| Rescue of expression and function of long QT syndrome-causing mutant hERG channels by enhancing channel stability in the plasma membrane. | Rescue of expression and function of long QT syndrome-causing mutant hERG channels by enhancing channel stability in the plasma membrane.
Davis J, Cornwell JD, Campagna N, Guo J, Li W, Yang T, Wang T, Zhang S., Free PMC Article | 10/23/2024 |
| An intracellular hydrophobic nexus critical for hERG1 channel slow deactivation. | An intracellular hydrophobic nexus critical for hERG1 channel slow deactivation.
Stevens-Sostre WA, Flores-Aldama L, Bustos D, Li J, Morais-Cabral JH, Delemotte L, Robertson GA., | 08/16/2024 |
| Clinical interpretation of KCNH2 variants using a robust PS3/BS3 functional patch-clamp assay. | Clinical interpretation of KCNH2 variants using a robust PS3/BS3 functional patch-clamp assay.
Thomson KL, Jiang C, Richardson E, Westphal DS, Burkard T, Wolf CM, Vatta M, Harrison SM, Ingles J, Bezzina CR, Kroncke BM, Vandenberg JI, Ng CA., Free PMC Article | 04/17/2024 |
| An LQT2-related mutation in the voltage-sensing domain is involved in switching the gating polarity of hERG. | An LQT2-related mutation in the voltage-sensing domain is involved in switching the gating polarity of hERG.
Liu Z, Wang F, Yuan H, Tian F, Yang C, Hu F, Liu Y, Tang M, Ping M, Kang C, Luo T, Yang G, Hu M, Gao Z, Li P., Free PMC Article | 03/11/2024 |
| Functional and clinical characterization of a novel homozygous KCNH2 missense variant in the pore region of Kv11.1 leading to a viable but severe long-QT syndrome. | Functional and clinical characterization of a novel homozygous KCNH2 missense variant in the pore region of Kv11.1 leading to a viable but severe long-QT syndrome.
Delinière A, Jaupart L, Janin A, Millat G, Boulin T, Andrini O, Chevalier P. | 02/5/2024 |
| Dissecting the associations of KCNH2 genetic polymorphisms with various types of cardiac arrhythmias. | Dissecting the associations of KCNH2 genetic polymorphisms with various types of cardiac arrhythmias.
Li S, Zhang Z, Ding Y, Yu T, Qin Z, Guo S. | 02/2/2024 |
| Identification and characterization of two novel KCNH2 mutations contributing to long QT syndrome. | Identification and characterization of two novel KCNH2 mutations contributing to long QT syndrome.
Owusu-Mensah A, Treat J, Bernardi J, Pfeiffer R, Goodrow R, Tsevi B, Lam V, Audette M, Cordeiro JM, Deo M., Free PMC Article | 01/30/2024 |
| Translation reinitiation in c.453delC frameshift mutation of KCNH2 producing functional hERG K+ channels with mild dominant negative effect in the heterozygote patient-derived iPSC cardiomyocytes. | Translation reinitiation in c.453delC frameshift mutation of KCNH2 producing functional hERG K+ channels with mild dominant negative effect in the heterozygote patient-derived iPSC cardiomyocytes.
Park NK, Park SJ, Park YG, Moon SH, Woo J, Kim HJ, Kim SJ, Choi SW. | 01/11/2024 |
| KCNH2A561V Heterozygous Mutation Inhibits KCNH2 Protein Expression via The Activation of UPR Mediated by ATF6. | KCNH2A561V Heterozygous Mutation Inhibits KCNH2 Protein Expression via The Activation of UPR Mediated by ATF6.
Chen B, Tan L, Chen D, Wang X, Liu J, Huang X, Wang Y, Huang S, Mao F, Lian J., Free PMC Article | 12/1/2023 |
| Integrins regulate hERG1 dynamics by girdin-dependent Galphai3: signaling and modeling in cancer cells. | Integrins regulate hERG1 dynamics by girdin-dependent Gαi3: signaling and modeling in cancer cells.
Duranti C, Iorio J, Bagni G, Chioccioli Altadonna G, Fillion T, Lulli M, D'Alessandro FN, Montalbano A, Lastraioli E, Fanelli D, Coppola S, Schmidt T, Piazza F, Becchetti A, Arcangeli A., Free PMC Article | 11/14/2023 |
| KCNH2 mutation c.3099_3112del causes congenital long QT syndrome type 2 with gender differences. | KCNH2 mutation c.3099_3112del causes congenital long QT syndrome type 2 with gender differences.
Ke Z, Li C, Bai G, Tan L, Wang J, Zhou M, Zhou J, Chen SY, Dong X., Free PMC Article | 11/2/2023 |
| KCNH2 variants in a family with epilepsy and long QT syndrome: A case report and literature review. | KCNH2 variants in a family with epilepsy and long QT syndrome: A case report and literature review.
Zhou Y, Hao N, Sander JW, Lin X, Xiong W, Zhou D. | 08/28/2023 |
| Hydroxychloroquine Attenuates hERG Channel by Promoting the Membrane Channel Degradation: Computational Simulation and Experimental Evidence for QT-Interval Prolongation with Hydroxychloroquine Treatment. | Hydroxychloroquine Attenuates hERG Channel by Promoting the Membrane Channel Degradation: Computational Simulation and Experimental Evidence for QT-Interval Prolongation with Hydroxychloroquine Treatment.
Wang X, Feng Y, Liu S, Liu J, Pan S, Wei L, Ma Y, Liu Z, Xing Y, Wang J, Cui Q, Zhang Y, Wang T, Cai C. | 08/23/2023 |
| Mechanisms of fever-induced QT prolongation and torsades de pointes in patients with KCNH2 mutation. | Mechanisms of fever-induced QT prolongation and torsades de pointes in patients with KCNH2 mutation.
Usuda K, Hayashi K, Nakajima T, Kurata Y, Cui S, Kusayama T, Tsuda T, Tada H, Kato T, Sakata K, Usui S, Fujino N, Tanaka Y, Kaneko Y, Kurabayashi M, Tange S, Saito T, Ohta K, Yamagishi M, Takamura M., Free PMC Article | 07/6/2023 |
| Non-missense variants of KCNH2 show better outcomes in type 2 long QT syndrome. | Non-missense variants of KCNH2 show better outcomes in type 2 long QT syndrome.
Aizawa T, Wada Y, Hasegawa K, Huang H, Imamura T, Gao J, Kashiwa A, Kohjitani H, Fukuyama M, Kato K, Kato ET, Hisamatsu T, Ohno S, Makiyama T, Kimura T, Horie M., Free PMC Article | 04/19/2023 |
| Reclassification of a likely pathogenic Dutch founder variant in KCNH2; implications of reduced penetrance. | Reclassification of a likely pathogenic Dutch founder variant in KCNH2; implications of reduced penetrance.
Copier JS, Bootsma M, Ng CA, Wilde AAM, Bertels RA, Bikker H, Christiaans I, van der Crabben SN, Hol JA, Koopmann TT, Knijnenburg J, Lommerse AAJ, van der Smagt JJ, Bezzina CR, Vandenberg JI, Verkerk AO, Barge-Schaapveld DQCM, Lodder EM., Free PMC Article | 03/24/2023 |
| Noncanonical electromechanical coupling paths in cardiac hERG potassium channel. | Noncanonical electromechanical coupling paths in cardiac hERG potassium channel.
Bassetto CAZ Jr, Costa F, Guardiani C, Bezanilla F, Giacomello A., Free PMC Article | 03/6/2023 |
| hERG1 channel subunit composition mediates proton inhibition of rapid delayed rectifier potassium current (IKr) in cardiomyocytes derived from hiPSCs. | hERG1 channel subunit composition mediates proton inhibition of rapid delayed rectifier potassium current (I(Kr)) in cardiomyocytes derived from hiPSCs.
Ukachukwu CU, Jimenez-Vazquez EN, Jain A, Jones DK., Free PMC Article | 03/1/2023 |
| The voltage-sensing domain of a hERG1 mutant is a cation-selective channel. | The voltage-sensing domain of a hERG1 mutant is a cation-selective channel.
Kudaibergenova M, Guo J, Khan HM, Lees-Miller J, Mousaei M, Miranda W, Ngo VA, Noskov SY, Tieleman DP, Duff HJ., Free PMC Article | 02/24/2023 |
| Integrated analysis of the voltage-gated potassium channel-associated gene KCNH2 across cancers. | Integrated analysis of the voltage-gated potassium channel-associated gene KCNH2 across cancers.
Zheng Z, Song Y., Free PMC Article | 02/23/2023 |
| Same family, same mutation, different ECG. | Same family, same mutation, different ECG.
Akbuğa K, Karanfil M., Free PMC Article | 01/21/2023 |
| KCNH2 encodes a nuclear-targeted polypeptide that mediates hERG1 channel gating and expression. | KCNH2 encodes a nuclear-targeted polypeptide that mediates hERG1 channel gating and expression.
Jain A, Stack O, Ghodrati S, Sanchez-Conde FG, Ukachukwu CU, Salwi S, Jimenez-Vazquez EN, Jones DK., Free PMC Article | 01/14/2023 |
| Analysis of CACNA1C and KCNH2 Risk Variants on Cardiac Autonomic Function in Patients with Schizophrenia. | Analysis of CACNA1C and KCNH2 Risk Variants on Cardiac Autonomic Function in Patients with Schizophrenia.
Refisch A, Komatsuzaki S, Ungelenk M, Schumann A, Chung HY, Schilling SS, Jantzen W, Schröder S, Nöthen MM, Mühleisen TW, Hübner CA, Bär KJ., Free PMC Article | 12/3/2022 |
| Multiple mechanisms underlie reduced potassium conductance in the p.T1019PfsX38 variant of hERG. | Multiple mechanisms underlie reduced potassium conductance in the p.T1019PfsX38 variant of hERG.
Al Salmani MK, Tavakoli R, Zaman W, Al Harrasi A., Free PMC Article | 09/10/2022 |
| Potassium Ion Channel Protein (KCNH) Levels in Patients with Fibromyalgia Syndrome. | Potassium Ion Channel Protein (KCNH) Levels in Patients with Fibromyalgia Syndrome.
Tas A, Hayta E, Karadag A, Zontul C, Ozmen E, Aydin S, Silig Y. | 07/16/2022 |