Our research is directed toward gaining a better understanding of how ion channels regulate electrical excitability of cells and of how defects in these channels lead to human disease. In the past decade, mutations of ion channel genes have been found for inherited diseases that cause episodic paralysis, familial migraine, episodic ataxia, fatal cardiac arrhythmias, and some forms of epilepsy. We have been studying the consequences of mutations in sodium, calcium, and chloride channels that have been linked to muscle disorders causing episodic paralysis or stiffness (myotonia). Many disease-associated mutations alter the tendency for a channel to open or close (gating behavior), and another interest of the lab is fundamental mechanisms of sodium channel gating.
RESEARCH INTERESTS
myotonia
periodic paralysis
electrophysiology
Ion channel physiology
RECENT PUBLICATIONS
Tsujino, A., Maertens, C., Ohno, K., Shen, X. M., Fukuda, T., Harper, C. M, Cannon, S. C., Engel, A. G., "Myasthenic syndrome caused by mutation of the SCN4A sodium channel" Proc Natl Acad Sci U S A, 100 (12):7377-7382, June 2003
Struyk, A.S. and Cannon, S.C., "The human skeletal muscle Na channel mutation R669H associated with hypokalemic periodic paralysis enhances slow inactivation" J. Neurosci, 20:8610-8617, 2000
Struyk, A.S. and Cannon, S.C, "Slow inactivation does not affect the aqueous accessibility of outer pore-lining residues of voltage-gated Na channels" Journal of General Physiology, 120:509-516, 2002
Struyk, A. and Cannon, S.C., "A Na+ Channel Mutation Linked to Hypokalemic Periodic Paralysis Exposes a Proton-selective Gating Pore" Journal of General Physiology, 130:11-20, July 2007
SIGNIFICANT PUBLICATIONS
Cannon, S.C., Brown, R.H., Corey, D.P., "A sodium channel defect in hyperkalemic periodic paralysis: potassium-induced failure of inactivation" Neuron, 6:619-626, 1991
Ranganathan R., Cannon, SC, Horvitz, RH, "MOD-1 is a serotonin-gated chloride channel that modulates C. elegans locomotory behaviour." Nature, 408:470-475, 2000
Mankodi, A., Takahashi, M.P., Jiang H., Beck, C., Bowers, W., Moxley, R.T., Cannon, S.C., Thornton, C., "Accumulation of poly(CUG) or (CCUG) RNA in the nucleus triggers aberrant splicing of chloride channel 1 pre-mRNA and chloride channelopathy in myotonic dystrophy" Molecular Cell, 10:35-44, 2002
Struyk, A. and Cannon, S.C., "A Na+ Channel Mutation Linked to Hypokalemic Periodic Paralysis Exposes a Proton-selective Gating Pore" Journal of General Physiology, 130:11-20, July 2007
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