Antigen: fusion protein amino acids 1,507-1,733 · Clone designation: S-57-46 · Host: mouse · Isotype: IgG2b · Application(s): WB, IHC, ICC, and IF · Ion channels are integral membrane proteins that help establish and control the small voltage gradient across the plasma membrane of living cells by allowing the flow of ions down their electrochemical gradient.¹ They are present in the membranes that surround all biological cells because their main function is to regulate the flow of ions across this membrane. Whereas some ion channels permit the passage of ions based on charge, others conduct based on a ionic species, such as sodium or potassium. Furthermore, in some ion channels, the passage is governed by a gate which is controlled by chemical or electrical signals, temperature, or mechanical forces. There are a few main classifications of gated ion channels. There are voltage-gated ion channels, ligand-gated, other gating systems, and finally those that are classified differently, having more exotic characteristics. The first are voltage-gated ion channels which open and close in response to membrane potential. These are then seperated into sodium, calcium, potassium, proton, transient receptor, and cyclic nucleotide-gated channels, each of which is responsible for a unique role. Ligand-gated ion channels are also known as ionotropic receptors, and they open in response to specific ligand molecules binding to the extracellular domain of the receptor protein. The other gated classifications include activation and inactivation by second messengers, inward-rectifier potassium channels, calcium-activated potassium channels, two-pore-domain potassium channels, light-gated channels, mechano-sensitive ion channels, and cyclic nucleotide-gated channels. Finally, the other classifications are based on less normal characteristics such as two-pore channels, and transient receptor potential channels.² Specifically, Cav1.2 is a cardiac L-type calcium channel and is important for excitation and contraction of the heart.³ It may be associated with a variant of Long QT syndrome called Timothy’s syndrome^4,5 and also with Brugada syndrome. Some references also suggest it is related to bipolar disease as well.⁵

1 Hille, B. Ion Channels of Excitable Membranes. 3rd (2001).

2 . What are ion channels? (2004).

3 Splawski, I., Timothy, K.W., Sharpe, L.M., et al. Cav1.2 calcium channel dysfunction causes a multisystem disorder including arrhythmia and autism. Cell 119 19-31 (2004).

4 Krey, J.F., and Dolmetsch, R. The Timothy Syndrome mutation in Cav1.2 causes dendritic retraction through calcium-independent activation of the rho A pathway. Biophys J 96(3) 221a-222a (2009).

5 Crotti, L., Celano, G., Dagradi, F., et al. Congenital long QT syndrome. Orphanet J Rare Dis 3 18-33 (2008).

Hille, B. Ion Channels of Excitable Membranes. 3rd (2001).

. What are ion channels? (2004).

Splawski, I., Timothy, K.W., Sharpe, L.M., et al. Cav1.2 calcium channel dysfunction causes a multisystem disorder including arrhythmia and autism. Cell 119 19-31 (2004).

Krey, J.F., and Dolmetsch, R. The Timothy Syndrome mutation in Cav1.2 causes dendritic retraction through calcium-independent activation of the rho A pathway. Biophys J 96(3) 221a-222a (2009).

Crotti, L., Celano, G., Dagradi, F., et al. Congenital long QT syndrome. Orphanet J Rare Dis 3 18-33 (2008).

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