Abstract
This part will focus on the carriers that are involved in the transport of electrolytes and acid-base equivalents, such Na+, K+, CL− H+, or HCO3 −. Often both functions are intricately linked not only by directly coupling the transport of. acid-base equivalents to that of electrolytes (i.e., Na+/H+ or Cl−/HCO3 − exchangers, Na+/HCO3 − cotransport) but also because both influence whole-cell function or body fluid composition and are often regulated by the same hormonal systems. Thus it is not surprising that disorders primarily affecting either electrolyte or acid—base transport often affect cellular or whole-body electrolyte and acid—base homeostasis. In contrast to the transport of acid—base equivalents, however, the transport of electrolytes is achieved by the concerted action of transporters and a variety of ion channels.
This is a preview of subscription content, log in via an institution to check access.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
Preview
Unable to display preview. Download preview PDF.
References
Agre, P., King, L.S., Yasui, M., Guggino, W.B., Ottersen, O.P., Fujiyoshi, Y., et al. (2002).Aquaporin Water Channels—From Atomic Structure to Clinical Medicine. J. Physiol., 542, 3–16.
Birkenhager, R., Otto, E., Schurmann, M.J., Vollmer, M., Ruf, E.M., Maier-Lutz, I., et al. (2001). Mutation of BSND Causes Bartter Syndrome with Sensorineural Deafness and Kidney Failure. Nat. Genet., 29, 310–314.
Burckhardt, G. and Wolff, N.A. (2000 ). Structure of Renal Organic Anion and Cation Transporters. Am. J. Physiol. Renal Physio l., 278. F853–866.
Cruz, D.N., Simon, D.B., Nelson-Williams, C., Farhi, A., Finberg, K., Burleson, L., et al. (2001). Mutations in the Na-Cl Cotransporter Reduce Blood Pressure in Humans. Hypertension, 37. 1458–1464.
De Jong, J.C., Willems, P.H., Mooren, F.J., Van Den Heuvel, L.P., Knoers, N.V., and Bindels, R.J. (2003). The Structural Unit of the Thiazide-Sensitive Nacl Cotransporter (NCC) is a Homodimer. J. Biol. Chem., 278, 24302–24307.
Eladari, D., Cheval, L., Quentin, F., Bertrand, O., Mouro, I., Cherif-Zahar, B., et al. (2002). Expression of RhCG, a New Putative NH3/NH4 + Transporter, Along the Rat Nephron. J. Am. Soc. Nephrol., 13, 1999–2008.
Enomoto, A., Kimura, H., Chairoungdua, A., Shigeta, Y., Jutabha, P., Cha, S.H., et al. (2002). Molecular Identification of a Renal Urate Anion Exchanger that Regulates Blood Urate Levels. Nature, 417, 447–452.
Estevez, R., Boettger, T., Stein, V., Birkenhager, R., Otto, E., Hildebrandt, F., et al. (2001). Barttin is a Cl− Channel Beta-Subunit Crucial for Renal Cl− Reabsorption and Inner Ear K+ Secretion. Nature, 414, 558–561.
Frattini, A., Orchard, P.J., Sobacchi, C., Giliani, S., Abinun, M., Mattsson, J.P., et al. (2000). Defects in TCIRG1 subunit of the vacuolar proton pump are responsible for a subset of human autosomal recessive osteopetrosis. Nat. Genet., 25. 343–346.
Hamrn, L.L. and Alpern, R.J. (2000). Cellular mechanisms of renal tubular acidification. In D.W, Seldin and G, Gebisch (eds), The Kidney: Physiology and Pathophysiology (3rd ed.). Philadelphia, PA: Lippincott-Williams & Wilkins. pp. 1935–1979.
Inui, K.I., Masuda, S. and Saito, H. (2000). Cellular and Molecular Aspects of Drug Transport in the Kidney. Kidney Int., 58, 944–958.
Karet, F.E., Finberg, K.E., Nelson, R.D., Nayir, A., Mocan, H., Sanjad, S.A., et al. (1999). Mutations in the Gene Encoding B1 Subunit of H+-ATPase Cause Renal Tubular Acidosis with Sensorineural Deafness. Nat. Genet., 21, 84–90.
Koepsell, H. (1998). Organic Cation Transporters in Intestine, Kidney, Liver, and Brain. Annu. Rev. Physiol., 60, 243–266.
Kriz, W. and Kaissling, B. (2000). Structural organization of the mammalian kidney. In D, Seldin and G, Gebisch (eds), The Kidney: Physiology and Pathopyh siology (3rd edn), Philadelphia, PA: Lippincott-Williams & Wilkins. pp. 587–654.
Lifton, R.P., Gharavi, A.G. and Geller, D.S. (2000). Molecular Mechanisms of Human Hypertension. Cell, 104, 545–556.
Marini, A.M., Matassi, G., Raynal, V., Andre, B., Cartron, J.P., and Cherif-Zahar, B. The Human Rhesus-Associated RhAG Protein and a Kidney Homologue Promote Ammonium Transport in Yeast. Nat Genet., 26, 341–344.
Martin, M.G., Turk, E., Lostao, M.P., Kerner, C., and Wright, E.M. (1996). Defects in Na+/Glucose Cotransporter (SGLT1) Trafficking and Function Cause Glucose-Galactose Malabsorption. Nat. Genet., 12, 216–220.
Nishi, T. and Forgac, M. (2002). The vacuolar (H+)-ATPases — Nature’s Most Versatile Proton Pumps. Nat. Rev. Mol. Cell. Biol., 3, 94–103.
Quentin, F., Eladari, D., Cheval, L., Lopez, C., Goossens, D., Colin, Y., et al. (2003). RhBG and RhCG, the Putative Ammonia Transporters, Are Expressed in the Same Cells in the Distal Nephron. J. Am. Soc. Nephrol., 14, 545–554.
Royaux, I.E., Wall, S.M., Karniski, L.P., Everett, L.A., Suzuki, K., Knepper, M.A., et al. (2001). Pendrin, Encoded by the Pendred Syndrome Gene, Resides in the Apical Region of Renal Intercalated Cells and Mediates Bicarbonate Secretion. Proc. Natl. Acad. Sci. USA, 98, 4221–4226.
Russel, F.G., Masereeuw, R., and van Aubel, R.A. Molecular Aspects of Renal Anionic Drug Transport. Annu. Rev. Physiol., 64, 563–594.
Sands, J.M. (2003). Mammalian Urea Transporters. Annu. Rev. Physiol., 65, 543–566.
Simon, D.B., Karet, F.E., Hamdan, J.M., DiPietro, A., Sanjad, S.A., and Lifton, R.P. (1996a). Bartter’s Syndrome, Hypokalaemic Alkalosis with Hypercalciuria, is caused by Mutations in the Na-K-2Cl cotransporter NKCC2. Nat. Genet., 13, 183–188.
Simon, D.B., Karet, F.E., Rodriguez-Soriano, J., Hamdan, J.H., DiPietro, A., Trachtrnan, H., et al. (1996b). Genetic Heterogeneity of Bartter’s Syndrome Revealed by Mutations in the K+ Channel, ROMK. Nat. Genet., 14, 152–156.
Simon, D.B., Nelson-Williams, C., Bia, M.J., Ellison, D., Karet, F.E., Molina, A.M., et al. (1996c). Gitelrnan’s Variant of Bartter’ s Syndrome, Inherited Hypokalaemic Alkalosis, is caused by Mutations in the Thiazide-Sensitive Na-Cl Cotransporter. Nat. Genet., 12, 24–30.
Simon, D.B., Bindra, R.S., Mansfield, T.A., Nelson-Williams, C., Mendonca, E., Stone, R., et al. (1997). Mutations in the Chloride Channel Gene, CLCNKB, Cause Banter’s Syndrome Type III. Nat. Genet., 17, 171–178.
Sly, W.S. and Shah, G.N. (2001). The Carbonic Anhydrase II Deficiency Syndrome: Osteopetrosis with Renal Tubular Acidosis and Cerebral Calcification. In C.R, Scriver, A.L, Baudet, W.S, Sly and D, Valle (eds), The Metabolic and Molecular Bases of Inherited Disease, New York: McGraw-Hill, pp. 5331–5343.
Smith, A.N., Skaug, J., Choate, K.A., Nayir, A., Bakkaloglu, A., Ozen, S., et al. (2000). Mutations in ATP6N1B, Encoding a New Kidney Vacuolar Proton Pump 116-kD Subunit, Cause Recessive Distal Renal Tubular Acidosis with Preserved Hearing. Nat. Genet., 26, 71–75.
Sweet, D.H., Bush, K.T., and Nigam, S.K. (2001). The Organic Anion Transporter Family: From Physiology to Ontogeny and the Clinic. Am. J. Physiol. Renal Physiol., 281, F197–F205.
van den Heuvel, L.P., Assink, K., Willemsen, M., and Monnens, L. (2002). Autosomal Recessive Renal Glucosuria Attributable to a Mutation in the Sodium Glucose Cotransporter (SGLT2). Hum. Genet., 111, 544–547.
Verrey, F., Hummler, E., Schild, L., and Rossier, B. (2000). Control of Na+ Transport by Aldosterone. In D.W, Seldin and G, Griebisch (eds), The Kidney: Physiology and Pathophysiology (3rd edn), Philadelphia, PA: Lippincott-Williams & Wilkins, pp. 1441–1471.
Wagner, C.A. and Geibel, J.P. (2002). Acid-Base Transport in the Collecting Duct. J. Nephrol., Suppl. 5, S112–S127.
Wilson, F.H., Disse-Nicodeme, S., Choate, K.A., Ishikawa, K., Nelson-Williams, C., Desitter, I., et al. (2001). Human Hypertension Caused by Mutations in WNK Kinascs. Science, 293, 1107–1112.
Wilson, F.H., Kahle, K.T., Sabath, E., Lalioti, M.D., Rapson, A.K., Hoover, R.S., et al. (2003). Molecular Pathogenesis of Inherited Hypertension with Hyper-kalemia: The Na-Cl Cotransporter is Inhibited by Wild-Type but not Mutant WNK4. Proc. Natl Acad. Sci. USA, 100, 680–684.
Woo, A.L., Noonan, W.T., Schultheis, P.J., Neumann, J.C., Manning, P.A., and Lorenz, J. Renal Function in NHE3-Deficient Mice with Transgenic Rescue of Small Intestinal Absorptive Defect. Am. J. Physiol. Renal Physiol., 284, F1190–F1198.
Yang, C.L., Angell, J., Mitchell, R. and Ellison, D.H. (2003). WNK Kinases Regulate Thiazide-Sensitive Na-Cl Cotransport. J. Clin. Lnvest., 111, 1039–1045.
Author information
Authors and Affiliations
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2003 Springer Science+Business Media New York
About this chapter
Cite this chapter
Wagner, C.A. (2003). Introduction. In: Bröer, S., Wagner, C.A. (eds) Membrane Transporter Diseases. Springer, Boston, MA. https://doi.org/10.1007/978-1-4419-9023-5_2
Download citation
DOI: https://doi.org/10.1007/978-1-4419-9023-5_2
Publisher Name: Springer, Boston, MA
Print ISBN: 978-1-4613-4761-3
Online ISBN: 978-1-4419-9023-5
eBook Packages: Springer Book Archive