Oculocutaneous albinism is the lack of color in an individual's skin, hair, and eyes. It is a condition that exists from birth. This is The Importance of Ion Channels: An Analysis of Long QT Syndrome. ion channels (Crotti et al., 2008). There are two particular variants of long QT syndrome; one is called Jervell and Lange-Nielsen (J-LN) syndrome, which is associated with deafness, and the other has been called Romano-Ward (RW) syndrome, in which there is no connection with deafness (Crotti et al., 2008). Romano-Ward syndrome is known to be the most common type of LQTS and is autosomal dominant (Russell et al., 1996), while Jervell and Lange-Nielsen syndrome is less common and is autosomal recessive (Crotti et al., 1996). al., 2008). ).Gene(s) responsible for or involved in the disorder The research community has divided long QT syndrome by types, based on the different mutations in four of the cardiac ion channel genes, KVLQT1, HERG, SCN5A and KCNE1 (Zareba et al., 1998). Mutations within these voltage-gated ion channels ultimately disrupt normal nerve impulses that take place in myocardial cells. Sodium and potassium channels play a key role during action potentials, because it is through these channels that their respective ions can enter and leave the cell to generate electrical excitation or inhibition. Such channels are composed of protein subunits, and damage within even one subunit can alter the overall function of the action potential, which will alter...... middle of article. ..... ed. Philadelphia, PA: Saunders Elsevier; 2007: 52. Priori, S., Napolitano, C., Schwartz, P., (1999). Low penetrance in long qt syndrome. Circulation 99, 529-533. Russell, M., Dick, M., Collins, F., Brody, L,. (1996). KVLQT1 mutations in three families with familial or sporadic long QT syndrome. Human Molecular Genetics 5, 1319-1324. Westenskow, P., Splawski, I., Timothy, K., Keating, M., Sanguinetti, M., (2004). Compound mutations: a common cause of severe long QT syndrome. Circulation 109, 1834-1841. Zareba, W., Moss, A., Schwartz, P., Vincent, M., Robinson, J., Priori, S., Benhorin, J., Locati, E., Towbin, J., Keating, M. , Lehmann, M., Hall, J., Andrews, M., Napolitano, C., Timothy, K., Zhang, L., Medina, A., MacCluer, J., (1998). Influence of genotype on the clinical course of long QT syndrome. The New England Journal of Medicine 339, 960-965.