Journal Articles — Edward C. Cooper, M.D., Ph.D.

Dr. Edward C. Cooper has authored and co-authored 66 journal articles. They are listed here in reverse chronological order and grouped by published year. Click on the Expand plus symbol or Collapse minus symbol symbol to expand/collapse the list of journal publications by year, respectively.

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Journal Articles Published in 2024 (n = 2)

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Abreo TJ, Thompson EC, Madabushi A, Soh H, Varghese N, Vanoye CG, et al. Plural molecular and cellular mechanisms of pore domain KCNQ2 encephalopathy. bioRxiv. 2024;:. PMID: 38260608.


Bayat A, Iavarone S, Miceli F, Jakobsen AV, Johannesen KM, Nikanorova M, et al. Phenotypic and functional assessment of two novel KCNQ2 gain-of-function variants Y141N and G239S and effects of amitriptyline treatment. Neurotherapeutics. 2024;21(1):e00296. PMID: 38241158.


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Journal Articles Published in 2023 (n = 1)

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Muller P, Takacs DS, Hedrich UBS, Coorg R, Masters L, Glinton KE, et al. KCNA1 gain-of-function epileptic encephalopathy treated with 4-aminopyridine. Ann Clin Transl Neurol. 2023;10(4):656-63. PMID: 36793218.


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Journal Articles Published in 2022 (n = 6)

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Boets S, Johannesen KM, Destree A, Manti F, Ramantani G, Lesca G, et al. Adult phenotype of KCNQ2 encephalopathy. J Med Genet. 2022;59(6):528-35. PMID: 33811133.


Cooper EC, Abreo T, Tran B. KCNQ channel PIP2 modulation: Two loose links, three rings, and a twist. Neuron. 2022;110(2):178-80. PMID: 35051360.


Jing J, Dunbar C, Sonesra A, Chavez A, Park S, Yang R, et al. Removal of KCNQ2 from parvalbumin-expressing interneurons improves anti-seizure efficacy of retigabine. Exp Neurol. 2022;355:114141. PMID: 35691372.


Mazzaferro S, Msekela DJ, Cooper EC, Maheshwari A, Sine SM. Genetic variant in nicotinic receptor alpha4-subunit causes sleep-related hyperkinetic epilepsy via increased channel opening. Int J Mol Sci. 2022;23(20):. PMID: 36292983.


Miceli F, Millevert C, Soldovieri MV, Mosca I, Ambrosino P, Carotenuto L, et al. KCNQ2 R144 variants cause neurodevelopmental disability with language impairment and autistic features without neonatal seizures through a gain-of-function mechanism. EBioMedicine. 2022;81:104130. PMID: 35780567.


Vanoye CG, Desai RR, Ji Z, Adusumilli S, Jairam N, Ghabra N, et al. High-throughput evaluation of epilepsy-associated KCNQ2 variants reveals functional and pharmacological heterogeneity. JCI Insight. 2022;7(5):. PMID: 35104249.


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Journal Articles Published in <2021 (n = 57)

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Miceli F, Carotenuto L, Barrese V, Soldovieri MV, Heinzen EL, Mandel AM, et al. A Novel Kv7.3 Variant in the Voltage-Sensing S4 Segment in a Family With Benign Neonatal Epilepsy: Functional Characterization and in vitro Rescue by beta-Hydroxybutyrate. Front Physiol. 2020;11:1040. PMID: 33013448.


Tran B, Ji ZG, Xu M, Tsuchida TN, Cooper EC. Two KCNQ2 encephalopathy variants in the calmodulin-binding helix A exhibit dominant-negative effects and altered PIP2 interaction. Front Physiol. 2020;11:1144. PMID: 33041849.


Goto A, Ishii A, Shibata M, Ihara Y, Cooper EC, Hirose S. Characteristics of KCNQ2 variants causing either benign neonatal epilepsy or developmental and epileptic encephalopathy. Epilepsia. 2019;60(9):1870-80. PMID: 31418850.


Sands TT, Miceli F, Lesca G, Beck AE, Sadleir LG, Arrington DK, et al. Autism and developmental disability caused by KCNQ3 gain-of-function variants. Ann Neurol. 2019;86(2):181-92. PMID: 31177578.


Soldovieri MV, Ambrosino P, Mosca I, Miceli F, Franco C, Canzoniero LMT, et al. Epileptic encephalopathy in a patient with a novel variant in the Kv7.2 S2 transmembrane segment: Clinical, genetic, and functional features. Int J Mol Sci. 2019;20(14):. PMID: 31295832.


Lopez AY, Wang X, Xu M, Maheshwari A, Curry D, Lam S, et al. Ankyrin-G isoform imbalance and interneuronopathy link epilepsy and bipolar disorder. Mol Psychiatry. 2017;22(10):1464-72. PMID: 27956739.


Millichap JJ, Miceli F, De Maria M, Keator C, Joshi N, Tran B, et al. Infantile spasms and encephalopathy without preceding neonatal seizures caused by KCNQ2 R198Q, a gain-of-function variant. Epilepsia. 2017;58(1):e10-e15. PMID: 27861786.


Mulkey SB, Ben-Zeev B, Nicolai J, Carroll JL, Gronborg S, Jiang YH, et al. Neonatal nonepileptic myoclonus is a prominent clinical feature of KCNQ2 gain-of-function variants R201C and R201H. Epilepsia. 2017;58(3):436-45. PMID: 28139826.


Millichap JJ, Park KL, Tsuchida T, Ben-Zeev B, Carmant L, Flamini R, et al. KCNQ2 encephalopathy: Features, mutational hot spots, and ezogabine treatment of 11 patients. Neurol Genet. 2016;2(5):e96. PMID: 27602407.


Hirst CS, Foong JP, Stamp LA, Fegan E, Dent S, Cooper EC, et al. Ion channel expression in the developing enteric nervous system. PLoS One. 2015;10(3):e0123436. PMID: 25798587.


Martinello K, Huang Z, Lujan R, Tran B, Watanabe M, Cooper EC, et al. Cholinergic afferent stimulation induces axonal function plasticity in adult hippocampal granule cells. Neuron. 2015;85(2):346-63. PMID: 25578363.


Xu M, Cooper EC. An Ankyrin-G N-terminal Gate and Protein Kinase CK2 Dually Regulate Binding of Voltage-gated Sodium and KCNQ2/3 Potassium Channels. J Biol Chem. 2015;290(27):16619-32. PMID: 25998125.


Battefeld A, Tran BT, Gavrilis J, Cooper EC, Kole MH. Heteromeric Kv7.2/7.3 channels differentially regulate action potential initiation and conduction in neocortical myelinated axons. J Neurosci. 2014;34(10):3719-32. PMID: 24599470.


Chang KJ, Zollinger DR, Susuki K, Sherman DL, Makara MA, Brophy PJ, et al. Glial ankyrins facilitate paranodal axoglial junction assembly. Nat Neurosci. 2014;17(12):1673-81. PMID: 25362471.


Ho TS, Zollinger DR, Chang KJ, Xu M, Cooper EC, Stankewich MC, et al. A hierarchy of ankyrin-spectrin complexes clusters sodium channels at nodes of Ranvier. Nat Neurosci. 2014;17(12):1664-72. PMID: 25362473.


Kang S, Xu M, Cooper EC, Hoshi N. Channel-anchored protein kinase CK2 and protein phosphatase 1 reciprocally regulate KCNQ2-containing M-channels via phosphorylation of calmodulin. J Biol Chem. 2014;289(16):11536-44. PMID: 24627475.


Kole MH, Cooper EC. Axonal Kv7.2/7.3 channels: Caught in the act. Channels (Austin). 2014;8(4):288-9. PMID: 25171180.


Millichap JJ, Cooper EC. KCNQ2 potassium channel epileptic encephalopathy syndrome: Divorce of an electro-mechanical couple? Epilepsy Curr. 2012;12(4):150-2. PMID: 22936888.


Cooper EC. Made for "anchorin": Kv7.2/7.3 (KCNQ2/KCNQ3) channels and the modulation of neuronal excitability in vertebrate axons. Semin Cell Dev Biol. 2011;22(2):185-92. PMID: 20940059.


Cooper EC. The cadherin superfamily and epileptogenesis: End of the beginning? Epilepsy Curr. 2009;9(3):87-9. PMID: 19471581.


Jin Z, Liang GH, Cooper EC, Jarlebark L. Expression and localization of K channels KCNQ2 and KCNQ3 in the mammalian cochlea. Audiol Neurootol. 2009;14(2):98-105. PMID: 18827480.


Raol YH, Lapides DA, Keating JG, Brooks-Kayal AR, Cooper EC. A KCNQ channel opener for experimental neonatal seizures and status epilepticus. Ann Neurol. 2009;65(3):326-36. PMID: 19334075.


Cooper EC. Funny team member makes key plays, but leaves the dendritic field when hit hard. Epilepsy Curr. 2008;8(4):103-5. PMID: 18596878.


Hill AS, Nishino A, Nakajo K, Zhang G, Fineman JR, Selzer ME, et al. Ion channel clustering at the axon initial segment and node of Ranvier evolved sequentially in early chordates. PLoS Genet. 2008;4(12):e1000317. PMID: 19112491.


Shah MM, Migliore M, Valencia I, Cooper EC, Brown DA. Functional significance of axonal Kv7 channels in hippocampal pyramidal neurons. Proc Natl Acad Sci U S A. 2008;105(22):7869-74. PMID: 18515424.


Cooper EC, Pan Z. Putting an end to DEND: a severe neonatal-onset epilepsy is treatable if recognized early. Neurology. 2007;69(13):1310-1. PMID: 17893291.


Cooper EC. (What to do) when epilepsy gene mutations stop making sense. Epilepsy Curr. 2007;7(1):23-5. PMID: 17304347.


Kuramoto N, Wilkins ME, Fairfax BP, Revilla-Sanchez R, Terunuma M, Tamaki K, et al. Phospho-dependent functional modulation of GABA(B) receptors by the metabolic sensor AMP-dependent protein kinase. Neuron. 2007;53(2):233-47. PMID: 17224405.


Cooper EC. Exploiting the other inhibitory ion: KCNQ potassium channels and regulation of excitability in developing and mature brain. Epilepsy Curr. 2006;6(4):133-5. PMID: 17260036.


Pan Z, Kao T, Horvath Z, Lemos J, Sul JY, Cranstoun SD, et al. A common ankyrin-G-based mechanism retains KCNQ and NaV channels at electrically active domains of the axon. J Neurosci. 2006;26(10):2599-613. PMID: 16525039.


Schwarz JR, Glassmeier G, Cooper EC, Kao TC, Nodera H, Tabuena D, et al. KCNQ channels mediate IKs, a slow K+ current regulating excitability in the rat node of Ranvier. J Physiol. 2006;573(Pt 1):17-34. PMID: 16527853.


Surti TS, Huang L, Jan YN, Jan LY, Cooper EC. Identification by mass spectrometry and functional characterization of two phosphorylation sites of KCNQ2/KCNQ3 channels. Proc Natl Acad Sci U S A. 2005;102(49):17828-33. PMID: 16319223.


Cooper EC, Baraban SC. Pain without gain (of function): Sodium channel dysfunction in epilepsy. Epilepsy Curr. 2004;4(4):158-9. PMID: 16059485.


Devaux JJ, Kleopa KA, Cooper EC, Scherer SS. KCNQ2 is a nodal K+ channel. J Neurosci. 2004;24(5):1236-44. PMID: 14762142.


Cooper EC, Jan LY. M-channels: Neurological diseases, neuromodulation, and drug development. Arch Neurol. 2003;60(4):496-500. PMID: 12707061.


Tsao JW, Cooper EC. Reflex-sensitive spinal segmental myoclonus associated with vitamin B12 deficiency. Neurology. 2003;61(6):867-8. PMID: 14504348.


Castro PA, Cooper EC, Lowenstein DH, Baraban SC. Hippocampal heterotopia lack functional Kv4.2 potassium channels in the methylazoxymethanol model of cortical malformations and epilepsy. J Neurosci. 2001;21(17):6626-34. PMID: 11517252.


Cooper EC, Harrington E, Jan YN, Jan LY. M channel KCNQ2 subunits are localized to key sites for control of neuronal network oscillations and synchronization in mouse brain. J Neurosci. 2001;21(24):9529-40. PMID: 11739564.


Cooper EC. Potassium channels: How genetic studies of epileptic syndromes open paths to new therapeutic targets and drugs. Epilepsia. 2001;42 Suppl 5:49-54. PMID: 11887968.


Cooper EC, Aldape KD, Abosch A, Barbaro NM, Berger MS, Peacock WS, et al. Colocalization and coassembly of two human brain M-type potassium channel subunits that are mutated in epilepsy. Proc Natl Acad Sci U S A. 2000;97(9):4914-9. PMID: 10781098.


Cooper EC, Jan LY. Ion channel genes and human neurological disease: Recent progress, prospects, and challenges. Proc Natl Acad Sci U S A. 1999;96(9):4759-66. PMID: 10220366.


Gleeson JG, Minnerath SR, Fox JW, Allen KM, Luo RF, Hong SE, et al. Characterization of mutations in the gene doublecortin in patients with double cortex syndrome. Ann Neurol. 1999;45(2):146-53. PMID: 9989615.


Brenman JE, Topinka JR, Cooper EC, McGee AW, Rosen J, Milroy T, et al. Localization of postsynaptic density-93 to dendritic microtubules and interaction with microtubule-associated protein 1A. J Neurosci. 1998;18(21):8805-13. PMID: 9786987.


Cooper EC, Milroy A, Jan YN, Jan LY, Lowenstein DH. Presynaptic localization of Kv1.4-containing A-type potassium channels near excitatory synapses in the hippocampus. J Neurosci. 1998;18(3):965-74. PMID: 9437018.


Gleeson JG, Allen KM, Fox JW, Lamperti ED, Berkovic S, Scheffer I, et al. Doublecortin, a brain-specific gene mutated in human X-linked lissencephaly and double cortex syndrome, encodes a putative signaling protein. Cell. 1998;92(1):63-72. PMID: 9489700.


Agnew WS, Cooper EC, Shenkel S, Correa AM, James WM, Ukomadu C, et al. Voltage-sensitive sodium channels: agents that perturb inactivation gating. Ann N Y Acad Sci. 1991;625:200-23. PMID: 1647724.


Cooper EC, Agnew WS. Reconstituted voltage-sensitive sodium channels from eel electroplax: activation of permeability by quaternary lidocaine, N-bromoacetamide, and N-bromosuccinimide. J Membr Biol. 1989;111(3):253-64. PMID: 2557451.


Shenkel S, Cooper EC, James W, Agnew WS, Sigworth FJ. Purified, modified eel sodium channels are active in planar bilayers in the absence of activating neurotoxins. Proc Natl Acad Sci U S A. 1989;86(23):9592-6. PMID: 2556720.


Cooper EC, Tomiko SA, Agnew WS. Reconstituted voltage-sensitive sodium channel from electrophorus electricus: Chemical modifications that alter regulation of ion permeability. Proc Natl Acad Sci U S A. 1987;84(17):6282-6. PMID: 2442755.


Agnew WS, Tomiko SA, Rosenberg RL, Emerick MC, Cooper EC. The structure and function of the voltage-sensitive Na channel. Ann N Y Acad Sci. 1986;479:238-56. PMID: 2433994.


Greenberg DA, Carpenter CL, Cooper EC. Stimulation of calcium uptake in PC12 cells by the dihydropyridine agonist BAY K 8644. J Neurochem. 1985;45(3):990-3. PMID: 2411863.


Greenberg DA, Cooper EC, Carpenter CL. Reversible dihydropyridine isothiocyanate binding to brain calcium channels. J Neurochem. 1985;44(1):319-21. PMID: 2578060.


Greenberg DA, Cooper EC, Carpenter CL. Calcium channel 'agonist' BAY K 8644 inhibits calcium antagonist binding to brain and PC12 cell membranes. Brain Res. 1984;305(2):365-8. PMID: 6204725.


Greenberg DA, Cooper EC, Carpenter CL. Calcium entry activators: distinct sites of dihydropyridine and aminopyridine action. Neurosci Lett. 1984;50(1-3):279-82. PMID: 6208517.


Greenberg DA, Cooper EC, Carpenter CL. Phenytoin interacts with calcium channels in brain membranes. Ann Neurol. 1984;16(5):616-7. PMID: 6095733.


Greenberg DA, Cooper EC, Gordon A, Diamond I. Ethanol and the gamma-aminobutyric acid-benzodiazepine receptor complex. J Neurochem. 1984;42(4):1062-8. PMID: 6321657.


Greenberg DA, Cooper EC. Effect of ethanol on [3H]nitrendipine binding to calcium channels in brain membranes. Alcohol Clin Exp Res. 1984;8(6):568-71. PMID: 6097136.

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