Stephen Caldwell Cannon

Title(s)Act/interim Department Chair, Molecular and Medical Pharmacology
ORCID ORCID Icon0000-0001-8428-3690 Additional info
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    Other Positions
    Title(s)Act/interim Director, Molecular and Medical Pharmacology

    Title(s)Department Chair, Physiology

    Title(s)Professor, Physiology

    Title(s)Professor, Neurology

    Collapse Biography 
    Collapse Education and Training
    Washington University, St. LouisBS06/1980Mech Engr
    Washington University, St. LouisMS06/1980Mech Engr
    Johns Hopkins School of Med, BaltimoreMD06/1986Medicine
    Johns Hopkins School of Med, BaltimorePhD06/1986Biomed Engr
    Collapse Awards and Honors
    Massachusetts General Hospital2000Derek Denny-Brown Neurological Scholar
    UT Southwestern Medical Center2002Linda and Mitch Hart Distinguished Chair in Neurology
    UT Southwestern Medical Center2008  - 2018MERIT Award, NIH
    UT Southwestern Medical Center2014Regents Outstanding Teaching Award
    Univresity of Rochester2019Paul Horowicz Endowed Lecture
    UCLA2022Biophysical Society Award in the Biophysics of Health and Disease

    Collapse Overview 
    Collapse Websites

    Collapse Research 
    Collapse Research Activities and Funding
    Pathophysiology of Myotonia and Periodic Paralysis
    NIH R01AR078198Jul 1, 2021 - Jun 30, 2026
    Role: Principal Investigator
    Disease Pathogenesis and Modification for CaV1.1-Associated Hypokalemic Periodic
    NIH R01AR063182Jul 1, 2012 - Jun 30, 2022
    Role: Principal Investigator
    Molecular Physiology of Myotonia and Periodic Paralysis
    NIH R37AR042703Mar 10, 1994 - Mar 31, 2020
    Role: Principal Investigator
    Molecular Physiology of Neuromusclar Diseases
    NIH R01AR042703Mar 10, 1994 - Mar 31, 2008
    Role: Principal Investigator

    Collapse Bibliographic 
    Collapse Publications
    Publications listed below are automatically derived from MEDLINE/PubMed and other sources, which might result in incorrect or missing publications. Researchers can login to make corrections and additions, or contact us for help. to make corrections and additions.
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    Altmetrics Details PMC Citations indicate the number of times the publication was cited by articles in PubMed Central, and the Altmetric score represents citations in news articles and social media. (Note that publications are often cited in additional ways that are not shown here.) Fields are based on how the National Library of Medicine (NLM) classifies the publication's journal and might not represent the specific topic of the publication. Translation tags are based on the publication type and the MeSH terms NLM assigns to the publication. Some publications (especially newer ones and publications not in PubMed) might not yet be assigned Field or Translation tags.) Click a Field or Translation tag to filter the publications.
    1. Retigabine suppresses loss of force in mouse models of hypokalaemic periodic paralysis. Brain. 2023 04 19; 146(4):1554-1560. Quiñonez M, DiFranco M, Wu F, Cannon SC. PMID: 36718088; PMCID: PMC10115351.
      View in: PubMed   Mentions: 1     Fields:    Translation:Animals
    2. Voltage-dependent Ca2+ release is impaired in hypokalemic periodic paralysis caused by CaV1.1-R528H but not by NaV1.4-R669H. Am J Physiol Cell Physiol. 2022 08 01; 323(2):C478-C485. DiFranco M, Cannon SC. PMID: 35759432; PMCID: PMC9359662.
      View in: PubMed   Mentions: 1     Fields:    Translation:AnimalsCells
    3. The distinct role of the four voltage sensors of the skeletal CaV1.1 channel in voltage-dependent activation. J Gen Physiol. 2021 11 01; 153(11). Savalli N, Angelini M, Steccanella F, Wier J, Wu F, Quinonez M, DiFranco M, Neely A, Cannon SC, Olcese R. PMID: 34546289; PMCID: PMC8460119.
      View in: PubMed   Mentions: 11     Fields:    Translation:Humans
    4. Gating pore currents occur in CaV1.1 domain III mutants associated with HypoPP. J Gen Physiol. 2021 11 01; 153(11). Wu F, Quinonez M, Cannon SC. PMID: 34463712; PMCID: PMC8563280.
      View in: PubMed   Mentions: 6     Fields:    Translation:Humans
    5. Epilepsy channelopathies go neddy: stabilizing NaV1.1 channels by neddylation. J Clin Invest. 2021 04 15; 131(8). Cannon SC. PMID: 33855971; PMCID: PMC8262487.
      View in: PubMed   Mentions: 1     Fields:    Translation:AnimalsCells
    6. Hypokalaemic periodic paralysis with a charge-retaining substitution in the voltage sensor. Brain Commun. 2020; 2(2):fcaa103. Kubota T, Wu F, Vicart S, Nakaza M, Sternberg D, Watanabe D, Furuta M, Kokunai Y, Abe T, Kokubun N, Fontaine B, Cannon SC, Takahashi MP. PMID: 33005891; PMCID: PMC7519726.
      View in: PubMed   Mentions: 9  
    7. A role for external Ca2+ in maintaining muscle contractility in periodic paralysis. J Gen Physiol. 2020 07 06; 152(7). Cannon SC. PMID: 32463879; PMCID: PMC7335007.
      View in: PubMed   Mentions: 1     Fields:    Translation:Animals
    8. Guidelines on clinical presentation and management of nondystrophic myotonias. Muscle Nerve. 2020 10; 62(4):430-444. Stunnenberg BC, LoRusso S, Arnold WD, Barohn RJ, Cannon SC, Fontaine B, Griggs RC, Hanna MG, Matthews E, Meola G, Sansone VA, Trivedi JR, van Engelen BGM, Vicart S, Statland JM. PMID: 32270509; PMCID: PMC8117169.
      View in: PubMed   Mentions: 25     Fields:    Translation:Humans
    9. Further evidence for shared genetic susceptibility in both sporadic and Thyrotoxic periodic paralysis. J Neurol Sci. 2020 05 15; 412:116794. Cannon SC. PMID: 32229026; PMCID: PMC8137387.
      View in: PubMed   Mentions: 3     Fields:    Translation:Humans
    10. Corrigendum: Myotonic Myopathy With Secondary Joint and Skeletal Anomalies From the c.2386C>G, p.L796V Mutation in SCN4A. Front Neurol. 2020; 11:181. Elia N, Nault T, McMillan HJ, Graham GE, Huang L, Cannon SC. PMID: 32265824; PMCID: PMC7099287.
      View in: PubMed   Mentions:
    11. Myotonic Myopathy With Secondary Joint and Skeletal Anomalies From the c.2386C>G, p.L769V Mutation in SCN4A. Front Neurol. 2020; 11:77. Elia N, Nault T, McMillan HJ, Graham GE, Huang L, Cannon SC. PMID: 32117035; PMCID: PMC7031655.
      View in: PubMed   Mentions: 3  
    12. A highly-selective chloride microelectrode based on a mercuracarborand anion carrier. Sci Rep. 2019 12 11; 9(1):18860. DiFranco M, Quinonez M, Dziedzic RM, Spokoyny AM, Cannon SC. PMID: 31827130; PMCID: PMC6906508.
      View in: PubMed   Mentions: 2     Fields:    Translation:Animals
    13. Resurgent and Gating Pore Currents Induced by De Novo SCN2A Epilepsy Mutations. eNeuro. 2019 Sep/Oct; 6(5). Mason ER, Wu F, Patel RR, Xiao Y, Cannon SC, Cummins TR. PMID: 31558572; PMCID: PMC6795554.
      View in: PubMed   Mentions: 18     Fields:    Translation:HumansAnimalsCells
    14. A four-electrode method to study dynamics of ion activity and transport in skeletal muscle fibers. J Gen Physiol. 2019 09 02; 151(9):1146-1155. Heiny JA, Cannon SC, DiFranco M. PMID: 31320390; PMCID: PMC6719403.
      View in: PubMed   Mentions: 4     Fields:    Translation:AnimalsCells
    15. Myasthenic congenital myopathy from recessive mutations at a single residue in NaV1.4. Neurology. 2019 03 26; 92(13):e1405-e1415. Elia N, Palmio J, Castañeda MS, Shieh PB, Quinonez M, Suominen T, Hanna MG, Männikkö R, Udd B, Cannon SC. PMID: 30824560; PMCID: PMC6453767.
      View in: PubMed   Mentions: 13     Fields:    Translation:HumansAnimalsCells
    16. Recovery from acidosis is a robust trigger for loss of force in murine hypokalemic periodic paralysis. J Gen Physiol. 2019 04 01; 151(4):555-566. Mi W, Wu F, Quinonez M, DiFranco M, Cannon SC. PMID: 30733232; PMCID: PMC6445579.
      View in: PubMed   Mentions: 3     Fields:    Translation:Animals
    17. Skeletal muscle channelopathy: a new risk for sudden infant death syndrome. Lancet. 2018 04 14; 391(10129):1457-1458. Cannon SC. PMID: 29605428.
      View in: PubMed   Mentions: 1     Fields:    Translation:Humans
    18. Kv4.2 autism and epilepsy mutation enhances inactivation of closed channels but impairs access to inactivated state after opening. Proc Natl Acad Sci U S A. 2018 04 10; 115(15):E3559-E3568. Lin MA, Cannon SC, Papazian DM. PMID: 29581270; PMCID: PMC5899440.
      View in: PubMed   Mentions: 14     Fields:    Translation:HumansAnimalsCells
    19. Stac3 enhances expression of human CaV1.1 in Xenopus oocytes and reveals gating pore currents in HypoPP mutant channels. J Gen Physiol. 2018 03 05; 150(3):475-489. Wu F, Quinonez M, DiFranco M, Cannon SC. PMID: 29386226; PMCID: PMC5839724.
      View in: PubMed   Mentions: 21     Fields:    Translation:HumansAnimalsCells
    20. Sodium Channelopathies of Skeletal Muscle. Handb Exp Pharmacol. 2018; 246:309-330. Cannon SC. PMID: 28939973; PMCID: PMC5866235.
      View in: PubMed   Mentions: 61     Fields:    Translation:HumansAnimals
    21. Review of the Diagnosis and Treatment of Periodic Paralysis. Muscle Nerve. 2018 04; 57(4):522-530. Statland JM, Fontaine B, Hanna MG, Johnson NE, Kissel JT, Sansone VA, Shieh PB, Tawil RN, Trivedi J, Cannon SC, Griggs RC. PMID: 29125635; PMCID: PMC5867231.
      View in: PubMed   Mentions: 67     Fields:    Translation:Humans
    22. An atypical CaV1.1 mutation reveals a common mechanism for hypokalemic periodic paralysis. J Gen Physiol. 2017 12 04; 149(12):1061-1064. Cannon SC. PMID: 29138267; PMCID: PMC5715912.
      View in: PubMed   Mentions: 4     Fields:    Translation:Humans
    23. Mind the magnesium, in dantrolene suppression of malignant hyperthermia. Proc Natl Acad Sci U S A. 2017 05 02; 114(18):4576-4578. Cannon SC. PMID: 28442565; PMCID: PMC5422787.
      View in: PubMed   Mentions: 3     Fields:    Translation:Humans
    24. Mice with an NaV1.4 sodium channel null allele have latent myasthenia, without susceptibility to periodic paralysis. Brain. 2016 06; 139(Pt 6):1688-99. Wu F, Mi W, Fu Y, Struyk A, Cannon SC. PMID: 27048647; PMCID: PMC4892753.
      View in: PubMed   Mentions: 12     Fields:    Translation:Animals
    25. When all is lost…a severe myopathy with hypotonia from sodium channel mutations. Brain. 2016 Mar; 139(Pt 3):642-4. Cannon SC. PMID: 26917582; PMCID: PMC4990650.
      View in: PubMed   Mentions: 1     Fields:    Translation:HumansAnimals
    26. A peptide encoded by a transcript annotated as long noncoding RNA enhances SERCA activity in muscle. Science. 2016 Jan 15; 351(6270):271-5. Nelson BR, Makarewich CA, Anderson DM, Winders BR, Troupes CD, Wu F, Reese AL, McAnally JR, Chen X, Kavalali ET, Cannon SC, Houser SR, Bassel-Duby R, Olson EN. PMID: 26816378; PMCID: PMC4892890.
      View in: PubMed   Mentions: 347     Fields:    Translation:HumansAnimalsCells
    27. Channelopathies of skeletal muscle excitability. Compr Physiol. 2015 Apr; 5(2):761-90. Cannon SC. PMID: 25880512; PMCID: PMC4754081.
      View in: PubMed   Mentions: 101     Fields:    Translation:HumansCells
    28. Disrupted coupling of gating charge displacement to Na+ current activation for DIIS4 mutations in hypokalemic periodic paralysis. J Gen Physiol. 2014 Aug; 144(2):137-45. Mi W, Rybalchenko V, Cannon SC. PMID: 25024265; PMCID: PMC4113897.
      View in: PubMed   Mentions: 13     Fields:    Translation:HumansAnimalsCells
    29. Nondystrophic myotonia: challenges and future directions. Exp Neurol. 2014 Mar; 253:28-30. Trivedi JR, Cannon SC, Griggs RC. PMID: 24361411; PMCID: PMC4078724.
      View in: PubMed   Mentions: 21     Fields:    Translation:Humans
    30. Beneficial effects of bumetanide in a CaV1.1-R528H mouse model of hypokalaemic periodic paralysis. Brain. 2013 Dec; 136(Pt 12):3766-74. Wu F, Mi W, Cannon SC. PMID: 24142145; PMCID: PMC3859222.
      View in: PubMed   Mentions: 16     Fields:    Translation:Animals
    31. Skeletal muscle-specific T-tubule protein STAC3 mediates voltage-induced Ca2+ release and contractility. Proc Natl Acad Sci U S A. 2013 Jul 16; 110(29):11881-6. Nelson BR, Wu F, Liu Y, Anderson DM, McAnally J, Lin W, Cannon SC, Bassel-Duby R, Olson EN. PMID: 23818578; PMCID: PMC3718085.
      View in: PubMed   Mentions: 83     Fields:    Translation:Animals
    32. Bumetanide prevents transient decreases in muscle force in murine hypokalemic periodic paralysis. Neurology. 2013 Mar 19; 80(12):1110-6. Wu F, Mi W, Cannon SC. PMID: 23427324; PMCID: PMC3662304.
      View in: PubMed   Mentions: 17     Fields:    Translation:Animals
    33. A calcium channel mutant mouse model of hypokalemic periodic paralysis. J Clin Invest. 2012 Dec; 122(12):4580-91. Wu F, Mi W, Hernández-Ochoa EO, Burns DK, Fu Y, Gray HF, Struyk AF, Schneider MF, Cannon SC. PMID: 23187123; PMCID: PMC3533564.
      View in: PubMed   Mentions: 56     Fields:    Translation:HumansAnimalsCells
    34. Small fiber neuropathy: a bit less idiopathic? Neurology. 2012 May 22; 78(21):1626-7. Cannon SC. PMID: 22539571.
      View in: PubMed   Mentions:    Fields:    Translation:Humans
    35. A sodium channel knockin mutant (NaV1.4-R669H) mouse model of hypokalemic periodic paralysis. J Clin Invest. 2011 Oct; 121(10):4082-94. Wu F, Mi W, Burns DK, Fu Y, Gray HF, Struyk AF, Cannon SC. PMID: 21881211; PMCID: PMC3195470.
      View in: PubMed   Mentions: 46     Fields:    Translation:HumansAnimals
    36. 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. Hum Mol Genet. 2011 Oct 15; 20(20):3925-32. Kudryashova E, Struyk A, Mokhonova E, Cannon SC, Spencer MJ. PMID: 21775502; PMCID: PMC3177646.
      View in: PubMed   Mentions: 27     Fields:    Translation:Animals
    37. Identification and functional characterization of Kir2.6 mutations associated with non-familial hypokalemic periodic paralysis. J Biol Chem. 2011 Aug 05; 286(31):27425-35. Cheng CJ, Lin SH, Lo YF, Yang SS, Hsu YJ, Cannon SC, Huang CL. PMID: 21665951; PMCID: PMC3149336.
      View in: PubMed   Mentions: 29     Fields:    Translation:HumansCells
    38. Leaky sodium channels from voltage sensor mutations in periodic paralysis, but not paramyotonia. Neurology. 2011 May 10; 76(19):1635-41. Francis DG, Rybalchenko V, Struyk A, Cannon SC. PMID: 21490317; PMCID: PMC3100087.
      View in: PubMed   Mentions: 30     Fields:    Translation:AnimalsCells
    39. Voltage-sensor mutations in channelopathies of skeletal muscle. J Physiol. 2010 Jun 01; 588(Pt 11):1887-95. Cannon SC. PMID: 20156847; PMCID: PMC2901977.
      View in: PubMed   Mentions: 63     Fields:    Translation:HumansAnimalsCells
    40. Sodium channels gone wild: resurgent current from neuronal and muscle channelopathies. J Clin Invest. 2010 Jan; 120(1):80-3. Cannon SC, Bean BP. PMID: 20038809; PMCID: PMC2798702.
      View in: PubMed   Mentions: 15     Fields:    Translation:HumansCells
    41. Getting a charge out of periodic paralysis? Neurology. 2009 May 05; 72(18):1540-1. Cannon SC. PMID: 19414720.
      View in: PubMed   Mentions:    Fields:    Translation:HumansCells
    42. Slow inactivation of the NaV1.4 sodium channel in mammalian cells is impeded by co-expression of the beta1 subunit. Pflugers Arch. 2009 Apr; 457(6):1253-63. Webb J, Wu FF, Cannon SC. PMID: 18941776; PMCID: PMC6207185.
      View in: PubMed   Mentions: 12     Fields:    Translation:HumansCells
    43. Gating pore currents in DIIS4 mutations of NaV1.4 associated with periodic paralysis: saturation of ion flux and implications for disease pathogenesis. J Gen Physiol. 2008 Oct; 132(4):447-64. Struyk AF, Markin VS, Francis D, Cannon SC. PMID: 18824591; PMCID: PMC2553391.
      View in: PubMed   Mentions: 48     Fields:    Translation:AnimalsCells
    44. Targeted mutation of mouse skeletal muscle sodium channel produces myotonia and potassium-sensitive weakness. J Clin Invest. 2008 Apr; 118(4):1437-49. Hayward LJ, Kim JS, Lee MY, Zhou H, Kim JW, Misra K, Salajegheh M, Wu FF, Matsuda C, Reid V, Cros D, Hoffman EP, Renaud JM, Cannon SC, Brown RH. PMID: 18317596; PMCID: PMC2260907.
      View in: PubMed   Mentions: 33     Fields:    Translation:HumansAnimalsCells
    45. Paradoxical depolarization of BA2+- treated muscle exposed to low extracellular K+: insights into resting potential abnormalities in hypokalemic paralysis. Muscle Nerve. 2008 Mar; 37(3):326-37. Struyk AF, Cannon SC. PMID: 18041053.
      View in: PubMed   Mentions: 40     Fields:    Translation:AnimalsCells
    46. Cold-induced defects of sodium channel gating in atypical periodic paralysis plus myotonia. Neurology. 2008 Mar 04; 70(10):755-61. Webb J, Cannon SC. PMID: 17898326; PMCID: PMC4094148.
      View in: PubMed   Mentions: 27     Fields:    Translation:HumansCells
    47. A Na+ channel mutation linked to hypokalemic periodic paralysis exposes a proton-selective gating pore. J Gen Physiol. 2007 Jul; 130(1):11-20. Struyk AF, Cannon SC. PMID: 17591984; PMCID: PMC2154364.
      View in: PubMed   Mentions: 104     Fields:    Translation:HumansAnimalsCells
    48. Physiologic principles underlying ion channelopathies. Neurotherapeutics. 2007 Apr; 4(2):174-83. Cannon SC. PMID: 17395127.
      View in: PubMed   Mentions: 16     Fields:    Translation:HumansAnimalsCells
    49. Pathomechanisms in channelopathies of skeletal muscle and brain. Annu Rev Neurosci. 2006; 29:387-415. Cannon SC. PMID: 16776591.
      View in: PubMed   Mentions: 78     Fields:    Translation:HumansAnimals
    50. A C-terminal skeletal muscle sodium channel mutation associated with myotonia disrupts fast inactivation. J Physiol. 2005 Jun 01; 565(Pt 2):371-80. Wu FF, Gordon E, Hoffman EP, Cannon SC. PMID: 15774523; PMCID: PMC1464529.
      View in: PubMed   Mentions: 25     Fields:    Translation:HumansCells
    51. Truncated ClC-1 mRNA in myotonic dystrophy exerts a dominant-negative effect on the Cl current. Neurology. 2004 Dec 28; 63(12):2371-5. Berg J, Jiang H, Thornton CA, Cannon SC. PMID: 15623702.
      View in: PubMed   Mentions: 15     Fields:    Translation:HumansAnimalsCells
    52. Paying the price at the pump: dystonia from mutations in a Na+/K+ -ATPase. Neuron. 2004 Jul 22; 43(2):153-4. Cannon SC. PMID: 15260948.
      View in: PubMed   Mentions: 11     Fields:    Translation:Humans
    53. Myasthenic syndrome caused by mutation of the SCN4A sodium channel. Proc Natl Acad Sci U S A. 2003 Jun 10; 100(12):7377-82. Tsujino A, Maertens C, Ohno K, Shen XM, Fukuda T, Harper CM, Cannon SC, Engel AG. PMID: 12766226; PMCID: PMC165883.
      View in: PubMed   Mentions: 68     Fields:    Translation:HumansCells
    54. An expanding view for the molecular basis of familial periodic paralysis. Neuromuscul Disord. 2002 Aug; 12(6):533-43. Cannon SC. PMID: 12117476.
      View in: PubMed   Mentions: 18     Fields:    Translation:Humans
    55. Expanded CUG repeats trigger aberrant splicing of ClC-1 chloride channel pre-mRNA and hyperexcitability of skeletal muscle in myotonic dystrophy. Mol Cell. 2002 Jul; 10(1):35-44. Mankodi A, Takahashi MP, Jiang H, Beck CL, Bowers WJ, Moxley RT, Cannon SC, Thornton CA. PMID: 12150905.
      View in: PubMed   Mentions: 295     Fields:    Translation:HumansAnimalsCells
    56. Sodium channel gating: no margin for error. Neuron. 2002 Jun 13; 34(6):853-4. Cannon SC. PMID: 12086630.
      View in: PubMed   Mentions: 5     Fields:    Translation:HumansCells