The classical tango is a dance seen as a a 2/4 or 4/4 rhythm where the partners dance inside a coordinated way, allowing active contact. linkage research of individuals with lengthy QT symptoms (LQTS1). Its gene item, Kv7.1 (also termed KvLQT1 or KCNQ1), is a voltage-gated potassium route -subunit, and its own expression was detected in a number of mammalian cells, including center, epithelia, and soft muscle (Shape ?(Shape1;1; Desk ?TableA1A1 in Appendix). Kv7.1 may assemble with different people from the KCNE category of regulatory -subunits to satisfy a number of physiological features. Open in another window Shape 1 Distribution of Kv7.1. Kv7.1 is expressed in a number of cells IL-10 throughout the body, including center, lung, internal ear, kidney, as well as the gastrointestinal system. In the center, Kv7.1 is mixed up in termination from the cardiac actions potential. The repolarizing potassium current, and mutations connected with cardiac arrhythmias (http://www.fsm.it/cardmoc/). Many of these mutations Fasudil HCl ic50 result in loss of route function leading to LQTS, a problem predisposing individuals to arrhythmia and cardiac unexpected loss of life. Besides its cardiac function, several lines of evidence suggest an important role of Kv7.1 and its accessory -subunit KCNE1 in the hearing process. In patients suffering from Jervell and Lange-Nielsen syndrome C the recessive form of inherited LQTS C cardiac arrhythmia is accompanied by profound bilateral deafness. Mutations in both and genes have been reported to cause this disorder (Jervell and Lange-Nielsen, 1957; Neyroud et al., 1997; Schulze-Bahr et al., 1997). In addition, targeted disruption of the gene in mice leads to deafness caused by morphological abnormalities of the inner ear (Lee et al., Fasudil HCl ic50 2000; Casimiro et al., 2001). Expression of Kv7.1 and KCNE1 has been detected in the marginal cells of the of the cochlea and the vestibular dark cells (Neyroud et al., 1997; Nicolas et al., 2001; Knipper et al., 2006; Hur et al., 2007). Both cell types are involved in the generation of the potassium-rich endolymph, and Kv7.1/KCNE1 channels have been suggested to be key mediators of this K+ secretion (Marcus and Shen, 1994; Shen et al., 1995; Wangemann, 1995; Wangemann et al., 1995; Sunose et al., 1997). In addition to the inner ear epithelium, Kv7.1 has been detected in a variety of other epithelial cell types, where it participates in secretory transduction. In the kidney, Kv7.1/KCNE1 channels seem to be located in the proximal tubule of the nephron (Sugimoto et al., 1990; Vallon et al., 2001), conducting a K+ current to counterbalance membrane depolarization induced by electrogenic Na+-coupled transport of glucose or amino acids (Vallon et al., 2001, 2005). The relevance of Kv7.1/KCNE1 channels for renal function is further underlined by the observation that KCNE1 knockout mice suffer from hypokalemia, urinary and fecal salt wasting, and volume depletion (Arrighi et al., 2001; Warth and Barhanin, 2002). Kv7.1 expression has also been detected in the small intestine and the colon (Schroeder et al., 2000; Dedek and Waldegger, 2001; Demolombe et al., 2001; Kunzelmann et al., 2001; Horikawa et al., 2005). In colonic crypt cells Kv7.1 is believed to assemble with another accessory -subunit, KCNE3, Fasudil HCl ic50 and to mediate a K+ conductance that provides the driving force for chloride secretion (Schroeder et al., 2000; Kunzelmann et al., 2001). Two further examples of Kv7.1 expression and function in chloride-secreting tissues are pancreatic acinar cells and airway epithelium (Kim and Greger, 1999; Kottgen et al., 1999; Mall et al., 2000; Demolombe et al., 2001; Grahammer et al., 2001b; Lee et al., 2004). In parietal cells of the stomach Kv7.1 coassembles with KCNE2 and participates in gastric acid secretion (Dedek and Waldegger, 2001; Demolombe et al., 2001; Grahammer et al., 2001a; Heitzmann et al., 2004). In KCNQ1 knockout mice gastric hyperplasia and profound hypochlorhydria have been observed, indicating the importance of Kv7.1 in normal stomach development and function (Lee et al., 2000). Kv7.1 expression has also been detected in the human thyroid gland, and it has been shown that mice lacking functional Kv7.1.
