Canalis ionticus natrii

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Canales iontici natrii sunt canales iontici cum permeabilitate selectiva iontis natrii (Na⁺) trans cellularum membranas plasmaticas. Intra cellulas excitabiles ut neurona, myocytos, varios typos cellularum glialium, cardiomyocytos canales natrii generationem partis ascendentis potentialis actionis adducunt. Canalium natrii ionticorum classificatio modum incitandi (tensione electrica, voltage-gated, et alligatura substantiarum, ligand-gated) respondet.

Mutationes geneticae illorum momentum functionis cordis habent^[2].

Tensione pendentes canales natrii

Tensione pendentes canales natrii ex subunitatibus et singulari alpha et altera sive duabus beta consistunt. Subunitas alpha canalis ipse format, at beta modulationis functionis necesse videtur.

Structura

Subunitas Alpha

Subunitates alpha designantur Na_V. Adhuc decem formae descriptae sunt, Na_V1.1 ad Na_V1.9 atque Na_x.

Mutationes subunitatum alpha cum morbis comitialibus, syndroma intervalli QT longi, paralysis hyperkaliaemica periodica concidant.

Nomen geni	subunitatis α	loco	Annotatio
SCN1A	Na_V1.1	neuronibus, cardiomyocytis	mutatio genetica sociata cum syndroma Dravet, syndroma West
SCN2A	Na_V1.2	neuronibus
SCN3A	Na_V1.3	neuronibus, cardiomyocytis
SCN4A	Na_V1.4	musculis sceletalis
SCN5A	Na_V1.5	neuronibus	mutatio genetica sociata cum syndroma Brugada
SCN7A	Na_x	neuronibus
SCN8A	Na_V1.6	neuronibus, musculis levibus	mutatio genetica sociata cum morbis comitialibus
SCN9A	Na_V1.7
SCN10A	Na_V1.8
SCN11A	Na_V1.9

Subunitas beta

Beta subunitates cum abbreviatura Na_V-ß simul et adhaesionis cellularis et modulationum functionis subunitatis alpha necesse sunt. Nomen genorum sunt SCN1B isoformae a, SCN1B isoformae b, SCN2B, SCN3B, SCN4B^[3]. Duae variantes subunitatis beta notae sunt, Na_V-ß1 enim et Na_V-ß1B. Hae variantes causae nonnullorum morborum sunt:

In cardiomyoctis canales tensione pendentes natrii propagationem contractionum intra cor perficiunt. Mutationes geni SCN1B syndroma Brugada^[4] (E87Q) perficere potest^[5]. Praeterea syndroma intervalli QT longi mutatione genetica oritur^[6]. Fibrillatio atrialis familiaris interdum mutationem geni SCN4B ostendit^[7].

Nomen geni	subunitatis ß	cum subunitate α	loco
SCN1B isoformae a	Na_V-ß1A	Na_V1.1 — Na_V1.7	neuronibus, musculis striatis, corde, cellulae gliales
SCN1B isoformae b	Na_V-ß1B	Na_V1.1 — Na_V1.7	neuronibus, musculis striatis, corde, cellulae gliales
SCN2B	Na_V-ß2	Na_V1.1, Na_V1.2, Na_V1.5 — Na_V1.7	neuronibus, corde, cellulae gliales
SCN3B	Na_V-ß3	Na_V1.1 — Na_V1.3, Na_V1.5	neuronibus, renibus
SCN4B	Na_V-ß4	Na_V1.1, Na_V1.2, Na_V1.5	corde, musculis striatis, neuronibus

Functio

Tres statuum canalium tensione pendentium natrii sunt, clausus enim et apertus et non activus.

Inhibitores canalium natrii

Inhibitores extracellulares sunt Saxitoxinum (STX), Neosaxitoxinum (NSTX), Tetrodotoxinum (TTX). Inhibitores intracellulares sunt Lidocainum, substantiae antiarrhythmicae classis I, variae substantiae anticonvulsivae ut Phenytoinum, Oxcarbazepinum. Lamotriginum quoque est inhibitor canalium natrii.

Nexus interni

Notae

↑ Yang Y, Mis MA, Estacion M, Dib-Hajj SD, Waxman SG (2018). "Na_V1.7 as a Pharmacogenomic Target for Pain: Moving Toward Precision Medicine". Trends Pharmacol Sci 39 (3): 258-75
↑ Remme CA (2013). Cardiac sodium channelopathy associated with SCN5A mutations: electrophysiological, molecular and genetic aspects. 591. pp. 4099–116
↑ Bouza AA1, Isom LL (2018). "Voltage-Gated Sodium Channel β Subunits and Their Related Diseases". Handb Exp Pharmacol 246: 423-50
↑ Watanabe H, Koopmann TT, Le Scouarnec S, Yang T, Ingram CR, Schott JJ, Demolombe S, Probst V, Anselme F, Escande D, Wiesfeld AC, Pfeufer A, Kääb S, Wichmann HE, Hasdemir C, Aizawa Y, Wilde AA, Roden DM, Bezzina CR (2008). "Sodium channel β1 subunit mutations associated with Brugada syndrome and cardiac conduction disease in humans". J Clin Invest 118 (6): 2260-8
↑ Edokobi N1, Isom LL (2018). "Voltage-Gated Sodium Channel β1/β1B Subunits Regulate Cardiac Physiology and Pathophysiology". Front Physiol 9: 351
↑ Riuró H, Campuzano O, Arbelo E, Iglesias A, Batlle M, Pérez-Villa F, Brugada J, Pérez GJ, Scornik FS, Brugada R (2014). "A missense mutation in the sodium channel β1b subunit reveals SCN1B as a susceptibility gene underlying long QT syndrome". Heart Rhythm 11 (7): 1202-9
↑ Li RG, Wang Q, Xu YJ, Zhang M, Qu XK, Liu X, Fang WY, Yang YQ (2013). "Mutations of the SCN4B-encoded sodium channel β4 subunit in familial atrial fibrillation". Int J Mol Med 32 (1): 144-50

[1] Yang Y, Mis MA, Estacion M, Dib-Hajj SD, Waxman SG (2018). "Na_V1.7 as a Pharmacogenomic Target for Pain: Moving Toward Precision Medicine". Trends Pharmacol Sci 39 (3): 258-75

[2] Remme CA (2013). Cardiac sodium channelopathy associated with SCN5A mutations: electrophysiological, molecular and genetic aspects. 591. pp. 4099–116

[3] Bouza AA1, Isom LL (2018). "Voltage-Gated Sodium Channel β Subunits and Their Related Diseases". Handb Exp Pharmacol 246: 423-50

[4] Watanabe H, Koopmann TT, Le Scouarnec S, Yang T, Ingram CR, Schott JJ, Demolombe S, Probst V, Anselme F, Escande D, Wiesfeld AC, Pfeufer A, Kääb S, Wichmann HE, Hasdemir C, Aizawa Y, Wilde AA, Roden DM, Bezzina CR (2008). "Sodium channel β1 subunit mutations associated with Brugada syndrome and cardiac conduction disease in humans". J Clin Invest 118 (6): 2260-8

[5] Edokobi N1, Isom LL (2018). "Voltage-Gated Sodium Channel β1/β1B Subunits Regulate Cardiac Physiology and Pathophysiology". Front Physiol 9: 351

[6] Riuró H, Campuzano O, Arbelo E, Iglesias A, Batlle M, Pérez-Villa F, Brugada J, Pérez GJ, Scornik FS, Brugada R (2014). "A missense mutation in the sodium channel β1b subunit reveals SCN1B as a susceptibility gene underlying long QT syndrome". Heart Rhythm 11 (7): 1202-9

[7] Li RG, Wang Q, Xu YJ, Zhang M, Qu XK, Liu X, Fang WY, Yang YQ (2013). "Mutations of the SCN4B-encoded sodium channel β4 subunit in familial atrial fibrillation". Int J Mol Med 32 (1): 144-50

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