KCNMA1 CHANNELOPATHY

Dinsdale RL and Meredith AL (2024). Evaluation of four KCNMA1 channelopathy variants on BK channel current under Cav1.2 activation.  Channels 18(1):2396346.

Meredith AL (2024). BK Channelopathies and KCNMA1-Linked Disease Models. Annual Reviews in Physiology 86(1): 277-300.

Moldenhauer HJ, Tammen K, and Meredith AL (2024). Structural mapping of patient-associated KCNMA1 gene variants. Biophysical Journal 123 (14): P1984-2000

Park SM, Roache CE, Iffland PH, Moldenhauer HJ, Matychak KK, Plante AE, Lieberman AG, Crino PB, and Meredith AL (2022).  BK channel properties correlate with neurobehavioral severity in three KCNMA1-linked channelopathy mouse models. eLife 2022;11:e77953.

• eLife Digest: ‘The root of the problem‘
• SOM Press Release

Moldenhauer HJ, Dinsdale, RL, Alvarez S, Fernández-Jaén A, and Meredith AL (2022).  Effect of an autism-associated KCNMB2 variant, G124R, on BK channel properties. Current Research in Physiology 5: 404-413.

Keros S, Heim J, Hakami W, Zohar-Dayan E, Ben-Zeev B, Grinspan Z, Kruer MC, Meredith AL (2021). Lisdexamfetamine therapy in paroxysmal non-kinesigenic dyskinesia associated with the KCNMA1-N999S variant. Movement Disorders Clinical Practice 9(2): 229–235.

Miller, J, Moldenhauer, HJ, Keros, S, Meredith, AL (2021).  An Emerging Spectrum of Variants and Clinical Features in KCNMA1-Linked Channelopathy. Channels 15(1):447‑464.

Buckley C, Williams J, Munteanu T, King M, Park SM, Meredith AL, Lynch T (2020). Dystonicus, Oculogyric Crisis and Paroxysmal Dyskinesia in a 25 Year-Old Woman with a Novel KCNMA1 Variant, K457E. Tremor & Other Hyperkinetic Movements 10(1): 49, pp. 1‑6.

Heim J, Vemuri A, Lewis S, Guida B, Troester M, Keros S, Meredith A, Kruer MC (2020) Cataplexy in patients harboring the KCNMA1 p.N999S mutation.  Movement Disorders Clinical Practice 7 (7): 861–862.

Bailey, CS, Moldenhauer, HJ, Park, SM, Keros, S, and Meredith, AL (2019).  KCNMA1-Linked Channelopathy. Journal of General Physiology 151 (10): 1173

Moldenhauer, HJ, Park, SM, and Meredith, AL (2020). Characterization of new human KCNMA1 Loss-of-Function Mutations. Biophysical Journal 114A.

Moldenhauer, HJ, Matychak, K, and Meredith, AL (2020). Comparative Gain-of-Function Effects of KCNMA1-N999S Mutation on Human BK Channel Properties. Journal of Neurophysiology 123:560.          

• JNP Podcast April 20, 2020

Plante, A, Lai, MH, Lu, J, and Meredith, AL (2019).  Effects of single nucleotide polymorphisms in human KCNMA1 on BK current properties.  Frontiers in Molecular Neuroscience. 12:285.

Imlach, WL, Finch, SC, Dunlop, J, Meredith, AL, Aldrich, RW, and Dalziel, JE (2008). The molecular mechanism of ‘ryegrass staggers,’ a neurological disorder of potassium channels. J Pharmacol Exp Ther. 327:657-664.

CIRCADIAN RHYTHM

Dinsdale R, Roache C, and Meredith AL (2023). Disease-associated KCNMA1 variants decrease circadian clock robustness in channelopathy mouse models. Journal of General Physiology; 155(11):e202313357

McNally BA, Plante AE, and Meredith AL (2021).  Contributions of Ca_V1.3 channels to Ca²⁺ current and Ca²⁺-activated BK current in the suprachiasmatic nucleus.  Frontiers in Physiology (Special Topic:  Regulatory Mechanisms of Ca²⁺-activated Ion Channels and Their Impacts on Physiological/Pathophysiological Functions).

Plante, AE, Rao, VP, Rizzo, MA, and Meredith, AL (2021).  Comparative Ca²⁺ channel contributions to intracellular Ca²⁺ levels in the circadian clock. Biophysical Reports 1(1): 100005

Plante, AE, Whitt, JP, and Meredith, AL (2021). BK channel activation by L-type Ca²⁺ channels Ca_V1.2 and Ca_V1.3 during the subthreshold phase of an action potential.  J Neurophysiology 126(2):427-439

Harvey, JRM, Plante, A, and Meredith, AL (2020).  Ion Channels Controlling Circadian Rhythms in Suprachiasmatic Nucleus Excitability.  Physiological Reviews 100(4):1415‑1454

McNally, BA, Plante, AE, and Meredith, AL (2019). Diurnal properties of voltage-gated Ca²⁺ currents in SCN and roles in action potential firing.  Journal of Physiology, 598(9):1775-1790.

Whitt, JP, McNally BA, and Meredith, AL. (2017).  Differential contribution of Ca²⁺ sources to day and night BK current activation in the circadian clock.  Journal of General Physiology 150(2):259.  

• See Research News Highlight, ‘Up all night: BK channels’ circadian dance with different calcium sources.  JGP 150(2):175.

Whitt, JP, Montgomery, JM, and Meredith, AL. (2016).  BK channel inactivation gates daytime excitability in the circadian clock.  Nature Communications 7:10837.

SOM Press Release

Shelley, C, Whitt, JP, Montgomery, JM, and Meredith, AL. (2013)  Phosphorylation of a constitutive serine inhibits BK channel variants containing the alternate exon ‘SRKR’.  Journal of General Physiology 142 (6):585-598.   

• JGP Commentary: Multilevel regulation: Controlling BK channels in central clock neurons

Hermanstyne, T.O., Meredith, A.L., Mong, J.A., Misonou, H.  Kv2.2: A novel molecular target to study the role of basal forebrain GABAergic neurons in the sleep-wake cycle.  Sleep 36(12):1839-1848.

Montgomery, JM, Whitt, JP, Wright, BN, Lai, ML, and Meredith, AL (2013).  Mis-expression of the BK K⁺ channel disrupts suprachiasmatic nucleus circuit rhythmicity and alters clock-controlled behavior. AJP- Cell Physiology 304(4):C299-C311.

Montgomery, JM, and Meredith, AL (2012).  Genetic activation of BK currents in vivo generates bi-directional effects on neuronal excitability.  PNAS 109(46):18997-9002.

Herrera, GM and Meredith, AL (2010).  Diurnal variation in urodynamics of rat.  PLoS One 5(8): e12298.

Kent, J and Meredith, AL (2008).  BK channels regulate spontaneous action potential rhythmicity in the suprachiasmatic nucleus. PLoS One 3(12):e3884.

Meredith AL, Wiler SW, Miller BH, Takahashi JS, Fodor AA, Ruby NF, and Aldrich RW (2006). BK calcium-activated potassium channels regulate circadian behavioral rhythms and pacemaker output. Nature Neurosci 9(8):1041-1049.

OTHER TRANSGENICS, CARDIAC, AND SMOOTH MUSCLE EXCITABILITY

Lai, MH, Wu, Y, Zhan, Z, Anderson, ME, Dalziel, JE, and Meredith AL (2014).  BK channels regulate sinoatrial node firing rate and cardiac pacing in vivo.  AJP- Heart 307(9):H1327-38.

Zemen, BG, Lai, MH, Whitt, JP, Khan, Z, Zhao, G, and Meredith, AL (2015).  Generation of Kcnma1^fl-tdTomato, a conditional deletion of the BK Channel alpha subunit in mouse.  Physiological Reports 3(11): e12612.

White, RS, Zemen, BG, Khan, Z, Montgomery, JR, Herrera, GM, and Meredith, AL (2015).  Evaluation of mouse urinary bladder smooth muscle for diurnal differences in contractile properties.  Frontiers in Pharmacology 5 (293): 1-8.

Imlach WL, Finch SC, Miller JH, Meredith AL, Dalziel JE (2010).  A role for BK channels in heart rate regulation in rodents. PLoS One 5(1): e8698.

Meredith AL, Thorneloe KS, Werner ME, Nelson MT, and Aldrich RW (2004). Overactive bladder and incontinence in the absence of the BK Ca²⁺-activated K⁺ channel.  Journal of Biological Chemistry 279:36746-36752.

BOOK CHAPTERS

Textbook of Ion Channels (Taylor & Francis, 2022)
Genetic Models and Transgenics (Volume 1, Chapter 15)
Alternative Splicing (Volume 3, Chapter 1)

Handbook of Ion Channels (Taylor & Francis, CRC Press, February, 2015)
Genetic Methods for Studying Ion Channel Function in Physiology and Disease. (Chapter 13)
Alternative Splicing. (Chapter 37)

COLLABORATIVE STUDIES

Zhang, J,  Guan , X, Li , Q, Meredith , AL, Pan, HL, & Yan, J (2018). Glutamate-activated K⁺ signaling complexes formed by BK channels and NMDA receptors.  PNAS 115(38):E9006-E9014.

Nelson, A.B., Faulstich, M., Moghadam, S., Onori, K., Meredith, A, and du Lac, S (2017). BK channels are required for multisensory plasticity in the oculomotor system.  Neuron 93(1):211-220. 

Hayashi, Y, Morinaga, S, Zhang, J, Satoh, Y, Meredith, AL, Nakata, T, Wu, Z, Kohsaka, S, Inoue, K, and Nakanishi, H (2016). BK channels in microglia are required for morphine-induced hyperalgesia.  Nature Communications 7: 11697.

Li B, Jie W, Huang L, Wei P, Li S, Luo Z, Friedman AK, Meredith AL, Han MH, Zhu XH, Gao TM (2014).  Nuclear BK channels regulate gene expression via the control of nuclear calcium signaling.  Nature Neurosci 17(8):1055-63.

Singh, H, Lu, R, Bopassa, JC, Meredith, AL, Stefani, E, and Toro, L (2013). Cardiac mitoBK_Ca K⁺ Channel is Encoded by Kcnma1 Gene and a Splicing Sequence Defines its Mitochondrial Location.  PNAS 110(26):10836-41.

Wahyu, ID, Kamasawa, N, Matsui, K, Meredith, AL, Watanabe, M, and Shigemoto, R (2013).  Quantitative localization of Cav2.1 (P/Q-type) voltage-dependent calcium channels in Purkinje cells: somatodendritic gradient and distinct somatic co-clustering with calcium-activated potassium channels. J Neuroscience 33(8):3668-3678.

Maison, SF, Pyott, SJ, Meredith, AL, and Liberman, MC (2013). Olivocochlear suppression of outer hair cells in vivo: evidence for combined action of BK and SK2 channels throughout the cochlea.  J Neurophysiology 109(6):1525-1534.

Girouard H, Bonev AD, Hannah, RM, Meredith AL, Aldrich RW and Nelson MT (2010). Astrocytic endfoot Ca²⁺ and BK channels determine both arteriolar dilation and constriction.  PNAS 107(8):3811-6.

Petkov, GV, Brown, SM, Bentcheva-Petkov, LM, Meredith, AL, Aldrich, RW, and Nelson, MT (2008). The large conductance Ca²⁺-activated K⁺ (BK) channel mediates the smooth muscle relaxation in response to b-adrenergic receptor stimulation in the urinary bladder:  Evidence from BK channel a-subunit knockout mice.  AJP- Renal Physiol. 295(4):F1149-57.

Werner ME, Meredith AL, Aldrich RW, and Nelson MT. (2008) Hyper-contractility and impaired sildenafil relaxations in the BK_Ca channel deletion model of erectile dysfunction. AJP- Renal Physiol. 295:F1149-57.

Pyott SJ, Meredith AL, Fodor AA, Yamoah EN and Aldrich RW. (2007). Normal cochlear function in mice lacking the BK channel α, β1 or β4 subunits.  Journal Biological Chemistry 282:3312-3324.

Werner, ME, Zvara, P, Meredith, AL, Aldrich RW, and Nelson, MT (2007). Frequency encoding of cholinergic- and purinergic-mediated signaling to mouse urinary bladder smooth muscle: Modulation by BK channels.  Am J Physiol Regul Integr Comp Physiol. 292: R616-624.

Filosa JA, Bonev AD, Straub SV, Meredith AL, Wilkerson MK, Aldrich RW and Nelson MT. (2006). Local potassium signaling couples neuron al activity to vasodilation in the brain. Nature Neurosci. 9(11):1397-1403.

Misonou H, Menegola M, Buchwalder L, Park EW, Meredith A, Rhodes KJ, Aldrich RW, and Trimmer JS. (2006). Localization of the BK Ca²⁺-activated K⁺ channel Slo1 in axons and nerve terminals in mammalian brain and cultured neurons. J Comp Neurol. 496:289‑302.

Werner, ME, Zvara, P, Meredith, AL, Aldrich RW, and Nelson, MT (2005). Erectile dysfunction in mice lacking the large conductance calcium-activated potassium (BK) channel.  J Physiol. 567(2):545-556.

Boluyt, MO, Robinson, KG, Meredith, AL, Sen, S, Lakatta, EF, Crow, MT, Brooks, WW, Conrad, CH, and Bing OH (2005). Heart failure after long-term supravalvular aortic constriction in rats.  Am J Hypertension 18(2):202-212.

Thorneloe, KS, Meredith, AL, Knorn, AM, Aldrich, RW and Nelson, MT (2005). Urodynamic properties and neurotransmitter dependence of urinary bladder contractility in the BK channel deletion model of overactive bladder.  Am J Physiol Renal Physiol. 289(3):F604-10.

Meredith A, and Johnson JE (2000). Negative autoregulation of MASH1 expression in CNS development.  Developmental Biology 222:336-346.

Horton S, Meredith A, Richardson JA, and Johnson JE. (1999). Correct coordination of neuronal differentiation events in ventral forebrain requires the bHLH factor MASH1. Molecular and Cellular Neuroscience 14:355-369.

Younes A, Boluyt MO, O’Neill LO, Meredith A, Crow MT, and Lakatta EG. (1995). Age-associated increase in rat ventricular ANP gene expression correlates with cardiac hypertrophy. American J of Physiology 269:H1003-H1008.

Boluyt MO, O’Neill LO, Bing OHL, Meredith A, Crow MT, and Lakatta EG. (1994). Alterations in cardiac gene expression during the transition from stable hypertrophy to heart failure. Circulation Research 75(1): 23-32.