponte Viana, Amanda Cabral Albuquerque, Anna Lidia Nunes Varela, Heberty Tarso Facundo
Summary: The study reveals competitive binding of glibenclamide and diazoxide at mitoSUR, with the cyclohexylurea portion of glibenclamide being essential for mitoKATP inhibition. IMP-A does not inhibit diazoxide-induced cardioprotection, but glibenclamide effectively inhibits it. This research provides valuable insights into the pharmacological and biochemical aspects of mitoKATP activation and cardioprotection, potentially leading to new therapeutic strategies for ischemia-reperfusion injury.
EUROPEAN JOURNAL OF PHARMACOLOGY
(2021)
Article
Multidisciplinary Sciences
DaNae R. Woodard, Emi Nakahara, John D. Hulleman
Summary: Among various F3 mutations, only one variant exhibited a significant secretion defect, emphasizing the importance of this particular mutation in F3 protein secretion.
SCIENTIFIC REPORTS
(2021)
Review
Neurosciences
Amalie Clement, Sarah Louise Christensen, Inger Jansen-Olesen, Jes Olesen, Song Guo
Summary: Migraine is a leading cause of disability worldwide and has a significant impact on work and social life. Blocking the ATP-sensitive potassium channel may be a promising approach for migraine treatment. However, further research is needed to understand the potential side effects and effectiveness of this approach.
FRONTIERS IN MOLECULAR NEUROSCIENCE
(2023)
Article
Pharmacology & Pharmacy
Shiru Bai, Xuechao Wang, Haibo Wu, Tianlei Chen, Xinning Li, Lina Zhang, Xiangming Li, Lu Er, Rongpin Du
Summary: The study demonstrated that anisodamine could protect against myocardial ischemia/reperfusion injury by improving mitochondrial energy metabolism, reducing oxidative stress, and stabilizing mitochondrial membrane potential. The opening of mitoK ATP may play a crucial role in the cardioprotective effects of anisodamine.
EUROPEAN JOURNAL OF PHARMACOLOGY
(2021)
Editorial Material
Pediatrics
Charles A. Stanley, Paul J. Rozance, Paul S. Thornton, Diva D. De Leon, Deborah Harris, Morey W. Haymond, Khalid Hussain, Lynne L. Levitsky, Mohammad H. Murad, Rebecca A. Simmons, Mark A. Sperling, David A. Weinstein, Neil H. White, Joseph I. Wolfsdorf
JOURNAL OF PEDIATRICS
(2015)
Article
Cell Biology
Sharona Tornovsky-Babeay, Daniela Dadon, Oren Ziv, Elhanan Tzipilevich, Tehila Kadosh, Rachel Schyr-Ben Haroush, Ayat Hija, Miri Stolovich-Rain, Judith Furth-Lavi, Zvi Granot, Shay Porat, Louis H. Philipson, Kevan C. Herold, Tricia R. Bhatti, Charles Stanley, Frances M. Ashcroft, Peter In't Veld, Ann Saada, Mark A. Magnuson, Benjamin Glaser, Yuval Dor
Article
Endocrinology & Metabolism
Richard G. Kibbey, Cheol Soo Choi, Hui-Young Lee, Over Cabrera, Rebecca L. Pongratz, Xiaojian Zhao, Andreas L. Birkenfeld, Changhong Li, Per-Olof Berggren, Charles Stanley, Gerald I. Shulman
Article
Endocrinology & Metabolism
Sara E. Pinney, Karthik Ganapathy, Jonathan Bradfield, David Stokes, Ariella Sasson, Katarzyna Mackiewicz, Kara Boodhansingh, Nkecha Hughes, Susan Becker, Stephanie Givler, Courtney Macmullen, Dimitrios Monos, Arupa Ganguly, Hakon Hakonarson, Charles A. Stanley
HORMONE RESEARCH IN PAEDIATRICS
(2013)
Article
Biochemistry & Molecular Biology
Changhong Li, Chengyang Liu, Itzhak Nissim, Jie Chen, Pan Chen, Nicolai Doliba, Tingting Zhang, Ilana Nissim, Yevgeny Daikhin, David Stokes, Marc Yudkoff, Michael J. Bennett, Charles A. Stanley, Franz M. Matschinsky, Ali Naji
JOURNAL OF BIOLOGICAL CHEMISTRY
(2013)
Article
Endocrinology & Metabolism
K. E. Snider, S. Becker, L. Boyajian, S. -L. Shyng, C. MacMullen, N. Hughes, K. Ganapathy, T. Bhatti, C. A. Stanley, A. Ganguly
JOURNAL OF CLINICAL ENDOCRINOLOGY & METABOLISM
(2013)
Article
Endocrinology & Metabolism
Cresio Alves, Julia Constanca, Diva D. De Leon, Kara Snider, Charles Stanley
JOURNAL OF PEDIATRIC ENDOCRINOLOGY & METABOLISM
(2013)
Article
Pediatrics
William H. Peranteau, Andrew A. Palladino, Tricia R. Bhatti, Susan A. Becker, Lisa J. States, Charles A. Stanley, N. Scott Adzick
JOURNAL OF PEDIATRIC SURGERY
(2013)
Article
Pediatrics
Pablo Laje, Andrew A. Palladino, Tricia R. Bhatti, Lisa J. States, Charles A. Stanley, N. Scott Adzick
JOURNAL OF PEDIATRIC SURGERY
(2013)
Article
Pediatrics
Pablo Laje, Lisa J. States, Hongming Zhuang, Susan A. Becker, Andrew A. Palladino, Charles A. Stanley, N. Scott Adzick
JOURNAL OF PEDIATRIC SURGERY
(2013)
Editorial Material
Pediatrics
Colin P. Hawkes, Charles A. Stanley
JOURNAL OF PEDIATRICS
(2014)
Article
Biochemistry & Molecular Biology
Ming Li, Changhong Li, Aron Allen, Charles A. Stanley, Thomas J. Smith
NEUROCHEMICAL RESEARCH
(2014)
Article
Medicine, General & Internal
L. C. Tegtmeyer, S. Rust, M. van Scherpenzeel, B. G. Ng, M. -E. Losfeld, S. Timal, K. Raymond, P. He, M. Ichikawa, J. Veltman, K. Huijben, Y. S. Shin, V. Sharma, M. Adamowicz, M. Lammens, J. Reunert, A. Witten, E. Schrapers, G. Matthijs, J. Jaeken, D. Rymen, T. Stojkovic, P. Laforet, F. Petit, O. Aumaitre, E. Czarnowska, M. Piraud, T. Podskarbi, C. A. Stanley, R. Matalon, P. Burda, S. Seyyedi, V. Debus, P. Socha, J. Sykut-Cegielska, F. van Spronsen, L. de Meirleir, P. Vajro, T. DeClue, C. Ficicioglu, Y. Wada, R. A. Wevers, D. Vanderschaeghe, N. Callewaert, R. Fingerhut, E. van Schaftingen, H. H. Freeze, E. Morava, D. J. Lefeber, T. Marquardt
NEW ENGLAND JOURNAL OF MEDICINE
(2014)
Article
Endocrinology & Metabolism
Tingting Zhang, Pan Chen, Charles A. Stanley, Toshinori Hoshi, Changhong Li
Review
Endocrinology & Metabolism
Paul S. Thornton, Charles A. Stanley, Diva D. De Leon
Summary: Congenital hyperinsulinism is the most common cause of persistent hypoglycemia in neonates, infants, and children. This field has made significant advancements, including the development of insulin radioimmunoassay, the discovery of different genetic subtypes, and the recognition and treatment of focal hyperinsulinism. However, treatment options for some patients remain limited, calling for further development.
HORMONE RESEARCH IN PAEDIATRICS
(2022)