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Dravet Syndrome: A Developmental and Epileptic Encephalopathy.

Lopez-Santiago L, Isom LL.

Epilepsy Curr. 2019 Jan;19(1):51-53. doi: 10.1177/1535759718822038. Epub 2019 Jan 30.


Correction: Ankyrin-G regulates forebrain connectivity and network synchronization via interaction with GABARAP.

Nelson AD, Caballero-Florán RN, Díaz JCR, Hull JM, Yuan Y, Li J, Chen K, Walder KK, Lopez-Santiago LF, Bennett V, McInnis MG, Isom LL, Wang C, Zhang M, Jones KS, Jenkins PM.

Mol Psychiatry. 2019 Jan 31. doi: 10.1038/s41380-019-0361-0. [Epub ahead of print]


Ankyrin-G regulates forebrain connectivity and network synchronization via interaction with GABARAP.

Nelson AD, Caballero-Florán RN, Rodríguez Díaz JC, Hull JM, Yuan Y, Li J, Chen K, Walder KK, Lopez-Santiago LF, Bennett V, McInnis MG, Isom LL, Wang C, Zhang M, Jones KS, Jenkins PM.

Mol Psychiatry. 2018 Nov 30. doi: 10.1038/s41380-018-0308-x. [Epub ahead of print] Erratum in: Mol Psychiatry. 2019 Jan 31;:.


Channelopathy as a SUDEP Biomarker in Dravet Syndrome Patient-Derived Cardiac Myocytes.

Frasier CR, Zhang H, Offord J, Dang LT, Auerbach DS, Shi H, Chen C, Goldman AM, Eckhardt LL, Bezzerides VJ, Parent JM, Isom LL.

Stem Cell Reports. 2018 Sep 11;11(3):626-634. doi: 10.1016/j.stemcr.2018.07.012. Epub 2018 Aug 23.


Mapt deletion fails to rescue premature lethality in two models of sodium channel epilepsy.

Chen C, Holth JK, Bunton-Stasyshyn R, Anumonwo CK, Meisler MH, Noebels JL, Isom LL.

Ann Clin Transl Neurol. 2018 Jul 5;5(8):982-987. doi: 10.1002/acn3.599. eCollection 2018 Aug.


The adhesion function of the sodium channel beta subunit (β1) contributes to cardiac action potential propagation.

Veeraraghavan R, Hoeker GS, Alvarez-Laviada A, Hoagland D, Wan X, King DR, Sanchez-Alonso J, Chen C, Jourdan J, Isom LL, Deschenes I, Smyth JW, Gorelik J, Poelzing S, Gourdie RG.

Elife. 2018 Aug 14;7. pii: e37610. doi: 10.7554/eLife.37610.


Voltage-Gated Sodium Channel β1/β1B Subunits Regulate Cardiac Physiology and Pathophysiology.

Edokobi N, Isom LL.

Front Physiol. 2018 Apr 23;9:351. doi: 10.3389/fphys.2018.00351. eCollection 2018. Review.


Opposing Phenotypes in Dravet Syndrome Patient-Derived Induced Pluripotent Stem Cell Neurons: Can Everyone Be Right?

Isom LL.

Epilepsy Curr. 2017 Jul-Aug;17(4):244-247. doi: 10.5698/1535-7597.17.4.244. No abstract available.


Voltage-Gated Sodium Channel β Subunits and Their Related Diseases.

Bouza AA, Isom LL.

Handb Exp Pharmacol. 2018;246:423-450. doi: 10.1007/164_2017_48. Review.


Voltage-gated sodium channel β subunits: The power outside the pore in brain development and disease.

Hull JM, Isom LL.

Neuropharmacology. 2018 Apr;132:43-57. doi: 10.1016/j.neuropharm.2017.09.018. Epub 2017 Sep 18. Review.


Neuronal hyperexcitability in a mouse model of SCN8A epileptic encephalopathy.

Lopez-Santiago LF, Yuan Y, Wagnon JL, Hull JM, Frasier CR, O'Malley HA, Meisler MH, Isom LL.

Proc Natl Acad Sci U S A. 2017 Feb 28;114(9):2383-2388. doi: 10.1073/pnas.1616821114. Epub 2017 Feb 13.


Scn2b Deletion in Mice Results in Ventricular and Atrial Arrhythmias.

Bao Y, Willis BC, Frasier CR, Lopez-Santiago LF, Lin X, Ramos-Mondragón R, Auerbach DS, Chen C, Wang Z, Anumonwo J, Valdivia HH, Delmar M, Jalife J, Isom LL.

Circ Arrhythm Electrophysiol. 2016 Dec;9(12). pii: e003923.


Expecting the Unexpected: Lack of In Vivo Network Defects in an Scn1a Model of Dravet Syndrome.

Hull JM, Isom LL.

Epilepsy Curr. 2016 Nov-Dec;16(6):408-410. No abstract available.


Cardiac arrhythmia in a mouse model of sodium channel SCN8A epileptic encephalopathy.

Frasier CR, Wagnon JL, Bao YO, McVeigh LG, Lopez-Santiago LF, Meisler MH, Isom LL.

Proc Natl Acad Sci U S A. 2016 Nov 8;113(45):12838-12843. doi: 10.1073/pnas.1612746113. Epub 2016 Oct 26.


Developmental and Regulatory Functions of Na(+) Channel Non-pore-forming β Subunits.

Winters JJ, Isom LL.

Curr Top Membr. 2016;78:315-51. doi: 10.1016/bs.ctm.2016.07.003. Epub 2016 Aug 2. Review.


Cannabinoid-induced depression of synaptic transmission is switched to stimulation when dopaminergic tone is increased in the globus pallidus of the rodent.

Caballero-Florán RN, Conde-Rojas I, Oviedo Chávez A, Cortes-Calleja H, Lopez-Santiago LF, Isom LL, Aceves J, Erlij D, Florán B.

Neuropharmacology. 2016 Nov;110(Pt A):407-418. doi: 10.1016/j.neuropharm.2016.08.002. Epub 2016 Aug 6.


β1-C121W Is Down But Not Out: Epilepsy-Associated Scn1b-C121W Results in a Deleterious Gain-of-Function.

Kruger LC, O'Malley HA, Hull JM, Kleeman A, Patino GA, Isom LL.

J Neurosci. 2016 Jun 8;36(23):6213-24. doi: 10.1523/JNEUROSCI.0405-16.2016.


Voltage-Gated Na+ Channels: Not Just for Conduction.

Kruger LC, Isom LL.

Cold Spring Harb Perspect Biol. 2016 Jun 1;8(6). pii: a029264. doi: 10.1101/cshperspect.a029264. Review.


Role of Sodium Channels in Epilepsy.

Kaplan DI, Isom LL, Petrou S.

Cold Spring Harb Perspect Med. 2016 Jun 1;6(6). pii: a022814. doi: 10.1101/cshperspect.a022814. Review.


Drugging the undruggable: gabapentin, pregabalin and the calcium channel α2δ subunit.

Offord J, Isom LL.

Crit Rev Biochem Mol Biol. 2015 Jul-Aug;51(4):246-56. doi: 10.3109/10409238.2016.1173010. Epub 2016 Apr 25. Review.


Late Na(+) current and protracted electrical recovery are critical determinants of the aging myopathy.

Signore S, Sorrentino A, Borghetti G, Cannata A, Meo M, Zhou Y, Kannappan R, Pasqualini F, O'Malley H, Sundman M, Tsigkas N, Zhang E, Arranto C, Mangiaracina C, Isobe K, Sena BF, Kim J, Goichberg P, Nahrendorf M, Isom LL, Leri A, Anversa P, Rota M.

Nat Commun. 2015 Nov 6;6:8803. doi: 10.1038/ncomms9803.


Sodium channel β subunits: emerging targets in channelopathies.

O'Malley HA, Isom LL.

Annu Rev Physiol. 2015;77:481-504. doi: 10.1146/annurev-physiol-021014-071846. Review.


More than a pore: ion channel signaling complexes.

Lee A, Fakler B, Kaczmarek LK, Isom LL.

J Neurosci. 2014 Nov 12;34(46):15159-69. doi: 10.1523/JNEUROSCI.3275-14.2014. Review.


SUMOylation of Neuronal K⁺ channels: a potential therapeutic pathway for epilepsy and SUDEP?

Yuan Y, Isom LL.

Neuron. 2014 Sep 3;83(5):996-8. doi: 10.1016/j.neuron.2014.08.026.


Untangling the dravet syndrome seizure network: the changing face of a rare genetic epilepsy.

Chopra R, Isom LL.

Epilepsy Curr. 2014 Mar;14(2):86-9. doi: 10.5698/1535-7597-14.2.86.


The role of non-pore-forming β subunits in physiology and pathophysiology of voltage-gated sodium channels.

Calhoun JD, Isom LL.

Handb Exp Pharmacol. 2014;221:51-89. doi: 10.1007/978-3-642-41588-3_4. Review.


Altered cardiac electrophysiology and SUDEP in a model of Dravet syndrome.

Auerbach DS, Jones J, Clawson BC, Offord J, Lenk GM, Ogiwara I, Yamakawa K, Meisler MH, Parent JM, Isom LL.

PLoS One. 2013 Oct 14;8(10):e77843. doi: 10.1371/journal.pone.0077843. eCollection 2013.


Dravet syndrome patient-derived neurons suggest a novel epilepsy mechanism.

Liu Y, Lopez-Santiago LF, Yuan Y, Jones JM, Zhang H, O'Malley HA, Patino GA, O'Brien JE, Rusconi R, Gupta A, Thompson RC, Natowicz MR, Meisler MH, Isom LL, Parent JM.

Ann Neurol. 2013 Jul;74(1):128-39. doi: 10.1002/ana.23897. Epub 2013 Jul 2. Erratum in: Ann Neurol. 2015 Nov;78(5):838.


Abnormal neuronal patterning occurs during early postnatal brain development of Scn1b-null mice and precedes hyperexcitability.

Brackenbury WJ, Yuan Y, O'Malley HA, Parent JM, Isom LL.

Proc Natl Acad Sci U S A. 2013 Jan 15;110(3):1089-94. doi: 10.1073/pnas.1208767110. Epub 2012 Dec 31.


Identification of the cysteine residue responsible for disulfide linkage of Na+ channel α and β2 subunits.

Chen C, Calhoun JD, Zhang Y, Lopez-Santiago L, Zhou N, Davis TH, Salzer JL, Isom LL.

J Biol Chem. 2012 Nov 9;287(46):39061-9. doi: 10.1074/jbc.M112.397646. Epub 2012 Sep 19.


Therapeutic potential for phenytoin: targeting Na(v)1.5 sodium channels to reduce migration and invasion in metastatic breast cancer.

Yang M, Kozminski DJ, Wold LA, Modak R, Calhoun JD, Isom LL, Brackenbury WJ.

Breast Cancer Res Treat. 2012 Jul;134(2):603-15. doi: 10.1007/s10549-012-2102-9. Epub 2012 Jun 8.


The sodium channel accessory subunit Navβ1 regulates neuronal excitability through modulation of repolarizing voltage-gated K⁺ channels.

Marionneau C, Carrasquillo Y, Norris AJ, Townsend RR, Isom LL, Link AJ, Nerbonne JM.

J Neurosci. 2012 Apr 25;32(17):5716-27. doi: 10.1523/JNEUROSCI.6450-11.2012.


Na Channel β Subunits: Overachievers of the Ion Channel Family.

Brackenbury WJ, Isom LL.

Front Pharmacol. 2011 Sep 28;2:53. doi: 10.3389/fphar.2011.00053. eCollection 2011.


Voltage-gated Na+ channel β1B: a secreted cell adhesion molecule involved in human epilepsy.

Patino GA, Brackenbury WJ, Bao Y, Lopez-Santiago LF, O'Malley HA, Chen C, Calhoun JD, Lafrenière RG, Cossette P, Rouleau GA, Isom LL.

J Neurosci. 2011 Oct 12;31(41):14577-91. doi: 10.1523/JNEUROSCI.0361-11.2011.


Subcellular heterogeneity of sodium current properties in adult cardiac ventricular myocytes.

Lin X, Liu N, Lu J, Zhang J, Anumonwo JM, Isom LL, Fishman GI, Delmar M.

Heart Rhythm. 2011 Dec;8(12):1923-30. doi: 10.1016/j.hrthm.2011.07.016. Epub 2011 Jul 20.


Interactions between ankyrin-G, Plakophilin-2, and Connexin43 at the cardiac intercalated disc.

Sato PY, Coombs W, Lin X, Nekrasova O, Green KJ, Isom LL, Taffet SM, Delmar M.

Circ Res. 2011 Jul 8;109(2):193-201. doi: 10.1161/CIRCRESAHA.111.247023. Epub 2011 May 26.


Na+ channel Scn1b gene regulates dorsal root ganglion nociceptor excitability in vivo.

Lopez-Santiago LF, Brackenbury WJ, Chen C, Isom LL.

J Biol Chem. 2011 Jul 1;286(26):22913-23. doi: 10.1074/jbc.M111.242370. Epub 2011 May 9.


PKCbeta co-localizes with the dopamine transporter in mesencephalic neurons.

O'Malley HA, Park Y, Isom LL, Gnegy ME.

Neurosci Lett. 2010 Aug 9;480(1):40-3.


Electrophysiology and beyond: multiple roles of Na+ channel β subunits in development and disease.

Patino GA, Isom LL.

Neurosci Lett. 2010 Dec 10;486(2):53-9. doi: 10.1016/j.neulet.2010.06.050. Epub 2010 Jun 23. Review.


Efficacy loss of the anticonvulsant carbamazepine in mice lacking sodium channel beta subunits via paradoxical effects on persistent sodium currents.

Uebachs M, Opitz T, Royeck M, Dickhof G, Horstmann MT, Isom LL, Beck H.

J Neurosci. 2010 Jun 23;30(25):8489-501. doi: 10.1523/JNEUROSCI.1534-10.2010.


Ordered assembly of the adhesive and electrochemical connections within newly formed intercalated disks in primary cultures of adult rat cardiomyocytes.

Geisler SB, Green KJ, Isom LL, Meshinchi S, Martens JR, Delmar M, Russell MW.

J Biomed Biotechnol. 2010;2010:624719. doi: 10.1155/2010/624719. Epub 2010 May 12.


Functional reciprocity between Na+ channel Nav1.6 and beta1 subunits in the coordinated regulation of excitability and neurite outgrowth.

Brackenbury WJ, Calhoun JD, Chen C, Miyazaki H, Nukina N, Oyama F, Ranscht B, Isom LL.

Proc Natl Acad Sci U S A. 2010 Feb 2;107(5):2283-8. doi: 10.1073/pnas.0909434107. Epub 2010 Jan 19.


Conduction block in PMP22 deficiency.

Bai Y, Zhang X, Katona I, Saporta MA, Shy ME, O'Malley HA, Isom LL, Suter U, Li J.

J Neurosci. 2010 Jan 13;30(2):600-8. doi: 10.1523/JNEUROSCI.4264-09.2010.


A functional null mutation of SCN1B in a patient with Dravet syndrome.

Patino GA, Claes LR, Lopez-Santiago LF, Slat EA, Dondeti RS, Chen C, O'Malley HA, Gray CB, Miyazaki H, Nukina N, Oyama F, De Jonghe P, Isom LL.

J Neurosci. 2009 Aug 26;29(34):10764-78. doi: 10.1523/JNEUROSCI.2475-09.2009.


Loss of plakophilin-2 expression leads to decreased sodium current and slower conduction velocity in cultured cardiac myocytes.

Sato PY, Musa H, Coombs W, Guerrero-Serna G, Patiño GA, Taffet SM, Isom LL, Delmar M.

Circ Res. 2009 Sep 11;105(6):523-6. doi: 10.1161/CIRCRESAHA.109.201418. Epub 2009 Aug 6.


The voltage-gated Na+ channel beta3 subunit does not mediate trans homophilic cell adhesion or associate with the cell adhesion molecule contactin.

McEwen DP, Chen C, Meadows LS, Lopez-Santiago L, Isom LL.

Neurosci Lett. 2009 Oct 25;462(3):272-5. doi: 10.1016/j.neulet.2009.07.020. Epub 2009 Jul 17.


Regulation of persistent Na current by interactions between beta subunits of voltage-gated Na channels.

Aman TK, Grieco-Calub TM, Chen C, Rusconi R, Slat EA, Isom LL, Raman IM.

J Neurosci. 2009 Feb 18;29(7):2027-42. doi: 10.1523/JNEUROSCI.4531-08.2009.


A novel adhesion molecule in human breast cancer cells: voltage-gated Na+ channel beta1 subunit.

Chioni AM, Brackenbury WJ, Calhoun JD, Isom LL, Djamgoz MB.

Int J Biochem Cell Biol. 2009 May;41(5):1216-27. doi: 10.1016/j.biocel.2008.11.001. Epub 2008 Nov 12.


scn1bb, a zebrafish ortholog of SCN1B expressed in excitable and nonexcitable cells, affects motor neuron axon morphology and touch sensitivity.

Fein AJ, Wright MA, Slat EA, Ribera AB, Isom LL.

J Neurosci. 2008 Nov 19;28(47):12510-22. doi: 10.1523/JNEUROSCI.4329-08.2008.

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