By Kirk L. Hamilton, Daniel C Devor
This ebook sheds new mild at the body structure, molecular biology and pathophysiology of epithelial ion channels and transporters. It combines the fundamental mobile types and services via a compelling medical viewpoint, addressing points from the laboratory bench to the bedside. the person chapters, written by way of best scientists and clinicians, discover particular ion channels and transporters positioned within the epithelial tissues of the kidney, gut, pancreas and breathing tract, all of which play a very important half in preserving homeostasis. extra themes contain the basics of epithelial delivery; mathematical modeling of ion delivery; cellphone quantity rules; membrane protein folding and trafficking; transepithelial delivery capabilities; and finally, a dialogue of delivery proteins as strength pharmacological objectives with a spotlight at the pharmacology of potassium channels.
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Additional resources for Ion Channels and Transporters of Epithelia in Health and Disease
Mutations in SLC26A4 are associated with deafness and goiter, a condition ﬁrst described by Vaughan Pendred (1896). The gene associated with Pendred syndrome was speculated initially to be a sulfate transporter based on sequence homology (Everett et al. 1997), but it is now known to function as a ClÀ/ HCO3À exchanger. D. C. Devor (Choi et al. 2011; Li et al. 2013) that SLC26A4 expression is required during fetal development in the endolymphatic sac, but not in the cochlea or vestibular sensory organs, for hearing and balance to develop, and that it is not required during postnatal life for hearing to be maintained.
Finally, Flores et al. 1 knockout mice and demonstrated that Ca2+-mediated ClÀ secretion was completely eliminated in both distal colon and small intestinal epithelium, which resulted in a marked reduction in water content in the stools. 1 is the basolateral membrane K+ channel activated by Ca2+-mediated agonists as a means of maintaining transepithelial ClÀ secretion across intestinal epithelia. 1 in epithelial transport, the reader is directed to Chap. 20 of this volume. 1)/KCNE3 (Mirp2) Is the cAMPActivated Basolateral Membrane K+ Channel The demonstration that 293B blocked cAMP-mediated transepithelial ClÀ secretion with high afﬁnity by inhibiting the basolateral membrane K+ conductance, with no effect on the apical membrane ClÀ conductance, was a critical turning point in the identiﬁcation of this K+ channel (Lohrmann et al.
The technique was enhanced to include electrical measurements and cable theory was introduced as a means to assess the outcomes (Burg et al. 1968; Lutz et al. 1973). In subsequent studies, the authors reported collecting duct transepithelial resistance of nearly 1 kΩ cm2 (Helman et al. 1971), approximately 40 times greater than the resistance reported for proximal convoluted tubules (Giebisch et al. 1964). The technique was used extensively to deﬁne ion transport characteristics in various nephron segments, including active ClÀ absorption in the thick ascending limb of Henle’s loop, and to localize the action of furosemide (Burg et al.