Table 2.
Summary of the different types of β subunits associated with the different VGSC, and the related channelopathies associated with the mutations in the genes that encode them (modified from Patino and Isom, 2010).
| Gene | Chromosome | β subunit | α subunit | Expression | Channelopathies | Model | Reference |
|---|---|---|---|---|---|---|---|
| SCN1B | 19q13.1 | β1 | NaV1.1–NaV1.7 | Central and peripheral neurons, glia, skeletal, and cardiac muscles. |
Seizures and epileptic syndromes: febrile seizures, Dravet syndrome, temporal lobe epilepsy |
H H,M H |
Coward et al. (2001), Audenaert et al. (2003), Chen et al. (2004), Pertin et al. (2005, 2007), Scheffer et al. (2007), O’Malley et al. (2009), Orrico et al. (2009), Patino et al. (2009) |
| Traumatic nerve injury | H | ||||||
| SCN2B | 11q23 | β2 | NaV1.1, NaV1.2, NaV1.5–NaV1.7 | Central and Peripheral neurons, glia, cardiac muscle. |
Multiple sclerosis, Neuropathic pain (post-trauma) |
M M |
Coward et al. (2001), Pertin et al. (2005), Lopez-Santiago et al. (2006), O’Malley et al. (2009) |
| Inflammatory pain, traumatic nerve injury |
M H |
||||||
| SCN3B | 11q23.3 | β3 | NaV1.1–NaV1.3, NaV1.5 |
Central and peripheral neurons, adrenal gland, kidney |
Temporal epilepsy, Traumatic nerve injury |
H H |
Casula et al. (2004), van Gassen et al. (2009) |
| SCN4B | 11q23.3 | β4 | NaV1.1, Na 1.2, NaV1.5 | Central and peripheral neurons, glia, skeletal and cardiac muscles. |
Huntington’s disease | H,M | Oyama et al. (2006) |
H, human; M, mouse.