- Peptide (C)TASEHSREPSAAGRLSD, corresponding to amino acid residues 465-481 of rat NaV1.1 (Accession P04774). Intracellular loop between domains I and II.
- Rat brain membranes (1:200).
- Western blot analysis of rat brain membranes:1. Anti-SCN1A (NaV1.1) Antibody (#ASC-001), (1:200).
2. Anti-SCN1A (NaV1.1) Antibody, preincubated with SCN1A/Nav1.1 Blocking Peptide (#BLP-SC001).
- Mouse cardiac myocytes (1:500) (Malhotra, J.D. et al. (2001) Circulation 103, 1303.).
- Rat brain sections.
Human brain (1:400) (Wang, W. et al. (2011) Brain Res. 1389, 61.).
- Rat DRG primary culture (fixed and permeabilized) (1:200).
Voltage-gated sodium channels (NaV) are essential for the generation of action potentials and for cell excitability1. NaV channels are activated in response to depolarization and selectively allow the flow of Na+ ions. To date, nine NaV α subunits have been cloned and named NaV1.1-NaV1.94-5. The NaV channels are classified into two groups according to their sensitivity to tetrodotoxin (TTX): TTX-sensitive (NaV1.1, NaV1.2, NaV1.3, NaV1.4, NaV1.6 and NaV1.7) and TTX-resistant (NaV1.5, NaV1.8 and NaV1.9)2-3.
Mammalian sodium channels are heterotrimers composed of a central, pore-forming α subunit and two auxiliary β subunits. The expression of the α subunit isoform is developmentally regulated and tissue specific. Na+ channels in the adult central nervous system and heart contain β1 through β4 subunits, whereas Na+ channels in adult skeletal muscle have only the β1 subunit6,7.
NaV1.1, also referred to as SCN1A, is a tetrodotoxin-sensitive channel and is broadly expressed in neurons7.
Mutations in NaV1.1 are associated with at least two forms of epilepsy. Gain-of-function missense mutations are a primary cause of generalized epilepsy with febrile seizures plus (GEFS+). Loss-of-function mutations cause severe myoclonic epilepsy of infancy (SMEI)8,9.