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  1. Article ; Online: Epilepsy channelopathies go neddy: stabilizing NaV1.1 channels by neddylation.

    Cannon, Stephen C

    The Journal of clinical investigation

    2021  Volume 131, Issue 8

    Abstract: Loss-of-function mutations of SCN1A encoding the pore-forming α subunit of the NaV1.1 neuronal sodium channel cause a severe developmental epileptic encephalopathy, Dravet syndrome (DS). In this issue of the JCI, Chen, Luo, Gao, et al. describe a ... ...

    Abstract Loss-of-function mutations of SCN1A encoding the pore-forming α subunit of the NaV1.1 neuronal sodium channel cause a severe developmental epileptic encephalopathy, Dravet syndrome (DS). In this issue of the JCI, Chen, Luo, Gao, et al. describe a phenocopy for DS in mice deficient for posttranslational conjugation with neural precursor cell expressed, developmentally downregulated 8 (NEDD8) (neddylation), selectively engineered in inhibitory interneurons. Pursuing the possibility that this phenotype is also caused by loss of NaV1.1, Chen, Luo, Gao, and colleagues show that interneuron excitability and GABA release are impaired, NaV1.1 degradation rate is increased with a commensurate decrease of NaV1.1 protein, and NaV1.1 is a substrate for neddylation. These findings establish neddylation as a mechanism for stabilizing NaV1.1 subunits and suggest another pathomechanism for epileptic sodium channelopathy.
    MeSH term(s) Animals ; Channelopathies/genetics ; Epilepsies, Myoclonic/genetics ; Epilepsy/genetics ; Interneurons ; Mice ; NAV1.1 Voltage-Gated Sodium Channel/genetics
    Chemical Substances NAV1.1 Voltage-Gated Sodium Channel ; Scn1a protein, mouse
    Language English
    Publishing date 2021-04-15
    Publishing country United States
    Document type Journal Article ; Research Support, N.I.H., Extramural ; Research Support, Non-U.S. Gov't ; Comment
    ZDB-ID 3067-3
    ISSN 1558-8238 ; 0021-9738
    ISSN (online) 1558-8238
    ISSN 0021-9738
    DOI 10.1172/JCI148370
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  2. Article ; Online: A role for external Ca2+ in maintaining muscle contractility in periodic paralysis.

    Cannon, Stephen C

    The Journal of general physiology

    2020  Volume 152, Issue 7

    MeSH term(s) Animals ; Calcium ; Depression ; Mice ; Muscle Contraction ; Muscles ; Paralysis, Hyperkalemic Periodic
    Chemical Substances Calcium (SY7Q814VUP)
    Language English
    Publishing date 2020-05-28
    Publishing country United States
    Document type Journal Article ; Research Support, N.I.H., Extramural ; Comment
    ZDB-ID 3118-5
    ISSN 1540-7748 ; 0022-1295
    ISSN (online) 1540-7748
    ISSN 0022-1295
    DOI 10.1085/jgp.202012615
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  3. Article ; Online: Further evidence for shared genetic susceptibility in both sporadic and Thyrotoxic periodic paralysis.

    Cannon, Stephen C

    Journal of the neurological sciences

    2020  Volume 412, Page(s) 116794

    MeSH term(s) Genetic Predisposition to Disease ; Humans ; Hypokalemic Periodic Paralysis/genetics ; Paralyses, Familial Periodic/genetics ; Paralysis ; Thyrotoxicosis
    Language English
    Publishing date 2020-03-20
    Publishing country Netherlands
    Document type Editorial
    ZDB-ID 80160-4
    ISSN 1878-5883 ; 0022-510X ; 0374-8642
    ISSN (online) 1878-5883
    ISSN 0022-510X ; 0374-8642
    DOI 10.1016/j.jns.2020.116794
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  4. Article ; Online: Skeletal muscle channelopathy: a new risk for sudden infant death syndrome.

    Cannon, Stephen C

    Lancet (London, England)

    2018  Volume 391, Issue 10129, Page(s) 1457–1458

    MeSH term(s) Channelopathies ; Humans ; Infant ; Muscle, Skeletal ; Risk Factors ; Sudden Infant Death
    Language English
    Publishing date 2018-04-05
    Publishing country England
    Document type Journal Article ; Comment
    ZDB-ID 3306-6
    ISSN 1474-547X ; 0023-7507 ; 0140-6736
    ISSN (online) 1474-547X
    ISSN 0023-7507 ; 0140-6736
    DOI 10.1016/S0140-6736(18)30477-X
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  5. Article ; Online: Mind the magnesium, in dantrolene suppression of malignant hyperthermia.

    Cannon, Stephen C

    Proceedings of the National Academy of Sciences of the United States of America

    2017  Volume 114, Issue 18, Page(s) 4576–4578

    Language English
    Publishing date 2017-05-02
    Publishing country United States
    Document type Journal Article
    ZDB-ID 209104-5
    ISSN 1091-6490 ; 0027-8424
    ISSN (online) 1091-6490
    ISSN 0027-8424
    DOI 10.1073/pnas.1704103114
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  6. Article ; Online: An atypical Ca

    Cannon, Stephen C

    The Journal of general physiology

    2017  Volume 149, Issue 12, Page(s) 1061–1064

    MeSH term(s) Calcium ; Calcium Channels/genetics ; Humans ; Hypokalemic Periodic Paralysis/genetics ; Mutation
    Chemical Substances Calcium Channels ; Calcium (SY7Q814VUP)
    Language English
    Publishing date 2017-11-14
    Publishing country United States
    Document type Journal Article ; Research Support, N.I.H., Extramural ; Comment
    ZDB-ID 3118-5
    ISSN 1540-7748 ; 0022-1295
    ISSN (online) 1540-7748
    ISSN 0022-1295
    DOI 10.1085/jgp.201711923
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  7. Article ; Online: Voltage-dependent Ca

    DiFranco, Marino / Cannon, Stephen C

    American journal of physiology. Cell physiology

    2022  Volume 323, Issue 2, Page(s) C478–C485

    Abstract: Hypokalemic periodic paralysis (HypoPP) is a channelopathy of skeletal muscle caused by missense mutations in the voltage sensor domains (usually at an arginine of the S4 segment) of the ... ...

    Abstract Hypokalemic periodic paralysis (HypoPP) is a channelopathy of skeletal muscle caused by missense mutations in the voltage sensor domains (usually at an arginine of the S4 segment) of the Ca
    MeSH term(s) Animals ; Calcium Channels, L-Type/genetics ; Disease Models, Animal ; Excitation Contraction Coupling ; Hypokalemic Periodic Paralysis/genetics ; Mice ; Muscle, Skeletal/metabolism ; Mutation, Missense ; NAV1.4 Voltage-Gated Sodium Channel/genetics
    Chemical Substances CACNA1S protein, mouse ; Calcium Channels, L-Type ; NAV1.4 Voltage-Gated Sodium Channel ; Scn4a protein, mouse
    Language English
    Publishing date 2022-06-27
    Publishing country United States
    Document type Journal Article ; Research Support, N.I.H., Extramural
    ZDB-ID 392098-7
    ISSN 1522-1563 ; 0363-6143
    ISSN (online) 1522-1563
    ISSN 0363-6143
    DOI 10.1152/ajpcell.00209.2022
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  8. Article ; Online: Retigabine suppresses loss of force in mouse models of hypokalaemic periodic paralysis.

    Quiñonez, Marbella / DiFranco, Marino / Wu, Fenfen / Cannon, Stephen C

    Brain : a journal of neurology

    2023  Volume 146, Issue 4, Page(s) 1554–1560

    Abstract: Recurrent episodes of weakness in periodic paralysis are caused by intermittent loss of muscle fibre excitability, as a consequence of sustained depolarization of the resting potential. Repolarization is favoured by increasing the fibre permeability to ... ...

    Abstract Recurrent episodes of weakness in periodic paralysis are caused by intermittent loss of muscle fibre excitability, as a consequence of sustained depolarization of the resting potential. Repolarization is favoured by increasing the fibre permeability to potassium. Based on this principle, we tested the efficacy of retigabine, a potassium channel opener, to suppress the loss of force induced by a low-K+ challenge in hypokalaemic periodic paralysis (HypoPP). Retigabine can prevent the episodic loss of force in HypoPP. Knock-in mutant mouse models of HypoPP (Cacna1s p.R528H and Scn4a p.R669H) were used to determine whether pre-treatment with retigabine prevented the loss of force, or post-treatment hastened recovery of force for a low-K+ challenge in an ex vivo contraction assay. Retigabine completely prevents the loss of force induced by a 2 mM K+ challenge (protection) in our mouse models of HypoPP, with 50% inhibitory concentrations of 0.8 ± 0.13 μM and 2.2 ± 0.42 μM for NaV1.4-R669H and CaV1.1-R528H, respectively. In comparison, the effective concentration for the KATP channel opener pinacidil was 10-fold higher. Application of retigabine also reversed the loss of force (rescue) for HypoPP muscle maintained in 2 mM K+. Our findings show that retigabine, a selective agonist of the KV7 family of potassium channels, is effective for the prevention of low-K+ induced attacks of weakness and to enhance recovery from an ongoing loss of force in mouse models of type 1 (Cacna1s) and type 2 (Scn4a) HypoPP. Substantial protection from the loss of force occurred in the low micromolar range, well within the therapeutic window for retigabine.
    MeSH term(s) Mice ; Animals ; Hypokalemic Periodic Paralysis ; Muscle, Skeletal ; Carbamates/pharmacology ; Carbamates/therapeutic use ; Phenylenediamines/pharmacology ; Phenylenediamines/therapeutic use
    Chemical Substances ezogabine (12G01I6BBU) ; Carbamates ; Phenylenediamines
    Language English
    Publishing date 2023-01-30
    Publishing country England
    Document type Journal Article ; Research Support, N.I.H., Extramural
    ZDB-ID 80072-7
    ISSN 1460-2156 ; 0006-8950
    ISSN (online) 1460-2156
    ISSN 0006-8950
    DOI 10.1093/brain/awac441
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  9. Article: Sodium Channelopathies of Skeletal Muscle.

    Cannon, Stephen C

    Handbook of experimental pharmacology

    2017  Volume 246, Page(s) 309–330

    Abstract: ... The ... ...

    Abstract The Na
    MeSH term(s) Animals ; Channelopathies/etiology ; Humans ; Muscular Diseases/etiology ; Mutation ; Myotonia/etiology ; Myotonia Congenita/etiology ; NAV1.4 Voltage-Gated Sodium Channel/genetics ; Paralysis, Hyperkalemic Periodic/etiology
    Chemical Substances NAV1.4 Voltage-Gated Sodium Channel
    Language English
    Publishing date 2017-09-22
    Publishing country Germany
    Document type Journal Article ; Research Support, N.I.H., Extramural ; Review
    ISSN 0171-2004
    ISSN 0171-2004
    DOI 10.1007/164_2017_52
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    Sign. bis Bd. 125 (1997): 1982 A 2146: Show issues
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  10. Article ; Online: Effects of Weight Cutting on Exercise Performance in Combat Athletes: A Meta-Analysis.

    Brechney, Grant C / Cannon, Jack / Goodman, Stephen P

    International journal of sports physiology and performance

    2022  Volume 17, Issue 7, Page(s) 995–1010

    Abstract: Weight cutting in combat sports is a prevalent practice whereby athletes voluntarily dehydrate themselves via various methods to induce rapid weight loss (RWL) to qualify for a lower weight category than that of their usual training body weight. The ... ...

    Abstract Weight cutting in combat sports is a prevalent practice whereby athletes voluntarily dehydrate themselves via various methods to induce rapid weight loss (RWL) to qualify for a lower weight category than that of their usual training body weight. The intention behind this practice is to regain the lost body mass and compete at a heavier mass than permitted by the designated weight category. The purpose of this study was to quantitatively synthesize the available evidence examining the effects of weight cutting on exercise performance in combat-sport athletes. Following a systematic search of the literature, meta-analyses were performed to compare maximal strength, maximal power, anaerobic capacity, and/or repeated high-intensity-effort performance before rapid weight loss (pre-RWL), immediately following RWL (post-RWL), and 3 to 36 hours after RWL following recovery and rapid weight gain (post-RWG). Overall, exercise performance was unchanged between pre-RWL and post-RWG (g = 0.22; 95% CI, -0.18 to 0.62). Between pre-RWL and post-RWL analyses revealed small reductions in maximal strength and repeated high-intensity-effort performance (g = -0.29; 95% CI, -0.54 to -0.03 and g = -0.37; 95% CI, -0.59 to -0.16, respectively; both P ≤ .03). Qualitative analysis indicates that maximal strength and power remained comparable between post-RWL and post-RWG. These data suggest that weight cutting in combat-sport athletes does not alter short-duration, repeated high-intensity-effort performance; however, there is evidence to suggest that select exercise performance outcomes may decline as a product of RWL. It remains unclear whether these are restored by RWG.
    MeSH term(s) Athletes ; Exercise ; Humans ; Martial Arts ; Weight Gain ; Weight Loss
    Language English
    Publishing date 2022-05-06
    Publishing country United States
    Document type Journal Article ; Meta-Analysis ; Systematic Review
    ISSN 1555-0273
    ISSN (online) 1555-0273
    DOI 10.1123/ijspp.2021-0104
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