Gene
scn1laa
- ID
- ZDB-GENE-040426-751
- Name
- sodium channel, voltage-gated, type I-like, alpha
- Symbol
- scn1laa Nomenclature History
- Previous Names
- Type
- protein_coding_gene
- Location
- Chr: 9 Mapping Details/Browsers
- Description
- Contributes to voltage-gated sodium channel activity. Predicted to be involved in several processes, including membrane depolarization during action potential; neuronal action potential; and sodium ion transmembrane transport. Predicted to act upstream of or within monoatomic ion transmembrane transport and sodium ion transport. Predicted to be located in plasma membrane. Predicted to be part of voltage-gated sodium channel complex. Predicted to be active in axon. Is expressed in heart; nervous system; and neural tube. Used to study Dravet syndrome. Human ortholog(s) of this gene implicated in Dravet syndrome; developmental and epileptic encephalopathy 6B; familial hemiplegic migraine 3; generalized epilepsy with febrile seizures plus; and generalized epilepsy with febrile seizures plus 2. Orthologous to several human genes including SCN7A (sodium voltage-gated channel alpha subunit 7).
- Genome Resources
- Note
- None
- Comparative Information
-
- All Expression Data
- 10 figures from 6 publications
- Cross-Species Comparison
- High Throughput Data
- Thisse Expression Data
-
- MGC:55600 (6 images)
Wild Type Expression Summary
Phenotype Summary
Mutations
| Allele | Type | Localization | Consequence | Mutagen | Supplier |
|---|---|---|---|---|---|
| la029626Tg | Transgenic insertion | Unknown | Unknown | DNA | |
| sa1674 | Allele with one point mutation | Unknown | Missense, Splice Site | ENU | |
| sa7636 | Allele with one point mutation | Unknown | Missense | ENU | |
| sa15331 | Allele with one point mutation | Unknown | Premature Stop | ENU | |
| sa21602 | Allele with one point mutation | Unknown | Missense, Splice Site | ENU | |
| sa21603 | Allele with one point mutation | Unknown | Splice Site | ENU | |
| sa41553 | Allele with one point mutation | Unknown | Premature Stop | ENU |
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Human Disease
| Disease Ontology Term | Multi-Species Data | OMIM Term | OMIM Phenotype ID |
|---|---|---|---|
| developmental and epileptic encephalopathy 6B | Alliance | Developmental and epileptic encephalopathy 6B, non-Dravet | 619317 |
| Dravet syndrome | Alliance | Dravet syndrome | 607208 |
| familial hemiplegic migraine 3 | Alliance | Migraine, familial hemiplegic, 3 | 609634 |
| generalized epilepsy with febrile seizures plus 2 | Alliance | Febrile seizures, familial, 3A | 604403 |
| generalized epilepsy with febrile seizures plus 2 | Alliance | Generalized epilepsy with febrile seizures plus, type 2 | 604403 |
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| Human Disease | Fish | Conditions | Citations |
|---|---|---|---|
| Dravet syndrome | scn1laasa1674/sa1674 (TL) | control | Griffin et al., 2017 |
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Domain, Family, and Site Summary
| Type | InterPro ID | Name |
|---|---|---|
| Domain | IPR005821 | Ion transport domain |
| Domain | IPR010526 | Sodium ion transport-associated domain |
| Domain | IPR024583 | Voltage-gated Na+ ion channel, cytoplasmic domain |
| Domain | IPR044564 | Voltage-gated sodium channel alpha subunit, inactivation gate |
| Family | IPR001696 | Voltage gated sodium channel, alpha subunit |
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Domain Details Per Protein
| Protein | Additional Resources | Length | Ion transport domain | Sodium ion transport-associated domain | Voltage-dependent channel domain superfamily | Voltage-gated cation channel calcium and sodium | Voltage-gated Na+ ion channel, cytoplasmic domain | Voltage gated sodium channel, alpha subunit | Voltage-gated sodium channel alpha subunit, inactivation gate |
|---|---|---|---|---|---|---|---|---|---|
| UniProtKB:Q20JQ9 | InterPro | 1955 | |||||||
| UniProtKB:A0A0R4IUM7 | InterPro | 1955 | |||||||
| UniProtKB:A0AB32TV97 | InterPro | 1832 |
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| Type | Name | Annotation Method | Has Havana Data | Length (nt) | Analysis |
|---|---|---|---|---|---|
| mRNA |
scn1laa-201
(1)
|
Ensembl | 1,053 nt | ||
| mRNA |
scn1laa-203
(1)
|
Ensembl | 6,421 nt | ||
| mRNA |
scn1laa-204
(1)
|
Ensembl | 1,255 nt | ||
| mRNA |
scn1laa-205
(1)
|
Ensembl | 2,501 nt | ||
| mRNA |
scn1laa-206
(1)
|
Ensembl | 5,868 nt |
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Interactions and Pathways
No data available
Plasmids
No data available
No data available
| Relationship | Marker Type | Marker | Accession Numbers | Citations |
|---|---|---|---|---|
| Contained in | BAC | CH211-66E19 | ZFIN Curated Data | |
| Contained in | BAC | CH211-120F14 | ZFIN Curated Data | |
| Contained in | Fosmid | CH1073-162M7 | ZFIN Curated Data | |
| Encodes | cDNA | MGC:55600 | ZFIN Curated Data | |
| Encodes | cDNA | MGC:158596 | ZFIN Curated Data | |
| Encodes | cDNA | MGC:165418 | ZFIN Curated Data |
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| Type | Accession # | Sequence | Length (nt/aa) | Analysis |
|---|---|---|---|---|
| RNA | RefSeq:NM_200132 (1) | 5868 nt | ||
| Genomic | GenBank:AL929294 (2) | 162474 nt | ||
| Polypeptide | UniProtKB:A0A0R4IUM7 (1) | 1955 aa |
- Osorio-Méndez, D., Miller, A., Begeman, I.J., Kurth, A., Hagle, R., Rolph, D., Dickson, A.L., Chen, C.H., Halloran, M., Poss, K.D., Kang, J. (2022) Voltage-gated sodium channel scn8a is required for innervation and regeneration of amputated adult zebrafish fins. Proceedings of the National Academy of Sciences of the United States of America. 119:e2200342119
- Weuring, W.J., Dilevska, I., Hoekman, J., van de Vondervoort, J., Koetsier, M., van 't Slot, R.H., Braun, K.P.J., Koeleman, B.P.C. (2021) CRISPRa-Mediated Upregulation of scn1laa During Early Development Causes Epileptiform Activity and dCas9-Associated Toxicity. The CRISPR journal. 4:575-582
- Haverinen, J., Hassinen, M., Korajoki, H., Vornanen, M. (2018) Cardiac voltage-gated sodium channel expression and electrophysiological characterization of the sodium current in the zebrafish (Danio rerio) ventricle. Progress in Biophysics and Molecular Biology. 138:59-68
- Bayés, À., Collins, M.O., Reig-Viader, R., Gou, G., Goulding, D., Izquierdo, A., Choudhary, J.S., Emes, R.D., Grant, S.G. (2017) Evolution of complexity in the zebrafish synapse proteome. Nature communications. 8:14613
- Gau, P., Curtright, A., Condon, L., Raible, D.W., Dhaka, A. (2017) An ancient neurotrophin receptor code; a single Runx/Cbfβ complex determines somatosensory neuron fate specification in zebrafish. PLoS Genetics. 13:e1006884
- Griffin, A., Hamling, K.R., Knupp, K., Hong, S., Lee, L.P., Baraban, S.C. (2017) Clemizole and modulators of serotonin signalling suppress seizures in Dravet syndrome. Brain : a journal of neurology. 140(3):669-683
- Baraban, S.C., Dinday, M.T., and Hortopan, G.A. (2013) Drug screening in Scn1a zebrafish mutant identifies clemizole as a potential Dravet syndrome treatment. Nature communications. 4:2410
- Varshney, G.K., Lu, J., Gildea, D., Huang, H., Pei, W., Yang, Z., Huang, S.C., Schoenfeld, D.S., Pho, N., Casero, D., Hirase, T., Mosbrook-Davis, D.M., Zhang, S., Jao, L.E., Zhang, B., Woods, I.G., Zimmerman, S., Schier, A.F., Wolfsberg, T., Pellegrini, M., Burgess, S.M., and Lin, S. (2013) A large-scale zebrafish gene knockout resource for the genome-wide study of gene function. Genome research. 23(4):727-735
- Won, Y.J., Ono, F., and Ikeda, S.R. (2012) Characterization of na(+) and ca(2+) channels in zebrafish dorsal root ganglion neurons. PLoS One. 7(8):e42602
- Chopra, S.S., Stroud, D.M., Watanabe, H., Bennett, J.S., Burns, C.G., Wells, K.S., Yang, T., Zhong, T.P., and Roden, D.M. (2010) Voltage-Gated Sodium Channels Are Required for Heart Development in Zebrafish. Circulation research. 106(8):1342-1350
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