Gene Symbol: Kv1.1
Description: potassium voltage-gated channel subfamily A member 1
Alias: AEMK, EA1, HBK1, HUK1, KV1.1, MBK1, MK1, RBK1, potassium voltage-gated channel subfamily A member 1, potassium channel, voltage gated shaker related subfamily A, member 1, potassium voltage-gated channel, shaker-related subfamily, member 1 (episodic ataxia with myokymia), voltage-gated K(+) channel HuKI, voltage-gated potassium channel HBK1, voltage-gated potassium channel subunit Kv1.1
Species: human
Products:     Kv1.1

Top Publications

  1. Jow F, Zhang Z, Kopsco D, Carroll K, Wang K. Functional coupling of intracellular calcium and inactivation of voltage-gated Kv1.1/Kvbeta1.1 A-type K+ channels. Proc Natl Acad Sci U S A. 2004;101:15535-40 pubmed
  2. Eunson L, Rea R, Zuberi S, Youroukos S, Panayiotopoulos C, Liguori R, et al. Clinical, genetic, and expression studies of mutations in the potassium channel gene KCNA1 reveal new phenotypic variability. Ann Neurol. 2000;48:647-56 pubmed
    Episodic ataxia type 1 (EA1) is an autosomal dominant central nervous system potassium channelopathy characterized by brief attacks of cerebellar ataxia and continuous interictal myokymia...
  3. Hoopengardner B, Bhalla T, Staber C, Reenan R. Nervous system targets of RNA editing identified by comparative genomics. Science. 2003;301:832-6 pubmed
    ..These results point to a pivotal role for RNA editing in nervous system function. ..
  4. Graves T, Rajakulendran S, Zuberi S, Morris H, Schorge S, Hanna M, et al. Nongenetic factors influence severity of episodic ataxia type 1 in monozygotic twins. Neurology. 2010;75:367-72 pubmed publisher
    Episodic ataxia type 1 (EA1) is a monogenic channelopathy caused by mutations of the potassium channel gene KCNA1...
  5. Tomlinson S, Tan S, Kullmann D, Griggs R, Burke D, Hanna M, et al. Nerve excitability studies characterize Kv1.1 fast potassium channel dysfunction in patients with episodic ataxia type 1. Brain. 2010;133:3530-40 pubmed publisher
    ..1 dysfunction in patients with episodic ataxia type 1. The simple 15 min test may be useful in diagnosis, since it can differentiate patients with episodic ataxia type 1 from normal controls with high sensitivity and specificity. ..
  6. Browne D, Gancher S, Nutt J, Brunt E, Smith E, Kramer P, et al. Episodic ataxia/myokymia syndrome is associated with point mutations in the human potassium channel gene, KCNA1. Nat Genet. 1994;8:136-40 pubmed
    ..Mutation analysis of the KCNA1 coding region in these families identified four different missense point mutations present in the heterozygous state, indicating that EA/myokymia can result from mutations in this gene. ..
  7. Comu S, Giuliani M, Narayanan V. Episodic ataxia and myokymia syndrome: a new mutation of potassium channel gene Kv1.1. Ann Neurol. 1996;40:684-7 pubmed
    ..This disease has been associated with point mutations in the potassium channel gene Kv1.1 (KCNA1), located at chromosome 12p13. Here, we describe a novel mutation within this gene in a newly diagnosed family. ..
  8. Scheffer H, Brunt E, Mol G, van der Vlies P, Stulp R, Verlind E, et al. Three novel KCNA1 mutations in episodic ataxia type I families. Hum Genet. 1998;102:464-6 pubmed
    ..By direct sequence analysis, a different missense mutation of the potassium channel gene (KCNA1) has been identified in three families with EA. ..
  9. Zuberi S, Eunson L, Spauschus A, De Silva R, Tolmie J, Wood N, et al. A novel mutation in the human voltage-gated potassium channel gene (Kv1.1) associates with episodic ataxia type 1 and sometimes with partial epilepsy. Brain. 1999;122 ( Pt 5):817-25 pubmed
    Episodic ataxia type 1 (EA1) is a rare autosomal dominant disorder characterized by brief episodes of ataxia associated with continuous interattack myokymia. Point mutations in the human voltage-gated potassium channel (Kv1...

More Information

Publications120 found, 100 shown here

  1. Kinali M, Jungbluth H, Eunson L, Sewry C, Manzur A, Mercuri E, et al. Expanding the phenotype of potassium channelopathy: severe neuromyotonia and skeletal deformities without prominent Episodic Ataxia. Neuromuscul Disord. 2004;14:689-93 pubmed
    ..His mother presented some years later with typical features of Episodic Ataxia type 1 (EA1), with episodes of ataxia lasting a few minutes provoked by exercise...
  2. Huang C, Wang W, Sun C, Wang Y, Li L, Li Y, et al. Echinocystic acid ameliorates hyperhomocysteinemia-induced vascular endothelial cell injury through regulating NF-?B and CYP1A1. Exp Ther Med. 2017;14:4174-4180 pubmed publisher
    ..NC), model control (MC), vitamin control (VC; folic acid 1 mg/kg + vitamin B2 2 mg/kg + vitamin B12 10u g/kg), EA1 (20 mg/kg EA) and EA2 (40 mg/kg EA)...
  3. Lebsir D, Guemri J, Kereselidze D, Grison S, Benderitter M, Pech A, et al. Repeated potassium iodide exposure during pregnancy impairs progeny's brain development. Neuroscience. 2019;: pubmed publisher
    ..To conclude, repeated KI prophylaxis is not adequate during pregnancy since it led to long-term irreversible neurotoxicity in the male progeny. ..
  4. Pérez Verdaguer M, Capera J, Ortego Domínguez M, Bielanska J, Comes N, Montoro R, et al. Caveolar targeting links Kv1.3 with the insulin-dependent adipocyte physiology. Cell Mol Life Sci. 2018;75:4059-4075 pubmed publisher
    ..Our data shed light on the putative role of Kv1.3 in weight gain and insulin-dependent responses contributing to knowledge about adipocyte physiology. ..
  5. Meng L, Xie Z, Zhang Q, Li Y, Yang F, Chen Z, et al. Scorpion Potassium Channel-blocking Defensin Highlights a Functional Link with Neurotoxin. J Biol Chem. 2016;291:7097-106 pubmed publisher
    ..These findings not only demonstrate that defensins from invertebrate animals are a novel type of potassium channel blockers but also provide evidence of a functional link between defensins and neurotoxins. ..
  6. Wang D, Liu Y, Lu P, Zhu D, Zhu Y. 15-oxo-ETE-induced internal carotid artery constriction in hypoxic rats is mediated by potassium channels. Physiol Res. 2016;65:391-9 pubmed
    ..1. In conclusion, these results suggest that 15-oxo-ETE leads to the contraction of the ICA, especially under hypoxic conditions and that specific Kv channels may play an important role in 15-oxo-ETE-induced ICA constriction. ..
  7. Kuwabara S, Misawa S. Acquired and genetic channelopathies: in vivo assessment of axonal excitability. Exp Neurol. 2015;263:368-71 pubmed publisher
    ..This commentary aims to highlight findings of this study in a broader context, and provides possible explanations for the discrepancy of patterns of axonal excitability changes in acquired and genetic potassium channelopathies. ..
  8. Chen J, Winston J, Fu Y, Guptarak J, Jensen K, Shi X, et al. Genesis of anxiety, depression, and ongoing abdominal discomfort in ulcerative colitis-like colon inflammation. Am J Physiol Regul Integr Comp Physiol. 2015;308:R18-27 pubmed publisher
    ..Alterations in the expression of voltage- and ligand-gated channels are associated with the induction of mood disorders following colon inflammation. ..
  9. Desir G, Hamlin H, Puente E, Reilly R, Hildebrandt F, Igarashi P. Isolation of putative voltage-gated epithelial K-channel isoforms from rabbit kidney and LLC-PK1 cells. Am J Physiol. 1992;262:F151-7 pubmed
    ..five putative transmembrane segments and are 79-97% identical to two Shaker isoforms expressed in rat brain (RBK1 and RBK2). Sequence similarity is greatest in the putative transmembrane segments S1-S5...
  10. Smoot B, Kober K, Paul S, Levine J, Abrams G, Mastick J, et al. Potassium Channel Candidate Genes Predict the Development of Secondary Lymphedema Following Breast Cancer Surgery. Nurs Res. 2017;66:85-94 pubmed publisher
    ..These preliminary findings suggest that K channel genes play a role in the development of secondary LE. ..
  11. Imperial J, Cabang A, Song J, Raghuraman S, Gajewiak J, Watkins M, et al. A family of excitatory peptide toxins from venomous crassispirine snails: using Constellation Pharmacology to assess bioactivity. Toxicon. 2014;89:45-54 pubmed publisher
  12. García Fernández R, Peigneur S, Pons T, Alvarez C, González L, Chavez M, et al. The Kunitz-Type Protein ShPI-1 Inhibits Serine Proteases and Voltage-Gated Potassium Channels. Toxins (Basel). 2016;8:110 pubmed publisher
    ..However, we detected two subtle patterns of coordinated amino acid substitutions flanking the conserved cysteine residues at the N- and C-terminal ends. ..
  13. Wang J, Li G, Tan B, Xiong X, Kong X, Xiao D, et al. Oral administration of putrescine and proline during the suckling period improves epithelial restitution after early weaning in piglets. J Anim Sci. 2015;93:1679-88 pubmed publisher
  14. Kanemasa T, Gan L, Perney T, Wang L, Kaczmarek L. Electrophysiological and pharmacological characterization of a mammalian Shaw channel expressed in NIH 3T3 fibroblasts. J Neurophysiol. 1995;74:207-17 pubmed
    ..1 currents could be varied over a wide range without attenuation of action potential height. Our results suggest that the Kv3.1 channel may provide rapidly firing neurons with a high safety factor for impulse propagation...
  15. Simeone T, Matthews S, Samson K, Simeone K. Regulation of brain PPARgamma2 contributes to ketogenic diet anti-seizure efficacy. Exp Neurol. 2017;287:54-64 pubmed publisher
    ..1KO mice by ~80% (P<0.01). Our findings implicate brain PPAR?2 among the mechanisms by which the KD reduces seizures and strongly support the development of PPAR?2 as a therapeutic target for severe, refractory epilepsy. ..
  16. Yin N, Zhang Y, Yun Z, Liu Q, Qu G, Zhou Q, et al. Silver nanoparticle exposure induces rat motor dysfunction through decrease in expression of calcium channel protein in cerebellum. Toxicol Lett. 2015;237:112-20 pubmed publisher
    ..Possible counteractions may accordingly be suggested to attenuate the unexpected harmful effects in biological applications of AgNPs. ..
  17. Orts D, Peigneur S, Madio B, Cassoli J, Montandon G, Pimenta A, et al. Biochemical and electrophysiological characterization of two sea anemone type 1 potassium toxins from a geographically distant population of Bunodosoma caissarum. Mar Drugs. 2013;11:655-79 pubmed publisher
    ..6 over hKv1.3, rKv1.1, Shaker IR and rKv1.2. Furthermore, we also report for the first time a venom composition and biological activity comparison between two geographically distant populations of sea anemones. ..
  18. Lu P, Chen X, Feng Y, Zeng Q, Jiang C, Zhu X, et al. Integrated transcriptome analysis of human iPS cells derived from a fragile X syndrome patient during neuronal differentiation. Sci China Life Sci. 2016;59:1093-1105 pubmed
    ..In conclusion, we demonstrated that the FMRP deficiency in FXS patients has significant impact on the gene expression patterns during development, which will help to discover potential targeting candidates for the cure of FXS symptoms. ..
  19. Mishra V, Gautier N, Glasscock E. Simultaneous Video-EEG-ECG Monitoring to Identify Neurocardiac Dysfunction in Mouse Models of Epilepsy. J Vis Exp. 2018;: pubmed publisher
    ..Video-EEG-ECG monitoring in mouse models of epilepsy or other neurological disease provides a powerful tool to identify dysfunction at the level of the brain, heart, or brain-heart interactions. ..
  20. Cao X, Oertel D. Genetic perturbations suggest a role of the resting potential in regulating the expression of the ion channels of the KCNA and HCN families in octopus cells of the ventral cochlear nucleus. Hear Res. 2017;345:57-68 pubmed publisher
  21. Manville R, Neverisky D, Abbott G. SMIT1 Modifies KCNQ Channel Function and Pharmacology by Physical Interaction with the Pore. Biophys J. 2017;113:613-626 pubmed publisher
  22. Zhu Z, Wang D, Jiao W, Chen G, Cao Y, Zhang Q, et al. Bioinformatics analyses of pathways and gene predictions in IL-1α and IL-1β knockout mice with spinal cord injury. Acta Histochem. 2017;119:663-670 pubmed publisher
    ..Compared with IL-1β-KO, IL-1α-KO may improve the outcome of SCI via the alteration of hematopoietic cell lineage and PI3K-Akt signalling pathways. ..
  23. Verschuren E, Hoenderop J, Peters D, Arjona F, Bindels R. Tubular flow activates magnesium transport in the distal convoluted tubule. FASEB J. 2019;33:5034-5044 pubmed publisher
    ..Verschuren, E. H. J., Hoenderop, J. G. J., Peters, D. J. M., Arjona, F. J., Bindels, R. J. M. Tubular flow activates magnesium transport in the distal convoluted tubule. ..
  24. Wang T, Wang D, Lyu Y, Feng E, Zhu L, Liu C, et al. Construction of a high-efficiency cloning system using the Golden Gate method and I-SceI endonuclease for targeted gene replacement in Bacillus anthracis. J Biotechnol. 2018;271:8-16 pubmed publisher
    ..Here, a B. anthracis S-layer protein EA1 allelic exchange vector was successfully constructed using the Golden Gate method...
  25. Liu Y, Sun Y, Li Y, Bai H, Xu S, Xu H, et al. Identification and differential expression of microRNAs in the testis of chicken with high and low sperm motility. Theriogenology. 2018;122:94-101 pubmed publisher
    ..qPCR was then used to further validate their expressions. The results here provided a deep insight into the expressions of the miRNAs in the testes of chickens and suggested their roles in sperm motility regulation. ..
  26. Wang Y, Yuan S, Tao H, Wang L, Zhang Z, Liu C. Production and cell surface display of recombinant anthrax protective antigen on the surface layer of attenuated Bacillus anthracis. World J Microbiol Biotechnol. 2015;31:345-52 pubmed publisher
    ..a translational fusion harboring the DNA fragments encoding the cell wall-targeting domain of the S-layer protein EA1 and the anthrax PA. Crerecombinase action at the loxP sites excised the antibiotic marker...
  27. Tomlinson S, Hanna M, Kullmann D, Tan S, Burke D. Clinical neurophysiology of the episodic ataxias: insights into ion channel dysfunction in vivo. Clin Neurophysiol. 2009;120:1768-76 pubmed publisher
    ..The clinical, genetic and electrophysiological features of EA1 and EA2 are outlined, and a protocol for the assessment of these patients is proposed.
  28. Chen J, Zhang C, Yang W, Cao Z, Li W, Chen Z, et al. SjAPI-2 is the first member of a new neurotoxin family with Ascaris-type fold and KCNQ1 inhibitory activity. Int J Biol Macromol. 2015;79:504-10 pubmed publisher
    ..5±169.9 nM. To the best of our knowledge, SjAPI-2 is the first neurotoxin with a unique Ascaris-type fold, providing novel insights into the divergent evolution of neurotoxins from venomous animals. ..
  29. Ford N, Hecht K, Hu D, Orr G, Xiong Y, Squier T, et al. Antigen Binding and Site-Directed Labeling of Biosilica-Immobilized Fusion Proteins Expressed in Diatoms. ACS Synth Biol. 2016;5:193-9 pubmed publisher
    ..tagged fusion proteins and (2) high and low molecular mass antigens, the Bacillus anthracis surface layer protein EA1 or small molecule explosive trinitrotoluene (TNT), to biosilica-immobilized single chain antibodies...
  30. Seagar M, Russier M, Caillard O, MAULET Y, Fronzaroli Molinieres L, de San Feliciano M, et al. LGI1 tunes intrinsic excitability by regulating the density of axonal Kv1 channels. Proc Natl Acad Sci U S A. 2017;114:7719-7724 pubmed publisher
    ..1 and Kv1.2 via a posttranscriptional mechanism, resulting in a reduction in the capacity of axonal D-type current to limit glutamate release, thus contributing to epileptogenesis. ..
  31. Sharma V, Nair S, Jaitley P, Nakade U, Sharma A, Choudhury S, et al. ATP-sensitive and maxi potassium channels regulate BRL 37344-induced tocolysis in buffaloes-an in vitro study. Theriogenology. 2018;107:194-202 pubmed publisher
  32. Ye F, Hu Y, Yu W, Xie Z, Hu J, Cao Z, et al. The Scorpion Toxin Analogue BmKTX-D33H as a Potential Kv1.3 Channel-Selective Immunomodulator for Autoimmune Diseases. Toxins (Basel). 2016;8:115 pubmed publisher
    ..Together, our data indicate that BmKTX-D33H is a Kv1.3 channel-specific blocker. Finally, the remarkable selectivity of BmKTX-D33H highlights the great potential of evolutionary-guided peptide drug design in future studies. ..
  33. Jones K, Corbin J, Huntsman M. Neonatal NMDA receptor blockade disrupts spike timing and glutamatergic synapses in fast spiking interneurons in a NMDA receptor hypofunction model of schizophrenia. PLoS ONE. 2014;9:e109303 pubmed publisher
    ..Overall, we propose these physiological disturbances represent a general impairment to the physiological maturation of FSIs which may contribute to schizophrenia-like behaviors produced by this model. ..
  34. Kuba H, Yamada R, Ishiguro G, Adachi R. Redistribution of Kv1 and Kv7 enhances neuronal excitability during structural axon initial segment plasticity. Nat Commun. 2015;6:8815 pubmed publisher
  35. Sakamoto K, Suzuki Y, Yamamura H, Ohya S, Muraki K, Imaizumi Y. Molecular mechanisms underlying pimaric acid-induced modulation of voltage-gated K+ channels. J Pharmacol Sci. 2017;133:223-231 pubmed publisher
    ..1-KV2.1 channels may be critical for the effects of PiMA, providing beneficial information for drug development of KV channel openers. ..
  36. Viering D, de Baaij J, Walsh S, Kleta R, Bockenhauer D. Genetic causes of hypomagnesemia, a clinical overview. Pediatr Nephrol. 2017;32:1123-1135 pubmed publisher
    ..In this review, we discuss general mechanisms and symptoms of genetic causes of hypomagnesemia as well as the specific molecular mechanisms and clinical phenotypes associated with each syndrome. ..
  37. Kuryshev Y, Gudz T, Brown A, Wible B. KChAP as a chaperone for specific K(+) channels. Am J Physiol Cell Physiol. 2000;278:C931-41 pubmed
    ..1 and Kv4.3 from heart. We propose that KChAP is a chaperone for specific Kv channels and may have this function in cardiomyocytes where Kv4.3 produces the transient outward current, I(to)...
  38. Méneret A, Roze E. Paroxysmal movement disorders: An update. Rev Neurol (Paris). 2016;172:433-445 pubmed publisher
    ..The present report is a general overview that aims to help clinicians diagnose and treat patients with paroxysmal movement disorders. ..
  39. Xiang F, Xie Z, Feng J, Yang W, Cao Z, Li W, et al. Plectasin, first animal toxin-like fungal defensin blocking potassium channels through recognizing channel pore region. Toxins (Basel). 2015;7:34-42 pubmed publisher
  40. Baloh R, Jen J. Genetics of familial episodic vertigo and ataxia. Ann N Y Acad Sci. 2002;956:338-45 pubmed
    ..An ion channel mutation shared by brain and inner ear could explain the combined central and peripheral features of the syndrome. ..
  41. Hall M, Weidner D, Edwards M, Schwalbe R. Complex N-Glycans Influence the Spatial Arrangement of Voltage Gated Potassium Channels in Membranes of Neuronal-Derived Cells. PLoS ONE. 2015;10:e0137138 pubmed publisher
    ..1a, like Kv3.1b, provides a mechanism for the distribution of these proteins to the cell body and outgrowths and thereby can generate different voltage-dependent conductances in these membranes. ..
  42. Swain S, Sahoo N, Dennhardt S, Schönherr R, Heinemann S. Ca(2+)/calmodulin regulates Kvβ1.1-mediated inactivation of voltage-gated K(+) channels. Sci Rep. 2015;5:15509 pubmed publisher
    ..1 subunit a multifunctional receptor that integrates cytosolic signals to be transduced to altered electrical cellular activity. ..
  43. Peigneur S, Esaki N, Yamaguchi Y, Tytgat J, Sato K. Effects of deletion and insertion of amino acids on the activity of HelaTx1, a scorpion toxin on potassium channels. Toxicon. 2016;111:1-5 pubmed publisher
    ..Further deletion of N-terminal Ser residue and insertion of Ala in the middle part of the molecule affected the CD spectra and resulted in the decrease of activity. ..
  44. Subramani B, Subbannagounder S, Palanivel S, Ramanathanpullai C, Sivalingam S, Yakub A, et al. Generation and characterization of human cardiac resident and non-resident mesenchymal stem cell. Cytotechnology. 2016;68:2061-73 pubmed publisher
    ..TTX in undifferentiated hUC-MSCs as of hC-MSCs. Thus, the hUC-MSCs can be exploited as a potential candidate for future cardiac regeneration. ..
  45. Al Mohammed H, Paray B, Rather I. Anticancer activity of EA1 extracted from Equisetum arvense. Pak J Pharm Sci. 2017;30:1947-1950 pubmed
    ..was carried out to investigate the anticancer activity of ethanol extract of arial parts of Equisetum arvense (EA1)...
  46. Lim I, Hall D, Hell J. Selectivity and promiscuity of the first and second PDZ domains of PSD-95 and synapse-associated protein 102. J Biol Chem. 2002;277:21697-711 pubmed
    ..Two of these proteins, brain-specific angiogenesis inhibitor 1 and protein kinase Calpha, co-immunoprecipitated with PSD-95 and SAP102 from rat brain extracts. ..
  47. Bergeron E, Patskovsky S, Rioux D, Meunier M. 3D multiplexed immunoplasmonics microscopy. Nanoscale. 2016;8:13263-72 pubmed publisher
    ..Thus, 3D multiplexed immunoplasmonics microscopy is ready for clinical applications as a cost-efficient alternative to immunofluorescence. ..
  48. Wang J, Wang J, Golovina V, Li L, Platoshyn O, Yuan J. Role of K(+) channel expression in polyamine-dependent intestinal epithelial cell migration. Am J Physiol Cell Physiol. 2000;278:C303-14 pubmed
    ..1 channel expression. The subsequent membrane hyperpolarization raises [Ca(2+)](cyt) by increasing the driving force (the electrochemical gradient) for Ca(2+) influx and thus stimulates cell migration...
  49. Enders T, Frick E, Strader L. An Arabidopsis kinase cascade influences auxin-responsive cell expansion. Plant J. 2017;92:68-81 pubmed publisher
    ..We found that MPK1 interacts with and phosphorylates ROP BINDING PROTEIN KINASE 1 (RBK1), a protein kinase that interacts with members of the Rho-like GTPases from Plants (ROP) small GTPase family...
  50. Aissaoui D, Mlayah Bellalouna S, Jebali J, Abdelkafi Koubaa Z, Souid S, Moslah W, et al. Functional role of Kv1.1 and Kv1.3 channels in the neoplastic progression steps of three cancer cell lines, elucidated by scorpion peptides. Int J Biol Macromol. 2018;111:1146-1155 pubmed publisher
    ..Our results showed that besides they can elucidate the implication of Kv1.1 and Kv1.3 channels in molecular mechanisms of neoplastic progression, KAaH1 and KAaH2 may be used as therapeutic tools against glioblastoma. ..
  51. Zhu P, Li J, Zhang L, Liang Z, Tang B, Liao W, et al. Development-related aberrations in Kv1.1 α-subunit exert disruptive effects on bioelectrical activities of neurons in a mouse model of fragile X syndrome. Prog Neuropsychopharmacol Biol Psychiatry. 2018;84:140-151 pubmed publisher
    ..These data suggest that the expression of the Kv1.1 α-subunit has a profound pathological relevance to hyperexcitability in FXS, as well as implications for normal development, maintenance, and control of neuronal activities. ..
  52. Pinatel D, Hivert B, Boucraut J, Saint Martin M, Rogemond V, Zoupi L, et al. Inhibitory axons are targeted in hippocampal cell culture by anti-Caspr2 autoantibodies associated with limbic encephalitis. Front Cell Neurosci. 2015;9:265 pubmed publisher
    ..This study provides new insight into the potential pathogenic effect of anti-Caspr2 autoantibodies in central hyperexcitability that may be related with perturbation of inhibitory interneuron activity. ..
  53. Begum R, Bakiri Y, Volynski K, Kullmann D. Action potential broadening in a presynaptic channelopathy. Nat Commun. 2016;7:12102 pubmed publisher
    ..Spike broadening leads to increased Ca(2+) influx and GABA release, and decreased spontaneous Purkinje cell firing. We find no evidence for developmental compensation for inherited Kv1.1 dysfunction. ..
  54. Willis M, Leitner I, Seppi K, Trieb M, Wietzorrek G, Marksteiner J, et al. Shaker-related voltage-gated potassium channels Kv1 in human hippocampus. Brain Struct Funct. 2018;223:2663-2671 pubmed publisher
    ..1 and Kv1.2 channel proteins are targeted to distinct compartments of the human hippocampal formation and that this expression pattern largely reflects their distribution profile in murine brain. ..
  55. Voigt N, Dobrev D. Atrial-Selective Potassium Channel Blockers. Card Electrophysiol Clin. 2016;8:411-21 pubmed publisher
    ..We summarize currently available K(+) channel blockers focusing on the most important compounds. ..
  56. Nguyen L, Anderson A. mTOR-dependent alterations of Kv1.1 subunit expression in the neuronal subset-specific Pten knockout mouse model of cortical dysplasia with epilepsy. Sci Rep. 2018;8:3568 pubmed publisher
    ..1 protein expression in association with mTOR hyperactivation in NS-Pten KO mice and suggest a role for mTOR signaling in the modulation of voltage-gated ion channel expression in this model. ..
  57. Tan K, Lennon V, Klein C, Boeve B, Pittock S. Clinical spectrum of voltage-gated potassium channel autoimmunity. Neurology. 2008;70:1883-90 pubmed publisher
    ..Evaluation for VGKC antibodies is recommended in the comprehensive autoimmune serologic testing of subacute idiopathic neurologic disorders. ..
  58. Kole M, Qian J, Waase M, Klassen T, Chen T, Augustine G, et al. Selective Loss of Presynaptic Potassium Channel Clusters at the Cerebellar Basket Cell Terminal Pinceau in Adam11 Mutants Reveals Their Role in Ephaptic Control of Purkinje Cell Firing. J Neurosci. 2015;35:11433-44 pubmed publisher
    ..Therefore, ADAM11 plays a critical role at this central synapse. ..
  59. Imbrici P, Gualandi F, D Adamo M, Masieri M, Cudia P, De Grandis D, et al. A novel KCNA1 mutation identified in an Italian family affected by episodic ataxia type 1. Neuroscience. 2008;157:577-87 pubmed publisher
    Episodic ataxia type 1 (EA1) is a rare human neurological syndrome characterized by continuous myokymia and attacks of generalized ataxia that can be triggered by abrupt movements, emotional stress and fatigue...
  60. Kimbrough M, Thrush C, Barrett E, Bentley F, Sexton K. Are Surgical Milestone Assessments Predictive of In-Training Examination Scores?. J Surg Educ. 2018;75:29-32 pubmed publisher
    ..65-0.76). The Medical Knowledge 1 (MK1) milestone significantly predicted ABSITE percentage correct scores, and explained 60% of the variance (adjusted R<..
  61. Solomon A, Reed R, Benkov K, Kingsbery J, Lusman S, Malter L, et al. Using the Objective Structured Clinical Examination to Assess ACGME Competencies in Pediatric Gastroenterology Fellows. J Pediatr Gastroenterol Nutr. 2017;64:e92-e95 pubmed publisher
    ..Median overall milestone competency scores were 6.9 (PC1), 4.8 (PC2), 5.9 (MK1), 5.7 (MK2), 6.4 (ICS1), 6.9 (Prof1), and 6.7 (Prof3)...
  62. Huang Y, Peng C, Yi Y, Gao B, Shi Q. A Transcriptomic Survey of Ion Channel-Based Conotoxins in the Chinese Tubular Cone Snail (Conus betulinus). Mar Drugs. 2017;15: pubmed publisher
    ..In a word, our present work suggests a high-throughput way to develop conotoxins as potential drugs for treatment of ion channel-associated human diseases. ..
  63. Kim E, Sheng M. Differential K+ channel clustering activity of PSD-95 and SAP97, two related membrane-associated putative guanylate kinases. Neuropharmacology. 1996;35:993-1000 pubmed
    ..4 clustering occurs in > 60% of cotransfected cells, whereas Kv1.1 and Kv1.2 form convincing clusters with PSD-95 only in approximately 10% of cells. ..
  64. Rasband M, Trimmer J. Subunit composition and novel localization of K+ channels in spinal cord. J Comp Neurol. 2001;429:166-76 pubmed
    ..Moreover, the conservation of these characteristics between human, rat, and bovine nodes of Ranvier suggests an essential role for this defined channel complex in spinal cord function. ..
  65. Klassen T, Bomben V, Patel A, Drabek J, Chen T, Gu W, et al. High-resolution molecular genomic autopsy reveals complex sudden unexpected death in epilepsy risk profile. Epilepsia. 2014;55:e6-12 pubmed publisher
    ..However, consideration of the overall biologically relevant variant complexity with its extensive functional epistatic interactions reveals potential personal risk more accurately. ..
  66. Kis Á, Laczi K, Zsíros S, Kós P, Tengölics R, Bounedjoum N, et al. Characterization of the Rhodococcus sp. MK1 strain and its pilot application for bioremediation of diesel oil-contaminated soil. Acta Microbiol Immunol Hung. 2017;:1-20 pubmed publisher
    ..We isolated a novel Rhodococcus sp. MK1 strain capable to degrade the components of diesel oil simultaneously...
  67. Chen H, von Hehn C, Kaczmarek L, Ment L, Pober B, Hisama F. Functional analysis of a novel potassium channel (KCNA1) mutation in hereditary myokymia. Neurogenetics. 2007;8:131-5 pubmed
    ..Electrophysiological studies of the mutant channel expressed in Xenopus oocytes indicated a loss of function. Co-expression of WT and mutant cRNAs significantly reduced whole-oocyte current compared to expression of WT Kv1.1 alone...
  68. Usman H, Mathew M. Potassium channel regulator KCNRG regulates surface expression of Shaker-type potassium channels. Biochem Biophys Res Commun. 2010;391:1301-5 pubmed publisher
    ..Our data indicates that KCNRG is an ER-associated protein, which we propose regulates Kv1 family channel proteins by retaining a fraction of channels in endomembranes. ..
  69. Imbrici P, D Adamo M, Grottesi A, Biscarini A, Pessia M. Episodic ataxia type 1 mutations affect fast inactivation of K+ channels by a reduction in either subunit surface expression or affinity for inactivation domain. Am J Physiol Cell Physiol. 2011;300:C1314-22 pubmed publisher
    Episodic ataxia type 1 (EA1) is an autosomal dominant disorder characterized by continuous myokymia and episodic attacks of ataxia. Mutations in the gene KCNA1 that encodes the voltage-gated potassium channel Kv1...
  70. Tristán Clavijo E, Scholl F, Macaya A, Iglesias G, Rojas A, Lucas M, et al. Dominant-negative mutation p.Arg324Thr in KCNA1 impairs Kv1.1 channel function in episodic ataxia. Mov Disord. 2016;31:1743-1748 pubmed publisher
    ..Arg324Thr in the KCNA1 gene is pathogenic and results in episodic ataxia type 1 through a dominant-negative effect. © 2016 International Parkinson and Movement Disorder Society. ..
  71. Gubitosi Klug R, Mancuso D, Gross R. The human Kv1.1 channel is palmitoylated, modulating voltage sensing: Identification of a palmitoylation consensus sequence. Proc Natl Acad Sci U S A. 2005;102:5964-8 pubmed
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    Episodic ataxia type 1 (EA1) is an autosomal-dominant neurological disease caused by point mutations in the potassium channel-encoding gene KCNA1. It is characterized by attacks of ataxia and continuous myokymia...
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    Mutations in the KCNA1 gene are known to cause episodic ataxia/myokymia syndrome type 1 (EA1)...
  74. Tomlinson S, Rajakulendran S, Tan S, Graves T, Bamiou D, Labrum R, et al. Clinical, genetic, neurophysiological and functional study of new mutations in episodic ataxia type 1. J Neurol Neurosurg Psychiatry. 2013;84:1107-12 pubmed publisher
    Heterozygous mutations in KCNA1 cause episodic ataxia type 1 (EA1), an ion channel disorder characterised by brief paroxysms of cerebellar dysfunction and persistent neuromyotonia...
  75. San Cristobal P, Lainez S, Dimke H, de Graaf M, Hoenderop J, Bindels R. Ankyrin-3 is a novel binding partner of the voltage-gated potassium channel Kv1.1 implicated in renal magnesium handling. Kidney Int. 2014;85:94-102 pubmed publisher
    ..Thus, our observations demonstrate a novel role for ANK3 in modulating the biophysical properties of Kv1.1. Such regulation appears to be particularly important in conditions of high dietary magnesium. ..
  76. Glasscock E, Voigt N, McCauley M, Sun Q, Li N, Chiang D, et al. Expression and function of Kv1.1 potassium channels in human atria from patients with atrial fibrillation. Basic Res Cardiol. 2015;110:505 pubmed publisher
    ..These findings provide evidence of an intrinsic cardiac role of Kv1.1 channels and indicate that they may contribute to atrial repolarization and AF susceptibility. ..
  77. Mishra V, Karumuri B, Gautier N, Liu R, Hutson T, Vanhoof Villalba S, et al. Scn2a deletion improves survival and brain-heart dynamics in the Kcna1-null mouse model of sudden unexpected death in epilepsy (SUDEP). Hum Mol Genet. 2017;26:2091-2103 pubmed publisher
    ..These results show that Scn2a gene deletion acts as protective genetic modifier of SUDEP and suggest measures of brain-heart association as potential indices of SUDEP susceptibility. ..
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    ..and an improved exposure assessment (EA) incorporating time-varying concentrations and residential addresses (EA1). We also compared the association across different EA approaches...
  79. Watanabe T, Shimazaki T, Oda Y. Coordinated Expression of Two Types of Low-Threshold K+ Channels Establishes Unique Single Spiking of Mauthner Cells among Segmentally Homologous Neurons in the Zebrafish Hindbrain. Eneuro. 2017;4: pubmed publisher
    ..1 channels increase firing threshold and decrease the onset latency of spiking. Altogether, coordinated expression of these low-threshold K+ channels with Kv?2 functionally differentiates M cells among homologous neurons. ..
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    ..Kcna1-/- Bad-/- mice also spent significantly less time in seizure than Kcna1-/- mice on P24 and the day of death, showing that BadKO provides seizure resistance in a genetic model of chronic epilepsy...
  81. Freeman S, Conley E, Brennand J, Russell N, Brammar W. Cloning and characterization of a cDNA encoding a human brain potassium channel. Biochem Soc Trans. 1990;18:891-2 pubmed
  82. Coleman S, Newcombe J, Pryke J, Dolly J. Subunit composition of Kv1 channels in human CNS. J Neurochem. 1999;73:849-58 pubmed
    ..2 only, whereas Kvbeta1.1 coprecipitated with all the alpha subunits present. This represents the first description of Kv1 subunit complexes in the human CNS and demonstrates regional variations, indicative of functional specialisation. ..
  83. Zhang Z, Rhodes K, Childers W, Argentieri T, Wang Q. Disinactivation of N-type inactivation of voltage-gated K channels by an erbstatin analogue. J Biol Chem. 2004;279:29226-30 pubmed
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    ..Further multicenter, multiethnic, and large sample size pharmacogenetic and case-control studies are warranted to confirm our negative results. ..
  85. Gonzalez C, López Rodríguez A, Srikumar D, Rosenthal J, Holmgren M. Editing of human K(V)1.1 channel mRNAs disrupts binding of the N-terminus tip at the intracellular cavity. Nat Commun. 2011;2:436 pubmed publisher
    ..By converting I to V, the intimacy of the interaction is reduced, allowing the inactivation gate to unbind with much faster kinetics. ..
  86. Naranjo D, Moldenhauer H, Pincuntureo M, Díaz Franulic I. Pore size matters for potassium channel conductance. J Gen Physiol. 2016;148:277-91 pubmed publisher
    ..Here we raise the idea that the physical dimensions of the hydrophobic internal vestibule limit ion transport in K(+) channels, accounting for their diversity in unitary conductance. ..
  87. Iyer S, Matthews S, Simeone T, Maganti R, Simeone K. Accumulation of rest deficiency precedes sudden death of epileptic Kv1.1 knockout mice, a model of sudden unexpected death in epilepsy. Epilepsia. 2017;: pubmed publisher
  88. Ouadid Ahidouch H, Chaussade F, Roudbaraki M, Slomianny C, Dewailly E, Delcourt P, et al. KV1.1 K(+) channels identification in human breast carcinoma cells: involvement in cell proliferation. Biochem Biophys Res Commun. 2000;278:272-7 pubmed
    ..These data provide the first evidence of Kv1.1 K(+) channels expression in MCF-7 cells and indicate that these channels are implicated in cell proliferation. ..
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    ..We have summarized clinical and molecular genetics findings in neuro-otolology during the last 2 years...
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    Episodic ataxia type 1 (EA1) is a human dominant neurological syndrome characterized by continuous myokymia, episodic attacks of ataxic gait and spastic contractions of skeletal muscles that can be triggered by emotional stress and ..
  91. Albrecht B, Weber K, Pongs O. Characterization of a voltage-activated K-channel gene cluster on human chromosome 12p13. Receptors Channels. 1995;3:213-20 pubmed
    ..Northern blot analyses of the KCNA1, KCNA5 and KCNA6 mRNAs in different human brain areas show that the genes are distinctly expressed. Therefore, they may be transcribed independently of each other. ..