Nav1.5

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Sodium channel, voltage-gated, type V, alpha subunit

Solution structure of the Na_v1.5 inactivation gate.^[1]

Available structures

Ortholog search: PDBe, RCSB

List of PDB id codes
2KBI, 2L53, 4DCK, 4DJC

Identifiers

Symbols

SCN5A; CDCD2; CMD1E; CMPD2; HB1; HB2; HBBD; HH1; ICCD; IVF; LQT3; Nav1.5; PFHB1; SSS1; VF1

External IDs

OMIM: 600163 MGI: 98251 HomoloGene: 22738 IUPHAR: Na_v1.5 ChEMBL: 1980 GeneCards: SCN5A Gene

Gene Ontology
Molecular function	• voltage-gated sodium channel activity • protein binding • calmodulin binding • enzyme binding • ankyrin binding • ion channel binding • voltage-gated sodium channel activity involved in regulation of cardiac muscle cell action potential • scaffold protein binding
Cellular component	• voltage-gated sodium channel complex • endoplasmic reticulum • plasma membrane • caveola • cell surface • intercalated disc • integral to membrane • T-tubule • sarcolemma
Biological process	• regulation of heart rate • cardiac ventricle development • brainstem development • sodium ion transport • positive regulation of sodium ion transport • response to denervation involved in regulation of muscle adaptation • telencephalon development • cerebellum development • sodium ion transmembrane transport • odontogenesis of dentin-containing tooth • positive regulation of action potential • positive regulation of epithelial cell proliferation • membrane depolarization • cardiac muscle contraction • regulation of ventricular cardiac muscle cell membrane repolarization • regulation of atrial cardiac muscle cell membrane depolarization • regulation of atrial cardiac muscle cell membrane repolarization • regulation of ventricular cardiac muscle cell membrane depolarization • cellular response to calcium ion • regulation of cardiac muscle cell action potential involved in contraction • regulation of cardiac muscle cell contraction • regulation of ventricular cardiac muscle cell action potential • membrane depolarization involved in regulation of action potential • membrane depolarization involved in regulation of cardiac muscle cell action potential • membrane depolarization involved in regulation of SA node cell action potential • AV node cell to bundle of His cell communication • bundle of His cell to Purkinje myocyte communication • SA node cell to atrial cardiac muscle cell communication • regulation of heart rate by cardiac conduction
Sources: Amigo / QuickGO

Orthologs

Species

Human

Mouse

RefSeq (mRNA)

RefSeq (protein)

Location (UCSC)

Chr 3:
38.59 – 38.69 Mb

Chr 9:
119.48 – 119.58 Mb

PubMed search

The Na_v1.5 is a sodium ion channel protein that in humans is encoded by the SCN5A gene. Mutations in the gene are associated with long QT syndrome type 3 (LQT3), Brugada syndrome, primary cardiac conduction disease and idiopathic ventricular fibrillation.

The protein encoded by this gene is an integral membrane protein and tetrodotoxin-resistant voltage-gated sodium channel subunit. The encoded protein is found primarily in cardiac muscle and is responsible for the initial upstroke of the action potential in an electrocardiogram. Defects in this gene are a cause of long QT syndrome type 3 (LQT3), an autosomal dominant cardiac disease.^[2]

Interactions

Nav1.5 has been shown to interact with Syntrophin, alpha 1.^[3]

Nav1.5 interacts with ankyrin-G through a nine-amino acid ankyrin-G binding sequence.^[4]

References

↑ PDB 1byy; Rohl CA, Boeckman FA, Baker C, Scheuer T, Catterall WA, Klevit RE (January 1999). "Solution structure of the sodium channel inactivation gate". Biochemistry 38 (3): 855–61. doi:10.1021/bi9823380. PMID 9893979.
↑ "Entrez Gene: SCN5A sodium channel, voltage-gated, type V, alpha subunit".
↑ Gee, S H; Madhavan R, Levinson S R, Caldwell J H, Sealock R, Froehner S C (Jan 1998). "Interaction of muscle and brain sodium channels with multiple members of the syntrophin family of dystrophin-associated proteins". J. Neurosci. 18 (1): 128–37. PMID 9412493. Cite uses deprecated parameters (help)
↑ Mohler PJ, Rivolta I, Napolitano C, LeMaillet G, Lambert S, Priori SG, Bennett V (December 2004). "Nav1.5 E1053K mutation causing Brugada syndrome blocks binding to ankyrin-G and expression of Nav1.5 on the surface of cardiomyocytes". Proc. Natl. Acad. Sci. U.S.A. 101 (50): 17533–8. doi:10.1073/pnas.0403711101. PMC 536011. PMID 15579534.

External links

GeneReviews/NIH/NCBI/UW entry on Brugada syndrome
GeneReviews/NIH/NCBI/UW entry on Romano-Ward Syndrome
SCN5A protein, human at the US National Library of Medicine Medical Subject Headings (MeSH)

PDB gallery

1byy: SODIUM CHANNEL IIA INACTIVATION GATE

Membrane transport protein: ion channels (TC 1A)

Ca²⁺: Calcium channel

Ligand-gated	Inositol trisphosphate receptor 1 2 3 Ryanodine receptor 1 2 3

Voltage-gated	L-type/Ca_vα 1.1 1.2 1.3 1.4 N-type/Ca_vα2.2 P-type/Ca_vα 2.1 Q-type/Ca_vα2.1 R-type/Ca_vα2.3 T-type/Ca_vα 3.1 3.2 3.3 α₂δ-subunits 1 2 β-subunits β1 β2 β3 β4 γ-subunits γ1 γ2 γ3 γ4 Cation channels of sperm 1 2 3 4 Two-pore channel 1 2

Na⁺: Sodium channel

Constitutively active	Epithelial sodium channel α β γ δ

Proton-gated	Amiloride-sensitive cation channel 1 2 3 4

Voltage-gated	Na_vα 1.1 1.2 1.3 1.4 1.5 1.6 1.7 1.8 1.9 7A Na_vβ 1 2 3 4

K⁺: Potassium channel

Calcium-activated	BK channel α1 β1 β2 β3 β4 SK channel SK1 SK2 SK3 IK channel IK1 K_Ca 1.1 2.1 2.2 2.3 3.1 4.1 4.2 5.1

Inward-rectifier	K_ATP K_ir 1.1 2.1 2.2 2.3 2.4 2.6 GIRK/K_ir 3.1 3.2 3.3 3.4 K_ir 4.1 4.2 5.1 6.1 6.2 7.1

Tandem pore domain	K2P 1 2 3 4 5 6 7 9 10 12 13 15 16 17 18

Voltage-gated	K_vα1-6 1.1 1.2 1.3 1.4 1.5 1.6 1.7 1.8 2.1 2.2 3.1 3.2 3.3 3.4 4.1 4.2 4.3 5.1 6.1 6.2 6.3 6.4 K_vα7-12 7.1 7.2 7.3 7.4 7.5 8.1 8.2 9.1 9.2 9.3 10.1 10.2 11.1/hERG 11.2 11.3 12.1 12.2 12.3 K_vβ 1 2 3 KCNIP 1 2 3 4 minK/ISK minK/ISK-like MiRP 1 2 3 Shaker gene

Miscellaneous

Cl^-: Chloride channel	ANO1 Bestrophin 1 2 CFTR CLCA 1 2 3 4 CLCN 1 2 3 4 5 6 7 KA KB CLIC 1 2 3 4 5 6 L1 CLNS 1A 1B

H⁺: Proton channel	HVCN1

M⁺: CNG cation channel	α 1 2 3 4 β 1 2 3 HCN FC 1 2 3 4

M⁺: TRP cation channel	TRPA (1) TRPC 1 2 3 4 4AP 5 6 7 TRPM 1 2 3 4 5 6 7 8 TRPML 1 2 3 TRPN TRPP 1 2 TRPV 1 2 3 4 5 6

H₂O (+ solutes): Porin	Aquaporin 0 1 2 3 4 5 6 7 8 9 Voltage-dependent anion channel 1 2 3 General bacterial porin family

Cytoplasm: Gap junction	Connexin: A GJA1 GJA3 GJA4 GJA5 GJA8 GJA9 GJA10 B GJB1 GJB2 GJB3 GJB4 GJB5 GJB6 GJB7 C GJC1 GJC2 GJC3 D GJD2 GJD3 GJD4) Innexin

By gating mechanism

Ion channel class	Ligand-gated Light-gated Voltage-gated Stretch-activated

see also disorders
B memb: cead, trns (1A, 1C, 1F, 2A, 3A1, 3A2-3, 3D), other

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Nav1.5

Interactions

See also

References

Further reading

External links