Stephen C Cannon

Summary

Affiliation: University of Texas Southwestern Medical Center
Country: USA

Publications

  1. ncbi request reprint Pathomechanisms in channelopathies of skeletal muscle and brain
    Stephen C Cannon
    Department of Neurology, University of Texas Southwestern Medical Center, Dallas, Texas 75390, USA
    Annu Rev Neurosci 29:387-415. 2006
  2. pmc Voltage-sensor mutations in channelopathies of skeletal muscle
    Stephen C Cannon
    Department of Neurology and Program in Neuroscience, 5323 Harry Hines Blvd, UT Southwestern Medical Center, Dallas, TX 75390 8813, USA
    J Physiol 588:1887-95. 2010
  3. pmc Sodium channels gone wild: resurgent current from neuronal and muscle channelopathies
    Stephen C Cannon
    Department of Neurology, University of Texas Southwestern Medical Center, 5223 Harry Hines Blvd, Dallas, Texas 75390 8813, USA
    J Clin Invest 120:80-3. 2010
  4. ncbi request reprint Physiologic principles underlying ion channelopathies
    Stephen C Cannon
    Department of Neurology, University of Texas Southwestern Medical Center, Dallas, Texas 55390 8813, USA
    Neurotherapeutics 4:174-83. 2007
  5. pmc A calcium channel mutant mouse model of hypokalemic periodic paralysis
    Fenfen Wu
    Department of Neurology and Neurotherapeutics, University of Texas Southwestern Medical Center, Dallas, Texas 75390 8813, USA
    J Clin Invest 122:4580-91. 2012
  6. pmc Gating pore currents in DIIS4 mutations of NaV1.4 associated with periodic paralysis: saturation of ion flux and implications for disease pathogenesis
    Arie F Struyk
    Department of Neurology, University of Texas Southwestern Medical Center, Dallas, TX 75390, USA
    J Gen Physiol 132:447-64. 2008
  7. pmc Targeted mutation of mouse skeletal muscle sodium channel produces myotonia and potassium-sensitive weakness
    Lawrence J Hayward
    Department of Neurology, University of Massachusetts Medical School, Worcester, Massachusetts 01655, USA
    J Clin Invest 118:1437-49. 2008
  8. pmc A sodium channel knockin mutant (NaV1.4-R669H) mouse model of hypokalemic periodic paralysis
    Fenfen Wu
    Department of Neurology and Neurotherapeutics, UT Southwestern Medical Center, Dallas, Texas 75390 8813, USA
    J Clin Invest 121:4082-94. 2011
  9. ncbi request reprint Cold-induced defects of sodium channel gating in atypical periodic paralysis plus myotonia
    Jadon Webb
    Department of Neurology, UT Southwestern Medical Center, 5323 Harry Hines Blvd, Dallas, TX 75390 8813, USA
    Neurology 70:755-61. 2008
  10. pmc Skeletal muscle-specific T-tubule protein STAC3 mediates voltage-induced Ca2+ release and contractility
    Benjamin R Nelson
    Department of Molecular Biology, University of Texas Southwestern Medical Center, Dallas, TX 75390, USA
    Proc Natl Acad Sci U S A 110:11881-6. 2013

Research Grants

  1. Molecular Physiology of Neuromusclar Diseases
    Stephen Cannon; Fiscal Year: 2007
  2. MOLECULAR PHYSIOLOGY OF NEUROMUSCULAR DISEASES
    Stephen Cannon; Fiscal Year: 2002

Collaborators

Detail Information

Publications27

  1. ncbi request reprint Pathomechanisms in channelopathies of skeletal muscle and brain
    Stephen C Cannon
    Department of Neurology, University of Texas Southwestern Medical Center, Dallas, Texas 75390, USA
    Annu Rev Neurosci 29:387-415. 2006
    ..The study of these disorders has provided insights on channel structure-function relations, the physiological roles of ion channels, and rational approaches toward therapeutic intervention for many disorders of cellular excitability...
  2. pmc Voltage-sensor mutations in channelopathies of skeletal muscle
    Stephen C Cannon
    Department of Neurology and Program in Neuroscience, 5323 Harry Hines Blvd, UT Southwestern Medical Center, Dallas, TX 75390 8813, USA
    J Physiol 588:1887-95. 2010
    ....
  3. pmc Sodium channels gone wild: resurgent current from neuronal and muscle channelopathies
    Stephen C Cannon
    Department of Neurology, University of Texas Southwestern Medical Center, 5223 Harry Hines Blvd, Dallas, Texas 75390 8813, USA
    J Clin Invest 120:80-3. 2010
    ..The results of this study suggest a widespread pathophysiological role for this mechanism, previously described to occur normally in only a few types of neurons...
  4. ncbi request reprint Physiologic principles underlying ion channelopathies
    Stephen C Cannon
    Department of Neurology, University of Texas Southwestern Medical Center, Dallas, Texas 55390 8813, USA
    Neurotherapeutics 4:174-83. 2007
    ....
  5. pmc A calcium channel mutant mouse model of hypokalemic periodic paralysis
    Fenfen Wu
    Department of Neurology and Neurotherapeutics, University of Texas Southwestern Medical Center, Dallas, Texas 75390 8813, USA
    J Clin Invest 122:4580-91. 2012
    ..4 R669H HypoPP mouse model. This "gating pore current" may be a common mechanism for paradoxical depolarization and susceptibility to HypoPP arising from missense mutations in the S4 voltage sensor of either calcium or sodium channels...
  6. pmc Gating pore currents in DIIS4 mutations of NaV1.4 associated with periodic paralysis: saturation of ion flux and implications for disease pathogenesis
    Arie F Struyk
    Department of Neurology, University of Texas Southwestern Medical Center, Dallas, TX 75390, USA
    J Gen Physiol 132:447-64. 2008
    ....
  7. pmc Targeted mutation of mouse skeletal muscle sodium channel produces myotonia and potassium-sensitive weakness
    Lawrence J Hayward
    Department of Neurology, University of Massachusetts Medical School, Worcester, Massachusetts 01655, USA
    J Clin Invest 118:1437-49. 2008
    ....
  8. pmc A sodium channel knockin mutant (NaV1.4-R669H) mouse model of hypokalemic periodic paralysis
    Fenfen Wu
    Department of Neurology and Neurotherapeutics, UT Southwestern Medical Center, Dallas, Texas 75390 8813, USA
    J Clin Invest 121:4082-94. 2011
    ..4 channels...
  9. ncbi request reprint Cold-induced defects of sodium channel gating in atypical periodic paralysis plus myotonia
    Jadon Webb
    Department of Neurology, UT Southwestern Medical Center, 5323 Harry Hines Blvd, Dallas, TX 75390 8813, USA
    Neurology 70:755-61. 2008
    ..An atypical phenotype with cold-induced hypokalemic paralysis and myotonia at warm temperatures was reported to segregate with the P1158S mutation...
  10. pmc Skeletal muscle-specific T-tubule protein STAC3 mediates voltage-induced Ca2+ release and contractility
    Benjamin R Nelson
    Department of Molecular Biology, University of Texas Southwestern Medical Center, Dallas, TX 75390, USA
    Proc Natl Acad Sci U S A 110:11881-6. 2013
    ..These findings reveal a previously uncharacterized, but required, component of the EC coupling machinery of skeletal muscle and introduce a candidate for consideration in myopathic disorders. ..
  11. doi request reprint Slow inactivation of the NaV1.4 sodium channel in mammalian cells is impeded by co-expression of the beta1 subunit
    Jadon Webb
    Neuroscience Program, UT Southwestern Medical Center, Dallas, TX 75390, USA
    Pflugers Arch 457:1253-63. 2009
    ..Furthermore, the beta1 effect on slow inactivation was shown to be independent of the negative coupling between fast and slow inactivation...
  12. pmc Leaky sodium channels from voltage sensor mutations in periodic paralysis, but not paramyotonia
    David G Francis
    Department of Anesthesiology, UT Southwestern Medical Center, Dallas, TX, USA
    Neurology 76:1635-41. 2011
    ..Herein, we test whether disease-associated Na(V)1.4 mutations in previously untested homologous regions of the channel also give rise to the anomalous current...
  13. pmc A C-terminal skeletal muscle sodium channel mutation associated with myotonia disrupts fast inactivation
    Fen fen Wu
    Department of Neurology, University of Texas Southwestern Medical Center, 5323 Harry Hines Blvd, Dallas, TX 75390 9036, USA
    J Physiol 565:371-80. 2005
    ....
  14. pmc A Na+ channel mutation linked to hypokalemic periodic paralysis exposes a proton-selective gating pore
    Arie F Struyk
    Department of Neurology, University of Texas Southwestern Medical Center, Dallas, TX 75390, USA
    J Gen Physiol 130:11-20. 2007
    ..Rather, it is possible that a sustained proton leak may contribute to instability of V(REST) indirectly, for instance, by interfering with intracellular pH homeostasis...
  15. pmc Slow inactivation does not block the aqueous accessibility to the outer pore of voltage-gated Na channels
    Arie F Struyk
    Department of Neurology, Massachusetts General Hospital, Boston, MA 02114, USA
    J Gen Physiol 120:509-16. 2002
    ..We interpret these results as evidence that the outer mouth of the Na pore remains open while the channel is slow inactivated...
  16. pmc Bumetanide prevents transient decreases in muscle force in murine hypokalemic periodic paralysis
    Fenfen Wu
    Department of Neurology and Neurotherapeutics, UT Southwestern Medical Center, Dallas, TX, USA
    Neurology 80:1110-6. 2013
    ..To test the hypothesis that inhibition of the Na-K-2Cl transporter with bumetanide will reduce the susceptibility to decreases in muscle force in a mouse model of hypokalemic periodic paralysis (HypoPP)...
  17. pmc 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
    Elena Kudryashova
    Department of Neurology, Center for Duchenne Muscular Dystrophy at UCLA, David Geffen School of Medicine at UCLA, Los Angeles, CA 90095, USA
    Hum Mol Genet 20:3925-32. 2011
    ..Thus, one potential mechanism of LGMD2H might be destabilization of mutated TRIM32 protein leading to a null phenotype...
  18. ncbi request reprint Paradoxical depolarization of BA2+- treated muscle exposed to low extracellular K+: insights into resting potential abnormalities in hypokalemic paralysis
    Arie F Struyk
    Department of Neurology, University of Texas Southwestern Medical Center, 6001 Forest Park Avenue, ND4 214B, Dallas, TX 75390 8813, USA
    Muscle Nerve 37:326-37. 2008
    ....
  19. pmc Beneficial effects of bumetanide in a CaV1.1-R528H mouse model of hypokalaemic periodic paralysis
    Fenfen Wu
    1 Department of Neurology and Neurotherapeutics, UT Southwestern Medical Centre, Dallas, Texas, USA
    Brain 136:3766-74. 2013
    ..4 or CaV1.1 mutations. ..
  20. ncbi request reprint Expanded CUG repeats trigger aberrant splicing of ClC-1 chloride channel pre-mRNA and hyperexcitability of skeletal muscle in myotonic dystrophy
    Ami Mankodi
    Department of Neurology, School of Medicine and Dentistry, University of Rochester, Box 673, 601 Elmwood Avenue, New York 14642, USA
    Mol Cell 10:35-44. 2002
    ..We propose that a transdominant effect of mutant RNA on RNA processing leads to chloride channelopathy and membrane hyperexcitability in DM...
  21. pmc Identification and functional characterization of Kir2.6 mutations associated with non-familial hypokalemic periodic paralysis
    Chih Jen Cheng
    Department of Medicine, Division of Nephrology, University of Texas, Southwestern Medical Center, Dallas, Texas 75390 8856, USA
    J Biol Chem 286:27425-35. 2011
    ..6 predisposes the sarcolemma to hypokalemia-induced paradoxical depolarization during attacks, which in turn leads to Na(+) channel inactivation and inexcitability of muscles...
  22. ncbi request reprint Paying the price at the pump: dystonia from mutations in a Na+/K+ -ATPase
    Stephen C Cannon
    Department of Neurology, UT Southwestern Medical Center at Dallas, TX 75390, USA
    Neuron 43:153-4. 2004
    ..In this issue of Neuron, de Carvalho Aguiar and colleagues report the identification of missense mutations in the gene for the Na+/K+ -ATPase alpha3 subunit (ATP1A3) as a cause of rapid-onset dystonia-parkinsonism (RDP, DYT12)...
  23. ncbi request reprint An expanding view for the molecular basis of familial periodic paralysis
    Stephen C Cannon
    Department of Neurology, Massachusetts General Hospital Wellman 423, 50 Blossom Street, Boston, MA 02114, USA
    Neuromuscul Disord 12:533-43. 2002
    ..This review focuses on the clinical features, molecular genetic defects, and pathophysiologic mechanisms that underlie familial periodic paralysis...
  24. pmc Gating behaviour of sodium currents in adult mouse muscle recorded with an improved two-electrode voltage clamp
    Yu Fu
    Program in Neuroscience, UT Southwestern Medical Center, Dallas, TX 75390, USA
    J Physiol 589:525-46. 2011
    ..These results provide normative data for sodium channels natively expressed in mouse muscle and illustrate the need to modify model simulations of muscle excitability to account for the hyperpolarized shift...
  25. ncbi request reprint Truncated ClC-1 mRNA in myotonic dystrophy exerts a dominant-negative effect on the Cl current
    Jim Berg
    Department of Neurobiology, Harvard Medical School, Boston, MA, USA
    Neurology 63:2371-5. 2004
    ....
  26. pmc Myasthenic syndrome caused by mutation of the SCN4A sodium channel
    Akira Tsujino
    Department of Neurology and Neuromuscular Research Laboratory, Mayo Clinic, Rochester, MN 55905, USA
    Proc Natl Acad Sci U S A 100:7377-82. 2003
    ..The V1442E mutation in SCN4A defines a novel disease mechanism and a novel phenotype with myasthenic features...
  27. ncbi request reprint Sodium channel gating: no margin for error
    Stephen C Cannon
    Department of Neurobiology, Harvard Medical School and Massachusetts General Hospital, Wellman 423 Massachusetts General Hospital, Boston, MA 02114, USA
    Neuron 34:853-4. 2002
    ....

Research Grants15

  1. Molecular Physiology of Neuromusclar Diseases
    Stephen Cannon; Fiscal Year: 2007
    ..abstract_text> ..
  2. MOLECULAR PHYSIOLOGY OF NEUROMUSCULAR DISEASES
    Stephen Cannon; Fiscal Year: 2002
    ....