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Stephen Caldwell Cannon, M.D., Ph.D.
Molecular, Cellular & Integrative Physiology GPB Home Area
Work Phone Number: 310-825-4463
Work Address:543-263B CHS
The primary research interests of our laboratory are how ion channels regulate the electrical excitability of cells and how defects in these channels lead to human disease. In the past two decades, mutations of ion channel genes have been found to be the primary cause for over 100 human diseases. Our research program is focused on the mechanistic basis for a group of inherited conditions that alter the electrical excitability of skeletal muscle, including periodic paralysis and myotonia. We have characterized the gating defects of mutant channels, generated computational models of muscle excitability, and produced genetically-engineered mice to gain insights on the pathomechanisms of these disorders and to explore therapeutic interventions.
Cannon Stephen C Channelopathies of skeletal muscle excitability Comprehensive Physiology, 2015; 5(2): 761-90.
Mi Wentao, Rybalchenko Volodymyr, Cannon Stephen C Disrupted coupling of gating charge displacement to Na+ current activation for DIIS4 mutations in hypokalemic periodic paralysis The Journal of general physiology, 2014; 144(2): 137-45.
Trivedi Jaya R, Cannon Stephen C, Griggs Robert C Nondystrophic myotonia: challenges and future directions Experimental neurology, 2014; 253(2): 28-30.
Wu Fenfen, Mi Wentao, Cannon Stephen C Beneficial effects of bumetanide in a CaV1.1-R528H mouse model of hypokalaemic periodic paralysis Brain : a journal of neurology, 2013; 136(Pt 12): 3766-74.
Nelson Benjamin R, Wu Fenfen, Liu Yun, Anderson Douglas M, McAnally John, Lin Weichun, Cannon Stephen C, Bassel-Duby Rhonda, Olson Eric N Skeletal muscle-specific T-tubule protein STAC3 mediates voltage-induced Ca2+ release and contractility Proceedings of the National Academy of Sciences of the United States of America, 2013; 110(29): 11881-6.
Wu Fenfen, Mi Wentao, Cannon Stephen C Bumetanide prevents transient decreases in muscle force in murine hypokalemic periodic paralysis Neurology, 2013; 80(12): 1110-6.
Wu Fenfen, Mi Wentao, HernÃ¡ndez-Ochoa Erick O, Burns Dennis K, Fu Yu, Gray Hillery F, Struyk Arie F, Schneider Martin F, Cannon Stephen C A calcium channel mutant mouse model of hypokalemic periodic paralysis The Journal of clinical investigation, 2012; 122(12): 4580-91.
Cannon Stephen C Small fiber neuropathy: a bit less idiopathic? Neurology, 2012; 78(21): 1626-7.
Wu Fenfen, Mi Wentao, Burns Dennis K, Fu Yu, Gray Hillery F, Struyk Arie F, Cannon Stephen C A sodium channel knockin mutant (NaV1.4-R669H) mouse model of hypokalemic periodic paralysis The Journal of clinical investigation, 2011; 121(10): 4082-94.
Kudryashova Elena, Struyk Arie, Mokhonova Ekaterina, Cannon Stephen C, Spencer Melissa J The common missense mutation D489N in TRIM32 causing limb girdle muscular dystrophy 2H leads to loss of the mutated protein in knock-in mice resulting in a Trim32-null phenotype Human molecular genetics, 2011; 20(20): 3925-32.
Cheng Chih-Jen, Lin Shih-Hua, Lo Yi-Fen, Yang Sung-Sen, Hsu Yu-Juei, Cannon Stephen C, Huang Chou-Long Identification and functional characterization of Kir2.6 mutations associated with non-familial hypokalemic periodic paralysis The Journal of biological chemistry, 2011; 286(31): 27425-35.
Francis David G, Rybalchenko Volodymyr, Struyk Arie, Cannon Stephen C Leaky sodium channels from voltage sensor mutations in periodic paralysis, but not paramyotonia Neurology, 2011; 76(19): 1635-41.
Cannon Stephen C Voltage-sensor mutations in channelopathies of skeletal muscle The Journal of physiology, 2010; 588(Pt 11): 1887-95.
Cannon Stephen C, Bean Bruce P Sodium channels gone wild: resurgent current from neuronal and muscle channelopathies The Journal of clinical investigation, 2010; 120(1): 80-3.
Matthews E, Fialho D, Tan S V, Venance S L, Cannon S C, Sternberg D, Fontaine B, Amato A A, Barohn R J, Griggs R C, Hanna M G, Hanna M G The non-dystrophic myotonias: molecular pathogenesis, diagnosis and treatment Brain : a journal of neurology, 2010; 133(Pt 1): 9-22.
Cannon Stephen C Getting a charge out of periodic paralysis? Neurology, 2009; 72(18): 1540-1.
Webb Jadon, Wu Fen-fen, Cannon Stephen C Slow inactivation of the NaV1.4 sodium channel in mammalian cells is impeded by co-expression of the beta1 subunit PflÃ¼gers Archiv : European journal of physiology, 2009; 457(6): 1253-63.
Struyk Arie F, Markin Vladislav S, Francis David, Cannon Stephen C Gating pore currents in DIIS4 mutations of NaV1.4 associated with periodic paralysis: saturation of ion flux and implications for disease pathogenesis The Journal of general physiology, 2008; 132(4): 447-64.
Hayward Lawrence J, Kim Joanna S, Lee Ming-Yang, Zhou Hongru, Kim Ji W, Misra Kumudini, Salajegheh Mohammad, Wu Fen-fen, Matsuda Chie, Reid Valerie, Cros Didier, Hoffman Eric P, Renaud Jean-Marc, Cannon Stephen C, Brown Robert H Targeted mutation of mouse skeletal muscle sodium channel produces myotonia and potassium-sensitive weakness The Journal of clinical investigation, 2008; 118(4): 1437-49.
Webb Jadon, Cannon Stephen C Cold-induced defects of sodium channel gating in atypical periodic paralysis plus myotonia Neurology, 2008; 70(10): 755-61.
Struyk Arie F, Cannon Stephen C Paradoxical depolarization of BA2+- treated muscle exposed to low extracellular K+: insights into resting potential abnormalities in hypokalemic paralysis Muscle & nerve, 2008; 37(3): 326-37.
Struyk Arie F, Cannon Stephen C A Na+ channel mutation linked to hypokalemic periodic paralysis exposes a proton-selective gating pore The Journal of general physiology, 2007; 130(1): 11-20.
Cannon Stephen C Physiologic principles underlying ion channelopathies Neurotherapeutics : the journal of the American Society for Experimental NeuroTherapeutics, 2007; 4(2): 174-83.
Cannon Stephen C Pathomechanisms in channelopathies of skeletal muscle and brain Annual review of neuroscience, 2006; 29(2): 387-415.
Venance S L, Cannon S C, Fialho D, Fontaine B, Hanna M G, Ptacek L J, Tristani-Firouzi M, Tawil R, Griggs R C, Griggs R C The primary periodic paralyses: diagnosis, pathogenesis and treatment Brain : a journal of neurology, 2006; 129(Pt 1): 8-17.
Wu Fen-fen, Gordon Erynn, Hoffman Eric P, Cannon Stephen C A C-terminal skeletal muscle sodium channel mutation associated with myotonia disrupts fast inactivation The Journal of physiology, 2005; 565(Pt 2): 371-80.
Berg Jim, Jiang Hong, Thornton Charles A, Cannon Stephen C Truncated ClC-1 mRNA in myotonic dystrophy exerts a dominant-negative effect on the Cl current Neurology, 2004; 63(12): 2371-5.
Cannon Stephen C Paying the price at the pump: dystonia from mutations in a Na+/K+ -ATPase Neuron, 2004; 43(2): 153-4.
Tsujino Akira, Maertens Chantal, Ohno Kinji, Shen Xin-Ming, Fukuda Taku, Harper C Michael, Cannon Stephen C, Engel Andrew G Myasthenic syndrome caused by mutation of the SCN4A sodium channel Proceedings of the National Academy of Sciences of the United States of America, 2003; 100(12): 7377-82.
Struyk Arie F, Cannon Stephen C Slow inactivation does not block the aqueous accessibility to the outer pore of voltage-gated Na channels The Journal of general physiology, 2002; 120(4): 509-16.
Cannon Stephen C An expanding view for the molecular basis of familial periodic paralysis Neuromuscular disorders : NMD, 2002; 12(6): 533-43.
Mankodi Ami, Takahashi Masanori P, Jiang Hong, Beck Carol L, Bowers William J, Moxley Richard T, Cannon Stephen C, Thornton Charles A Expanded CUG repeats trigger aberrant splicing of ClC-1 chloride channel pre-mRNA and hyperexcitability of skeletal muscle in myotonic dystrophy Molecular cell, 2002; 10(1): 35-44.
Cannon Stephen C Sodium channel gating: no margin for error Neuron, 2002; 34(6): 853-4.
Takahashi M P, Cannon S C Mexiletine block of disease-associated mutations in S6 segments of the human skeletal muscle Na(+) channel The Journal of physiology, 2001; 537(Pt 3): 701-14.
Wu F F, Takahashi M P, Pegoraro E, Angelini C, Colleselli P, Cannon S C, Hoffman E P A new mutation in a family with cold-aggravated myotonia disrupts Na(+) channel inactivation Neurology, 2001; 56(7): 878-84.
Struyk A F, Scoggan K A, Bulman D E, Cannon S C The human skeletal muscle Na channel mutation R669H associated with hypokalemic periodic paralysis enhances slow inactivation The Journal of neuroscience : the official journal of the Society for Neuroscience, 2000; 20(23): 8610-7.
Ranganathan R, Cannon S C, Horvitz H R MOD-1 is a serotonin-gated chloride channel that modulates locomotory behaviour in C. elegans Nature, 2000; 408(6811): 470-5.
Morrill J A, Cannon S C COOH-terminal truncated alpha(1S) subunits conduct current better than full-length dihydropyridine receptors The Journal of general physiology, 2000; 116(3): 341-8.
Ruff R L, Cannon S C Defective slow inactivation of sodium channels contributes to familial periodic paralysis Neurology, 2000; 54(11): 2190-2.
Vedantham V, Cannon S C Rapid and slow voltage-dependent conformational changes in segment IVS6 of voltage-gated Na(+) channels Biophysical journal, 2000; 78(6): 2943-58.
Cannon S C Spectrum of sodium channel disturbances in the nondystrophic myotonias and periodic paralyses Kidney international, 2000; 57(3): 772-9.
Morrill J A, Cannon S C Effects of mutations causing hypokalaemic periodic paralysis on the skeletal muscle L-type Ca2+ channel expressed in Xenopus laevis oocytes The Journal of physiology, 1999; 520 Pt 2(3): 321-36.
Hayward L J, Sandoval G M, Cannon S C Defective slow inactivation of sodium channels contributes to familial periodic paralysis Neurology, 1999; 52(7): 1447-53.
Takahashi M P, Cannon S C Enhanced slow inactivation by V445M: a sodium channel mutation associated with myotonia Biophysical journal, 1999; 76(2): 861-8.
Vedantham V, Cannon S C The position of the fast-inactivation gate during lidocaine block of voltage-gated Na+ channels The Journal of general physiology, 1999; 113(1): 7-16.
Morrill J A, Brown R H, Cannon S C Gating of the L-type Ca channel in human skeletal myotubes: an activation defect caused by the hypokalemic periodic paralysis mutation R528H The Journal of neuroscience : the official journal of the Society for Neuroscience, 1998; 18(24): 10320-34.
Green D S, George A L, Cannon S C Human sodium channel gating defects caused by missense mutations in S6 segments associated with myotonia: S804F and V1293I The Journal of physiology, 1998; 510 ( Pt 3)(24): 685-94.
Vedantham V, Cannon S C Slow inactivation does not affect movement of the fast inactivation gate in voltage-gated Na+ channels The Journal of general physiology, 1998; 111(1): 83-93.
Green D S, Hayward L J, George A L, Cannon S C A proposed mutation, Val781Ile, associated with hyperkalemic periodic paralysis and cardiac dysrhythmia is a benign polymorphism Annals of neurology, 1997; 42(2): 253-6.
Cannon S C From mutation to myotonia in sodium channel disorders Neuromuscular disorders : NMD, 1997; 7(4): 241-9.
Hayward L J, Brown R H, Cannon S C Slow inactivation differs among mutant Na channels associated with myotonia and periodic paralysis Biophysical journal, 1997; 72(3): 1204-19.
Grosson C L, Cannon S C, Corey D P, Gusella J F Sequence of the voltage-gated sodium channel beta1-subunit in wild-type and in quivering mice Brain research. Molecular brain research, 1996; 42(2): 222-6.
Cannon S C Slow inactivation of sodium channels: more than just a laboratory curiosity Biophysical journal, 1996; 71(1): 5-7.
Hayward L J, Brown R H, Cannon S C Inactivation defects caused by myotonia-associated mutations in the sodium channel III-IV linker The Journal of general physiology, 1996; 107(5): 559-76.
Cannon S C Sodium channel defects in myotonia and periodic paralysis Annual review of neuroscience, 1996; 19(5): 141-64.
Cannon S C Ion-channel defects and aberrant excitability in myotonia and periodic paralysis Trends in neurosciences, 1996; 19(1): 3-10.
Chen C, Cannon S C Modulation of Na+ channel inactivation by the beta 1 subunit: a deletion analysis PflÃ¼gers Archiv : European journal of physiology, 1995; 431(2): 186-95.
Cannon S C, Hayward L J, Beech J, Brown R H Sodium channel inactivation is impaired in equine hyperkalemic periodic paralysis Journal of neurophysiology, 1995; 73(5): 1892-9.
Cannon S C, Corey D P Loss of Na+ channel inactivation by anemone toxin (ATX II) mimics the myotonic state in hyperkalaemic periodic paralysis The Journal of physiology, 1993; 466(1): 501-20.
Cannon S C, Brown R H, Corey D P Theoretical reconstruction of myotonia and paralysis caused by incomplete inactivation of sodium channels Biophysical journal, 1993; 65(1): 270-88.
Strittmatter S M, Cannon S C, Ross E M, Higashijima T, Fishman M C GAP-43 augments G protein-coupled receptor transduction in Xenopus laevis oocytes Proceedings of the National Academy of Sciences of the United States of America, 1993; 90(11): 5327-31.
McClatchey A I, Cannon S C, Slaugenhaupt S A, Gusella J F The cloning and expression of a sodium channel beta 1-subunit cDNA from human brain Human molecular genetics, 1993; 2(6): 745-9.
Cannon S C, McClatchey A I, Gusella J F Modification of the Na+ current conducted by the rat skeletal muscle alpha subunit by coexpression with a human brain beta subunit PflÃ¼gers Archiv : European journal of physiology, 1993; 423(1-2): 155-7.
Cannon S C, Strittmatter S M Functional expression of sodium channel mutations identified in families with periodic paralysis Neuron, 1993; 10(2): 317-26.
Cannon S C, Heacock D M Incorporating peer review in performance appraisal Nursing management, 1991; 22(5): 88, 90.
Cannon S C, Brown R H, Corey D P A sodium channel defect in hyperkalemic periodic paralysis: potassium-induced failure of inactivation Neuron, 1991; 6(4): 619-26.
Cannon S C, Miller J M, Crowther J, Moscow D Effect of electrical stimulation on middle latency response in the guinea pig American journal of otolaryngology, 1991; 11(4): 251-5.
Jyung R W, Miller J M, Cannon S C Evaluation of eighth nerve integrity by the electrically evoked middle latency response Otolaryngology--head and neck surgery : official journal of American Academy of Otolaryngology-Head and Neck Surgery, 1989; 101(6): 670-82.
Crowther J, Cannon S C, Miller J M, Jyung R W, Kileny P Anesthesia effects on the electrically evoked middle latency response in guinea pigs Otolaryngology--head and neck surgery : official journal of American Academy of Otolaryngology-Head and Neck Surgery, 1989; 101(1): 51-5.
Kartush J M, Cannon S C, Bojrab D I, Graham M D, Schmaltz S, Kemink J L Use of bacitracin for neurotologic surgery The Laryngoscope, 1988; 98(10): 1050-4.
Cannon S C, Niparko J K Pathologic quiz case 1. Lingual osteoma Archives of otolaryngology--head & neck surgery, 1988; 114(1): 92-4.
Cannon S C, Robinson D A Loss of the neural integrator of the oculomotor system from brain stem lesions in monkey Journal of neurophysiology, 1987; 57(5): 1383-409.
Cannon S C, Leigh R J, Zee D S, Abel L A The effect of the rotational magnification of corrective spectacles on the quantitative evaluation of the VOR Acta oto-laryngologica, 1987; 100(1-2): 81-8.
Cannon S C, Robinson D A An improved neural-network model for the neural integrator of the oculomotor system: more realistic neuron behavior Biological cybernetics, 1985; 53(2): 93-108.
Cannon S C, Robinson D A, Shamma S A proposed neural network for the integrator of the oculomotor system Biological cybernetics, 1983; 49(2): 127-36.
Zahalak G I, Cannon S C Predictions of the existence, frequency, and amplitude of physiological tremor in normal man based on measured frequency-response characteristics Journal of biomechanical engineering, 1983; 105(3): 249-57.
Cannon S C, Zahalak G I The mechanical behavior of active human skeletal muscle in small oscillations Journal of biomechanics, 1982; 15(2): 111-21.