The University of Queensland

Erythromelalgia caused by the missense mutation p.Arg220Pro in an alternatively spliced exon of SCN9A (NaV1.7)

Deuis, Jennifer R., Kumble, Smitha, Keramidas, Angelo, Ragnarsson, Lotten, Simons, Cas, Pais, Lynn, White, Susan M and Vetter, Irina (2024). Erythromelalgia caused by the missense mutation p.Arg220Pro in an alternatively spliced exon of SCN9A (NaV1.7). Human Molecular Genetics, 33 (2), 103-109. doi: 10.1093/hmg/ddad152

GRIN1 variants associated with neurodevelopmental disorders reveal channel gating pathomechanisms

Ragnarsson, Lotten, Zhang, Zihan, Das, Sooraj S., Tran, Poanna, Andersson, Åsa, Portes, Vincent Des, Demestre, Cecilia Altuzzara, Remerand, Ganaelle, Labalme, Audrey, Chatron, Nicolas, Sanlaville, Damien, Lesca, Gaetan, Anggono, Victor, Vetter, Irina and Keramidas, Angelo (2023). GRIN1 variants associated with neurodevelopmental disorders reveal channel gating pathomechanisms. Epilepsia, 64 (12), 3377-3388. doi: 10.1111/epi.17776

Correlations of receptor desensitization of gain-of-function <i>GABRB3</i> variants with clinical severity

Lin, Susan X N, Ahring, Philip K, Keramidas, Angelo, Liao, Vivian W Y, Møller, Rikke S, Chebib, Mary and Absalom, Nathan L (2023). Correlations of receptor desensitization of gain-of-function GABRB3 variants with clinical severity. Brain, 147 (1), 224-239. doi: 10.1093/brain/awad285

Ant venoms contain vertebrate-selective pain-causing sodium channel toxins

Robinson, Samuel D., Deuis, Jennifer R., Touchard, Axel, Keramidas, Angelo, Mueller, Alexander, Schroeder, Christina I., Barassé, Valentine, Walker, Andrew A., Brinkwirth, Nina, Jami, Sina, Bonnafé, Elsa, Treilhou, Michel, Undheim, Eivind A. B., Schmidt, Justin O., King, Glenn F. and Vetter, Irina (2023). Ant venoms contain vertebrate-selective pain-causing sodium channel toxins. Nature Communications, 14 (1) 2977, 1-11. doi: 10.1038/s41467-023-38839-1

Anti-seizure mechanisms of midazolam and valproate at the β2(L51M) variant of the GABAA receptor

Kuanyshbek, Alibek, Wang, Meng, Andersson, Åsa, Tuifua, Marie, Palmer, Elizabeth E., Sachdev, Rani K., Mu, Ting-Wei, Vetter, Irina and Keramidas, Angelo (2022). Anti-seizure mechanisms of midazolam and valproate at the β2(L51M) variant of the GABAA receptor. Neuropharmacology, 221 109295, 109295. doi: 10.1016/j.neuropharm.2022.109295

Pharmacological activation of ATF6 remodels the proteostasis network to rescue pathogenic GABAA receptors

Wang, Meng, Cotter, Edmund, Wang, Ya-Juan, Fu, Xu, Whittsette, Angela L., Lynch, Joseph W., Wiseman, R. Luke, Kelly, Jeffery W., Keramidas, Angelo and Mu, Ting-Wei (2022). Pharmacological activation of ATF6 remodels the proteostasis network to rescue pathogenic GABAA receptors. Cell and Bioscience, 12 (1) 48, 1-20. doi: 10.1186/s13578-022-00783-w

Shisa7-dependent regulation of GABAA receptor single-channel gating kinetics

Castellano, David, Wu, Kunwei, Keramidas, Angelo and Lu, Wei (2022). Shisa7-dependent regulation of GABAA receptor single-channel gating kinetics. Journal of Neuroscience, 42 (47), 8758-8766. doi: 10.1523/JNEUROSCI.0510-22.2022

A pain-causing and paralytic ant venom glycopeptide

Robinson, Samuel D., Kambanis, Lucas, Clayton, Daniel, Hinneburg, Hannes, Corcilius, Leo, Mueller, Alexander, Walker, Andrew A., Keramidas, Angelo, Kulkarni, Sameer S., Jones, Alun, Vetter, Irina, Thaysen-Andersen, Morten, Payne, Richard J., King, Glenn F. and Undheim, Eivind A.B. (2021). A pain-causing and paralytic ant venom glycopeptide. iScience, 24 (10) 103175, 1-21. doi: 10.1016/j.isci.2021.103175

Regulation of NMDA receptor trafficking and gating by activity-dependent CaMKIIα phosphorylation of the GluN2A subunit

Yong, Xuan Ling Hilary, Zhang, Lingrui, Yang, Liming, Chen, Xiumin, Tan, Jing Zhi Anson, Yu, Xiaojun, Chandra, Mintu, Livingstone, Emma, Widagdo, Jocelyn, Vieira, Marta M., Roche, Katherine W., Lynch, Joseph W., Keramidas, Angelo, Collins, Brett M. and Anggono, Victor (2021). Regulation of NMDA receptor trafficking and gating by activity-dependent CaMKIIα phosphorylation of the GluN2A subunit. Cell Reports, 36 (1) 109338, 1-21. doi: 10.1016/j.celrep.2021.109338

Proteostasis regulators restore function of epilepsy-associated GABAA receptors

Di, Xiao-Jing, Wang, Ya-Juan, Cotter, Edmund, Wang, Meng, Whittsette, Angela L., Han, Dong-Yun, Sangwung, Panjamaporn, Brown, Renae, Lynch, Joseph W., Keramidas, Angelo and Mu, Ting-Wei (2020). Proteostasis regulators restore function of epilepsy-associated GABAA receptors. Cell Chemical Biology, 27 (1), 1-e7. doi: 10.1016/j.chembiol.2020.08.012

Effects of GluN2A and GluN2B gain-of-function epilepsy mutations on synaptic currents mediated by diheteromeric and triheteromeric NMDA receptors

Chen, Xiumin, Keramidas, Angelo, Harvey, Robert J. and Lynch, Joseph W. (2020). Effects of GluN2A and GluN2B gain-of-function epilepsy mutations on synaptic currents mediated by diheteromeric and triheteromeric NMDA receptors. Neurobiology of Disease, 140 104850, 104850. doi: 10.1016/j.nbd.2020.104850

A novel glycine receptor variant with startle disease affects syndapin I and glycinergic inhibition

Langlhofer, Georg, Schaefer, Natascha, Maric, Hans M., Keramidas, Angelo, Zhang, Yan, Baumann, Peter, Blum, Robert, Breitinger, Ulrike, Strømgaard, Kristian, Schlosser, Andreas, Kessels, Michael M., Koch, Dennis, Qualmann, Britta, Breitinger, Hans-Georg, Lynch, Joseph W. and Villmann, Carmen (2020). A novel glycine receptor variant with startle disease affects syndapin I and glycinergic inhibition. Journal of Neuroscience, 40 (25), JN-RM. doi: 10.1523/jneurosci.2490-19.2020

The effects of insecticides on two splice variants of the glutamate-gated chloride channel receptor of the major malaria vector, Anopheles gambiae

Atif, Mohammed, Lynch, Joseph W. and Keramidas, Angelo (2019). The effects of insecticides on two splice variants of the glutamate-gated chloride channel receptor of the major malaria vector, Anopheles gambiae. British Journal of Pharmacology, 177 (1) bph.14855, 175-187. doi: 10.1111/bph.14855

GluClR-mediated inhibitory postsynaptic currents reveal targets for ivermectin and potential mechanisms of ivermectin resistance

Atif, Mohammed, Smith, Jennifer J., Estrada-Mondragon, Argel, Xiao, Xue, Salim, Angela A., Capon, Robert J., Lynch, Joseph W. and Keramidas, Angelo (2019). GluClR-mediated inhibitory postsynaptic currents reveal targets for ivermectin and potential mechanisms of ivermectin resistance. PLOS Pathogens, 15 (1) e1007570, e1007570. doi: 10.1371/journal.ppat.1007570

SAHA (Vorinostat) corrects inhibitory synaptic deficits caused by missense epilepsy mutations to the GABA(A) receptor gamma 2 subunit

Durisic, Nela, Keramidas, Angelo, Dixon, Christine L. and Lynch, Joseph W. (2018). SAHA (Vorinostat) corrects inhibitory synaptic deficits caused by missense epilepsy mutations to the GABA(A) receptor gamma 2 subunit. Frontiers in Molecular Neuroscience, 11 89, 89. doi: 10.3389/fnmol.2018.00089

Structure/Function Studies of the alpha 4 Subunit Reveal Evolutionary Loss of a GlyR Subtype Involved in Startle and Escape Responses

Leacock, Sophie, Syed, Parnayan, James, Victoria M., Bode, Anna, Kawakami, Koichi, Keramidas, Angelo, Suster, Maximiliano, Lynch, Joseph W. and Harvey, Robert J. (2018). Structure/Function Studies of the alpha 4 Subunit Reveal Evolutionary Loss of a GlyR Subtype Involved in Startle and Escape Responses. Frontiers in Molecular Neuroscience, 11 23, 23. doi: 10.3389/fnmol.2018.00023

Inhibitory synapse deficits caused by familial α1 GABAA receptor mutations in epilepsy

Chen, Xiumin, Durisic, Nela, Lynch, Joseph W. and Keramidas, Angelo (2017). Inhibitory synapse deficits caused by familial α1 GABAA receptor mutations in epilepsy. Neurobiology of Disease, 108, 213-224. doi: 10.1016/j.nbd.2017.08.020

Effects of glutamate and ivermectin on single glutamate-gated chloride channels of the parasitic nematode H. contortus

Atif, Mohammed, Estrada-Mondragon, Argel, Nguyen, Bindi, Lynch, Joseph W. and Keramidas, Angelo (2017). Effects of glutamate and ivermectin on single glutamate-gated chloride channels of the parasitic nematode H. contortus. PLoS Pathogens, 13 (10) e1006663, e1006663. doi: 10.1371/journal.ppat.1006663

Physiological and pharmacological properties of inhibitory postsynaptic currents mediated by α5β1γ2, α5β2γ2 and α5β3γ2 GABAA receptors

Chen, Xiumin, Keramidas, Angelo and Lynch, Joseph W. (2017). Physiological and pharmacological properties of inhibitory postsynaptic currents mediated by α5β1γ2, α5β2γ2 and α5β3γ2 GABAA receptors. Neuropharmacology, 125, 243-253. doi: 10.1016/j.neuropharm.2017.07.027

Gamma 1-containing GABA-A receptors cluster at synapses where they mediate slower synaptic currents than gamma 2-containing GABA-A receptors

Dixon, Christine L., Sah, Pankaj, Keramidas, Angelo, Lynch, Joseph W. and Durisic, Nela (2017). Gamma 1-containing GABA-A receptors cluster at synapses where they mediate slower synaptic currents than gamma 2-containing GABA-A receptors. Frontiers in Molecular Neuroscience, 10 178, 178. doi: 10.3389/fnmol.2017.00178

Structure-function analysis of the GlyR alpha 2 subunit autism mutation p.R323L reveals a gain-of-function

Zhang, Yan, Ho, Thi Nhu Thao, Harvey, Robert J., Lynch, Joseph W. and Keramidas, Angelo (2017). Structure-function analysis of the GlyR alpha 2 subunit autism mutation p.R323L reveals a gain-of-function. Frontiers in Molecular Neuroscience, 10 158, 158. doi: 10.3389/fnmol.2017.00158

Probing the structural mechanism of partial agonism in glycine receptors using the fluorescent artificial amino acid, ANAP

Soh, Ming S., Estrada-Mondragon, Argel, Durisic, Nela, Keramidas, Angelo and Lynch, Joseph W. (2017). Probing the structural mechanism of partial agonism in glycine receptors using the fluorescent artificial amino acid, ANAP. ACS Chemical Biology, 12 (3), 805-813. doi: 10.1021/acschembio.6b00926

Potent neuroprotection after stroke afforded by a double-knot spider-venom peptide that inhibits acid-sensing ion channel 1a

Chassagnon, Irene R., McCarthy, Claudia A., Chin, Yanni K.-Y., Pineda, Sandy S., Keramidas, Angelo, Mobli, Mehdi, Pham, Vi, De Silva, T. Michael, Lynch, Joseph W., Widdop, Robert E., Rash, Lachlan D. and King, Glenn F. (2017). Potent neuroprotection after stroke afforded by a double-knot spider-venom peptide that inhibits acid-sensing ion channel 1a. Proceedings from the National Academy of Sciences of the United States of America, 114 (14), 3750-3755. doi: 10.1073/pnas.1614728114

Multiple sodium channel isoforms mediate the pathological effects of Pacific ciguatoxin-1

Inserra, Marco C., Isreal, Mathilde, Caldwell, Ashlee, Castro, Joel, Deuis, Jennifer R., Harrington, Andrea M., Keramidas, Angelo, Garcia-Caraballo, Sonia, Maddern, Jessica, Erickson, Andelain, Grundy, Luke, Rychkov, Grigori Y., Zimmerman, Katharina, Lewis, Richard J., Brierley, Stuart M. and Vetter, Irina (2017). Multiple sodium channel isoforms mediate the pathological effects of Pacific ciguatoxin-1. Scientific Reports, 7 (1) 42810, 42810. doi: 10.1038/srep42810

Ivermectin-activated, cation-permeable glycine receptors for the chemogenetic control of neuronal excitation

Islam, Robiul, Keramidas, Angelo, Xu, Li, Durisic, Nela, Sah, Pankaj and Lynch, Joseph W. (2016). Ivermectin-activated, cation-permeable glycine receptors for the chemogenetic control of neuronal excitation. ACS Chemical Neuroscience, 7 (12), 1647-1657. doi: 10.1021/acschemneuro.6b00168

The free zinc concentration in the synaptic cleft of artificial glycinergic synapses rises to at least 1 µm

Zhang, Yan, Keramidas, Angelo and Lynch, Joseph W. (2016). The free zinc concentration in the synaptic cleft of artificial glycinergic synapses rises to at least 1 µm. Frontiers in Molecular Neuroscience, 9 (SEP2016) 88, 88. doi: 10.3389/fnmol.2016.00088

Investigating the mechanism by which gain-of-function mutations to the a1 glycine receptor cause hyperekplexia

Zhang, Yan, Bode, Anna, Nguyen, Bindi, Angelo Keramidas and Lynch, Joseph W. (2016). Investigating the mechanism by which gain-of-function mutations to the a1 glycine receptor cause hyperekplexia. Journal of Biological Chemistry, 291 (29), 15332-15341. doi: 10.1074/jbc.M116.728592

Correlating structural and energetic changes in glycine receptor activation

Scott, Suzanne, Lynch, Joseph W and Keramidas, Angelo (2015). Correlating structural and energetic changes in glycine receptor activation. Journal of Biological Chemistry, 290 (9), 5621-5634. doi: 10.1074/jbc.M114.616573

Functional reconstitution of glycinergic synapses incorporating defined glycine receptor subunit combinations

Zhang, Yan, Dixon, Christine L., Keramidas, Angelo and Lynch, Joseph W. (2015). Functional reconstitution of glycinergic synapses incorporating defined glycine receptor subunit combinations. Neuropharmacology, 89, 391-397. doi: 10.1016/j.neuropharm.2014.10.026

Zolpidem and eszopiclone prime α1β2γ2 GABAA receptors for longer duration of activity

Dixon, Christine L., Harrison, Neil L., Lynch, Joseph W. and Keramidas, Angelo (2015). Zolpidem and eszopiclone prime α1β2γ2 GABAA receptors for longer duration of activity. British Journal of Pharmacology, 172 (14), 3522-3536. doi: 10.1111/bph.13142

GABAa receptor α and γ subunits shape synaptic currents via different mechanisms

Dixon, Christine, Sah, Pankaj, Lynch, Joseph W. and Keramidas, Angelo (2014). GABAa receptor α and γ subunits shape synaptic currents via different mechanisms. Journal of Biological Chemistry, 289 (9), 5399-5411. doi: 10.1074/jbc.M113.514695

New hyperekplexia mutations provide insight into glycine receptor assembly, trafficking, and activation mechanisms

Bode, Anna, Wood, Sian-Elin, Mullins, Jonathan G. L., Keramidas, Angelo, Cushion, Thomas D., Thomas, Rhys H., Pickrell, William O., Drew, Cheney J. G., Masri, Amira, Jones, Elizabeth A., Vassallo, Grace, Born, Alfred P., Alehan, Fusun, Aharoni, Sharon, Bannasch, Gerald, Bartsch, Marius, Kara, Bulent, Krause, Amanda, Karam, Elie G., Matta, Stephanie, Jain, Vivek, Mandel, Hanna, Freilinger, Michael, Graham, Gail E., Hobson, Emma, Chatfield, Sue, Vincent-Delorme, Catherine, Rahme, Jubran E., Afawi, Zaid ... Lynch, Joseph W. (2013). New hyperekplexia mutations provide insight into glycine receptor assembly, trafficking, and activation mechanisms. Journal of Biological Chemistry, 288 (47), 33745-33759. doi: 10.1074/jbc.M113.509240

Novel missense mutations in the glycine receptor β subunit gene (GLRB) in startle disease

James, Victoria M., Bode, Anna, Chung, Seo-Kyung, Gill, Jennifer L., Nielsen, Maartje, Cowan, Frances M., Vujic, Mihailo, Thomas, Rhys H., Rees, Mark I., Harvey, Kirsten, Keramidas, Angelo, Topf, Maya, Ginjaar, Ieke, Lynch, Joseph W. and Harvey, Robert J. (2013). Novel missense mutations in the glycine receptor β subunit gene (GLRB) in startle disease. Neurobiology of Disease, 52, 137-149. doi: 10.1016/j.nbd.2012.12.001

An outline of desensitization in pentameric ligand-gated ion channel receptors

Keramidas, Angelo and Lynch, Joseph W. (2012). An outline of desensitization in pentameric ligand-gated ion channel receptors. Cellular and Molecular Life Sciences, 70 (7), 1241-1253. doi: 10.1007/s00018-012-1133-z

The activation mechanism of alpha(1)beta(2)gamma(2S) and alpha(3)beta(3)gamma(2S) GABA(A) receptors

Keramidas, Angelo and Harrison, Neil L. (2010). The activation mechanism of alpha(1)beta(2)gamma(2S) and alpha(3)beta(3)gamma(2S) GABA(A) receptors. Journal of General Physiology, 135 (1), 59-75. doi: 10.1085/jgp.200910317

Taurine is a potent activator of extrasynaptic GABAA receptors in the thalamus

Jia, Fan, Yue, Minerva, Chandra, Dev, Keramidas, Angelo, Goldstein, Peter A., Homanics, Gregg E. and Harrison, Neil L. (2008). Taurine is a potent activator of extrasynaptic GABAA receptors in the thalamus. Journal of Neuroscience, 28 (1), 106-115. doi: 10.1523/JNEUROSCI.3996-07.2008

The pre-M1 segment of the alpha 1 subunit is a transduction element in the activation of the GABA(A) receptor

Keramidas, Angelo, Kash, Thomas L. and Harrison, Neil L. (2006). The pre-M1 segment of the alpha 1 subunit is a transduction element in the activation of the GABA(A) receptor. The Journal of Physiology, 575 (1), 11-22. doi: 10.1113/jphysiol.2005.102756

Identification of molluscan nicotinic acetylcholine receptor (nAChR) subunits involved in formation of cation- and anion-selective nAChRs

van Nierop, Pim, Keramidas, Angelo, Bertrand, Sonia, van Minnen, Jan, Gouwenberg, Yvonne, Bertrand, Daniel and Smit, August B. (2005). Identification of molluscan nicotinic acetylcholine receptor (nAChR) subunits involved in formation of cation- and anion-selective nAChRs. Journal of Neuroscience, 25 (46), 10617-10626. doi: 10.1523/JNEUROSCI.2015-05.2005

Ligand-gated ion channels: Mechanisms underlying ion selectivity

Keramidas, Angelo, Moorhouse, Andrew J., Schofield, Peter R. and Barry, Peter H. (2004). Ligand-gated ion channels: Mechanisms underlying ion selectivity. Progress in Biophysics and Molecular Biology, 86 (2), 161-204. doi: 10.1016/j.pbiomolbio.2003.09.002

The contribution of proline 250 (P-2’) to pore diameter and ion selectivity in the human glycine receptor channel

Lee, David J.-S., Keramidas, Angelo, Moorhouse, Andrew J., Schofield, Peter R. and Barry, Peter H. (2003). The contribution of proline 250 (P-2’) to pore diameter and ion selectivity in the human glycine receptor channel. Neuroscience Letters, 351 (3), 196-200. doi: 10.1016/S0304-3940(03)00977-7

The contribution of proline 250 (P-2 ') to pore diameter and ion selectivity in the human glycine receptor channel

Lee, DJS, Keramidas, A, Moorhouse, AJ, Schofield, PR and Barry, PH (2003). The contribution of proline 250 (P-2 ') to pore diameter and ion selectivity in the human glycine receptor channel. Neuroscience Letters, 351 (3), 196-200. doi: 10.1016/S0304-3940(03)00977-7

Cation-selective mutations in the M2 domain of the inhibitory glycine receptor channel reveal determinants of ion-charge selectivity

Keramidas, Angelo, Moorhouse, Andrew J., Pierce, Kerrie D. and Schofield, Peter R. (2002). Cation-selective mutations in the M2 domain of the inhibitory glycine receptor channel reveal determinants of ion-charge selectivity. Journal of General Physiology, 119 (5), 393-410. doi: 10.1085/jgp.20028552

Single channel analysis of conductance and rectification in cation-selective, mutant glycine receptor channels

Moorhouse, Andrew J., Keramidas, Angelo, Zaykin, Andrey, Schofield, Peter R. and Barry, Peter H. (2002). Single channel analysis of conductance and rectification in cation-selective, mutant glycine receptor channels. Journal of General Physiology, 119 (5), 411-425. doi: 10.1085/jgp.20028553

M2 pore mutations convert the glycine receptor channel from being anion- to cation-selective

Keramidas, A, Moorhouse, AJ, French, CR, Schofield, PR and Barry, PH (2000). M2 pore mutations convert the glycine receptor channel from being anion- to cation-selective. Biophysical Journal, 79 (1), 247-259. doi: 10.1016/S0006-3495(00)76287-4

Measurement of the limiting equivalent conductivities and mobilities of the most prevalent ionic species of EGTA (EGTA2- and EGTA3-) for use in electrophysiological experiments

Keramidas, Angelo , Barry, Peter H., Moorhouse, Andrew and Kuhlmann, Levin (1999). Measurement of the limiting equivalent conductivities and mobilities of the most prevalent ionic species of EGTA (EGTA2- and EGTA3-) for use in electrophysiological experiments. Journal of Neuroscience Methods, 89 (1), 41-47. doi: 10.1016/S0165-0270(99)00036-9

Agonist-dependent single channel current and Gating in alpha(4)beta(2)delta and alpha(1)beta(2)gamma(2S) GABA(A) receptors

Keramidas, Angelo and Harrison, Neil L. (2008). Agonist-dependent single channel current and Gating in alpha(4)beta(2)delta and alpha(1)beta(2)gamma(2S) GABA(A) receptors. New York, United States: Rockefeller University Press. doi: 10.1085/jgp.200709871

Mechanism of ion permeation in the glycine receptor and its cation-selective mutations

O'Mara, Megan, Keramidas, Angelo, Barry, Peter H. and Chung, Shin-Ho (2003). Mechanism of ion permeation in the glycine receptor and its cation-selective mutations. 47th Annual Meeting of the Biophysical Society, San Antonio, Texas, 1-5 March 2003. Bethesda, MD, U.S.A.: Cell Press for the Biophysical Society.

Mechanism of ion permeation in the glycine receptor and its cation-selective mutations

O'Mara, M., Keramidas, A., Barry, P. H. and Chung, S. H. (2003). Mechanism of ion permeation in the glycine receptor and its cation-selective mutations. 47th Annual Meeting of the Biophysical-Society, San Antonio Texas, 1-5 March 2003. Bethesda, MD United States: Biophysical Society.