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Gene and Protein Information  |
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| Species | TM | P Loops | AA | Chromosomal Location | Gene Symbol | Gene Name | Reference |
| Human | 24 | 1 | 1836 | 17q23.3 | SCN4A | sodium voltage-gated channel alpha subunit 4 | 2,11,67 |
| Mouse | 24 | 1 | 1841 | 11 68.91 cM | Scn4a | sodium channel, voltage-gated, type IV, alpha | 1,72 |
| Rat | 24 | 1 | 1840 | 10q32.1 | Scn4a | sodium voltage-gated channel alpha subunit 4 | 1,60 |
| Previous and Unofficial Names |
| μ1 | SkM1 | HYKPP | HYPP | NCHVS | microI | mu-1 | sodium channel protein skeletal muscle subunit alpha | sodium channel protein type 4 subunit alpha | mH2 | sodium channel, voltage-gated, type IV, alpha subunit | sodium channel, voltage gated, type IV alpha subunit | sodium channel |
| Database Links |
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| Alphafold | P35499 (Hs), Q9ER60 (Mm), P15390 (Rn) |
| ChEMBL Target | CHEMBL2072 (Hs), CHEMBL3616353 (Mm), CHEMBL3509 (Rn) |
| DrugBank Target | P35499 (Hs) |
| Ensembl Gene | ENSG00000007314 (Hs), ENSMUSG00000001027 (Mm), ENSRNOG00000012134 (Rn) |
| Entrez Gene | 6329 (Hs), 110880 (Mm), 25722 (Rn) |
| Human Protein Atlas | ENSG00000007314 (Hs) |
| KEGG Gene | hsa:6329 (Hs), mmu:110880 (Mm), rno:25722 (Rn) |
| OMIM | 603967 (Hs) |
| Orphanet | ORPHA118507 (Hs) |
| Pharos | P35499 (Hs) |
| RefSeq Nucleotide | NM_000334 (Hs), NM_133199 (Mm), NM_013178 (Rn) |
| RefSeq Protein | NP_000325 (Hs), NP_573462 (Mm), NP_037310 (Rn) |
| UniProtKB | P35499 (Hs), Q9ER60 (Mm), P15390 (Rn) |
| Wikipedia | SCN4A (Hs) |
| Selected 3D Structures |
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| Associated Proteins |
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| Functional Characteristics |
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| Activation V0.5 = -30 mV. Fast inactivation (0.6 ms) | |
| Ion Selectivity and Conductance |
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| Voltage Dependence |
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Download all structure-activity data for this target as a CSV file 
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| Key to terms and symbols | View all chemical structures | Click column headers to sort | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
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| Key to terms and symbols | View all chemical structures | Click column headers to sort | ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
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| Tissue Distribution
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| Physiological Functions
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| Physiological Functions Comments | ||||||||
| The stated role of Nv1.4 has been reported for mamalian skeletal muscles [60-61]. | ||||||||
| Phenotypes, Alleles and Disease Models
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| Clinically-Relevant Mutations and Pathophysiology
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| The majority of Nav1.4 mutations in these diseases alter the inactivation properties of the channel leading to susceptability to periods of hyperactivity (causing myotonia) or inactivation (causing paralysis). | ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
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38. McKerrall SJ, Nguyen T, Lai KW, Bergeron P, Deng L, DiPasquale A, Chang JH, Chen J, Chernov-Rogan T, Hackos DH et al.. (2019) Structure- and Ligand-Based Discovery of Chromane Arylsulfonamide Nav1.7 Inhibitors for the Treatment of Chronic Pain. J Med Chem, 62 (8): 4091-4109. [PMID:30943032]
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57. Stunnenberg BC, Ginjaar HB, Trip J, Faber CG, van Engelen BG, Drost G. (2010) Isolated eyelid closure myotonia in two families with sodium channel myotonia. Neurogenetics, 11 (2): 257-60. [PMID:19876661]
58. Sun W, Cohen SA, Barchi RL. (1995) Localization of epitopes for monoclonal antibodies directed against the adult rat skeletal muscle sodium channel (rSkM1) using polymerase chain reaction, fusion proteins, and western blotting. Anal Biochem, 226 (1): 188-91. [PMID:7785772]
59. Tanaka JC, Eccleston JF, Barchi RL. (1983) Cation selectivity characteristics of the reconstituted voltage-dependent sodium channel purified from rat skeletal muscle sarcolemma. J Biol Chem, 258 (12): 7519-26. [PMID:6305944]
60. Trimmer JS, Cooperman SS, Agnew WS, Mandel G. (1990) Regulation of muscle sodium channel transcripts during development and in response to denervation. Dev Biol, 142 (2): 360-7. [PMID:2175278]
61. Trimmer JS, Cooperman SS, Tomiko SA, Zhou JY, Crean SM, Boyle MB, Kallen RG, Sheng ZH, Barchi RL, Sigworth FJ et al.. (1989) Primary structure and functional expression of a mammalian skeletal muscle sodium channel. Neuron, 3 (1): 33-49. [PMID:2559760]
62. Tsujino A, Maertens C, Ohno K, Shen XM, Fukuda T, Harper CM, Cannon SC, Engel AG. (2003) Myasthenic syndrome caused by mutation of the SCN4A sodium channel. Proc Natl Acad Sci USA, 100 (12): 7377-82. [PMID:12766226]
63. Vicart S, Sternberg D, Fournier E, Ochsner F, Laforet P, Kuntzer T, Eymard B, Hainque B, Fontaine B. (2004) New mutations of SCN4A cause a potassium-sensitive normokalemic periodic paralysis. Neurology, 63 (11): 2120-7. [PMID:15596759]
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