Structural basis for gating pore current in periodic paralysis

Potassium-sensitive hypokalaemic and normokalaemic periodic paralysis are inherited skeletal muscle diseases characterized by episodes of flaccid muscle weakness 1 , 2 . They are caused by single mutations in positively charged residues (‘gating charges’) in the S4 transmembrane segment of the volta...

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Veröffentlicht in:	Nature (London) 2018-05, Vol.557 (7706), p.590-594
Hauptverfasser:	Jiang, Daohua, Gamal El-Din, Tamer M., Ing, Christopher, Lu, Peilong, Pomès, Régis, Zheng, Ning, Catterall, William A.
Format:	Artikel
Sprache:	eng
Schlagworte:	631/378 631/535/1266 82/16 9/74 Binding Sites Calcium Calcium channels Calcium channels (voltage-gated) Cations Causes of Channel gating Computer simulation Crystal structure Electric Conductivity Electric potential Familial periodic paralysis Gene mutation Genetic aspects Guanidine - metabolism Health aspects Humanities and Social Sciences Humans Hydrophobicity Hypokalemic Periodic Paralysis - genetics Hypokalemic Periodic Paralysis - metabolism Ion Channel Gating - genetics Ions Letter Molecular chains Molecular dynamics Molecular Dynamics Simulation Molecular modelling multidisciplinary Muscles Mutation NAV1.4 Voltage-Gated Sodium Channel - chemistry NAV1.4 Voltage-Gated Sodium Channel - genetics NAV1.4 Voltage-Gated Sodium Channel - metabolism Observations Paralyses, Familial Periodic - genetics Paralyses, Familial Periodic - metabolism Paralysis Potassium Resveratrol Science Science (multidisciplinary) Skeletal muscle Sodium Sodium - metabolism Sodium channels Sodium channels (voltage-gated) Thermodynamics Variation
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