Bicarbonate transport in cell physiology and disease

Biochemical Journal

Volumn 417, Issue 2, 2009, Pages 423-439

  Cordat, Emmanuelle ^a   Casey, Joseph R ^a  

^a UNIVERSITY OF ALBERTA   (Canada)

Author keywords

Bicarbonate transport; Cl HCO3 anion exchanger (AE); Na+ HCO3 co transporter (NBC); pH regulation; Solute carrier (SLC); Volume regulation

Indexed keywords

BICARBONATE TRANSPORT; CL-/HCO3 ANION EXCHANGER (AE); NA+/HCO3- CO-TRANSPORTER (NBC); PH REGULATION; SOLUTE CARRIER (SLC); VOLUME REGULATION;

ACOUSTIC EMISSIONS; BIOCHEMISTRY; CYTOLOGY; ION EXCHANGE RESINS; MAMMALS; NEGATIVE IONS; PHYSIOLOGY; PROTEINS; SODIUM; WASTE DISPOSAL;

CELL MEMBRANES;

ANION CHANNEL; BICARBONATE; BICARBONATE SODIUM COTRANSPORTER; BICARBONATE TRANSPORTER; BLOOD GROUP ANTIGEN; CARBON DIOXIDE; CARRIER PROTEIN; CARRIER PROTEINS AND BINDING PROTEINS; CHLORIDE BICARBONATE EXCHANGER; METAL TRANSPORTER ZIP8; METAL TRANSPORTER ZIP9; PENDRIN; SOLUTE CARRIER PROTEIN 26A3; SOLUTE CARRIER PROTEIN 26A6; SOLUTE CARRIER PROTEIN 39A; SOLUTE CARRIER PROTEIN 4A1; SOLUTE CARRIER PROTEIN 4A11; TRANSMEMBRANE CONDUCTANCE REGULATOR; UNCLASSIFIED DRUG; ACID;

ANION EXCHANGE; BICARBONATE TRANSPORT; BONE DEVELOPMENT; BRAIN FUNCTION; CARDIOVASCULAR FUNCTION; CELL MEMBRANE TRANSPORT; CEREBROSPINAL FLUID; CORNEA DISEASE; CYSTIC FIBROSIS; ELLIPTOCYTOSIS; EMBRYO DEVELOPMENT; EPILEPSY; ERYTHROCYTE DISORDER; FEMALE FERTILITY; GASTROINTESTINAL TRACT; HARBOYAN SYNDROME; HEARING IMPAIRMENT; HEART VENTRICLE HYPERTROPHY; HEREDITARY SPHEROCYTOSIS; HUMAN; KIDNEY DISTAL TUBULE ACIDOSIS; KIDNEY PROXIMAL TUBULE ACIDOSIS; KIDNEY TUBULE ACIDOSIS; NONHUMAN; OOCYTE DEVELOPMENT; PANCREAS; PENDRED SYNDROME; PHYLOGENY; PRIORITY JOURNAL; PROTEIN FUNCTION; RETINA DISEASE; REVIEW; SALIVARY GLAND; SOUTHEAST ASIAN OVALOCYTOSIS; SPERMATOGENESIS; SPERMATOZOON CAPACITATION; STOMACH ACID SECRETION; STOMACH PARIETAL CELL; THYROID FUNCTION; TOOTH DEVELOPMENT; TRANSPORT AT THE CELLULAR LEVEL; VISION; ANIMAL; CELL FUNCTION; GENERAL ASPECTS OF DISEASE; ION TRANSPORT; METABOLISM; REPRODUCTION;

MAMMALIA;

ACIDS; ANIMALS; BICARBONATES; CELL PHYSIOLOGICAL PHENOMENA; DISEASE; HUMANS; ION TRANSPORT; REPRODUCTION;

EID: 58249090515     PISSN: 02646021     EISSN: 14708728     Source Type: Journal    
DOI: 10.1042/BJ20081634     Document Type: Review

References (229)

1. Sherman, T., Chernova, M. N., Clark, J. S., Jiang, L., Alper, S. L. and Nehrke, K. (2005) The abts and sulp families of anion transporters from Caenorhabditis elegans. Am. J. Physiol. Cell. Physiol. 289, C341-C351
2. Kopito, R. R. and Lodish, H. F. (1985) Primary structure and transmembrane orientation of the murine anion exchange protein. Nature 316, 234-238
3. Lohi, H., Kujala, M., Makela, S., Lehtonen, E., Kestila, M., Saarialho-Kere, U., Markovich, D. and Kere, J. (2002) Functional characterization of three novel tissue-specific anion exchangers: SLC26A7, A8 and A9. J. Biol. Chem. 277, 14246-14254
4. Wain, H. M., Lush, M. J., Ducluzeau, F., Khodiyar, V. K. and Povey, S. (2004) Genew: the Human Gene Nomenclature Database, 2004 updates. Nucleic Acids Res. 32, D255-D257
5. Everett, L. A., Glaser, B., Beck, J. C., Idol, J. R., Buchs, A., Heyman, M., Adawi, F., Hazani, E., Nassir, E., Baxevanis, A. D. et al. (1997) Pendred syndrome is caused by mutations in a putative sulphate transporter gene (PDS). Nat. Genet. 17, 411-422
6. Kopito, R. R. and Lodish, H. L. (1985) Structure of the murine anion exchange protein. J. Cell. Biochem. 29, 1-17
7. Zhu, Q. and Casey, J. R. (2004) The substrate anion selectivity filter in the human erythrocyte Cl-/HCO3- exchange protein, AE1. J. Biol. Chem. 279, 23565-23573
8. Grinstein, S., Ship, S. and Rothstein, A. (1978) Anion transport in relation to proteolytic dissection of band 3 protein. Biochim. Biophys. Acta 507, 294-304
9. Alper, S. L., Kopito, R. R., Libresco, S. M. and Lodish, H. F. (1988) Cloning and characterization of a murine Band 3-related cDNA from kidney and a lymphoid cell line. J. Biol. Chem. 263, 17092-17099
10. 3- exchanger. J. Biol. Chem. 265, 462-471
11. Zolotarev, A. S., Chernova, M. N., Yannoukakos, D. and Alper, S. L. (1996) Proteolytic cleavage sites of native AE2 anion exchanger in gastric mucosal membranes. Biochemistry 35, 10367-10376
12. 3- exchanger. J. Biol. Chem. 267, 7927-7935
13. 3- exchanger are encoded by mRNAs transcribed from alternative promoters. J. Biol. Chem. 271, 7835-7843
14. Sterling, D. and Casey, J. R. (1999) Transport activity of AE3 chloride/ bicarbonate anion-exchange proteins and their regulation by intracellular pH. Biochem. J. 344, 221-229
15. Gross, E., Hawkins, K., Abuladze, N., Pushkin, A., Cotton, C. U., Hopfer, U. and Kurtz, I. (2001) The stoichiometry of the electrogenic sodium bicarbonate cotransporter NBC1 is cell-type dependent. J. Physiol. 531, 597-603
16. + stoichiometry from 3:1 to 2:1 in murine proximal tubule cells. J. Physiol. 537, 659-665
17. 3- cotransporter stoichiometry. Am. J. Physiol. Renal Physiol. 283, F876-F887
18. 2- transport (QAbstract). FASEB J. 16, A795
19. 3 exchanger in the basolateral membrane of the renal CCD and the SMG duct. Am. J. Physiol. Cell Physiol. 283, C1206-C1218
20. Parker, M. D., Boron, W. F. and Tanner, M. J. (2002) Characterization of human 'AE4' as an electroneutral sodium bicarbonate cotransporter. FASEB J. 16, A796
21. - transporters. Pflugers Arch. 14, 14
22. - exchanger. J. Biol. Chem. 276, 8358-8363
23. - exchanger: cloning, tissue distribution, and functional characterization. J. Biol. Chem. 275, 35486-35490
24. - self-exchange activity. J. Biol. Chem. 283, 12777-12788
25. Parker, M. D., Ourmozdi, E. P. and Tanner, M. J. (2001) Human BTR1, a new bicarbonate transporter superfamily member and human AE4 from kidney. Biochem. Biophys. Res. Commun. 282, 1103-1109
26. Takano, J., Noguchi, K., Yasumori, M., Kobayashi, M., Gajdos, Z., Miwa, K., Hayashi, H., Yoneyama, T. and Fujiwara, T. (2002) Arabidopsis boron transporter for xylem loading. Nature 420, 337-340
27. +-coupled borate transporter essential for cellular boron homeostasis and cell growth and proliferation. Mol. Cell 16, 331-341
28. Desir, J., Moya, G., Reish, O., Van Regemorter, N., Deconinck, H., David, K. L., Meire, F. M. and Abramowicz, M. (2007) Borate transporter SLC4A11 mutations cause both Harboyan syndrome and non-syndromic corneal endothelial dystrophy. J. Med. Genet. 44, 322-326
29. Vithana, E. N., Morgan, P., Sundaresan, P., Ebenezer, N. D., Tan, D. T., Mohamed, M. D., Anand, S., Khine, K. O., Venkataraman, D., Yong, V. H. et al. (2006) Mutations in sodium-borate cotransporter SLC4A11 cause recessive congenital hereditary endothelial dystrophy (CHED2). Nat. Genet. 38, 755-757
30. Vithana, E. N., Morgan, P. E., Ramprasad, V., Tan, D. T., Yong, V. H., Venkataraman, D., Venkatraman, A., Yam, G. H., Nagasamy, S., Law, R. W. et al. (2008) SLC4A11 mutations in Fuchs Endothelial Corneal Dystrophy (FECD). Hum. Mol. Genet. 17, 656-666
31. Dawson, P. A. and Markovich, D. (2005) Pathogenetics of the human SLC26 transporters. Curr. Med. Chem. 12, 385-396.
32. Dorwart, M. R., Shcheynikov, N., Yang, D. and Muallem, S. (2008) The solute carrier 26 family of proteins in epithelial ion transport. Physiology 23, 104-114
33. Markovich, D., Bissig, M., Sorribas, V., Hagenbuch, B., Meier, P. J. and Murer, H. (1994) Expression of rat renal sulfate transport systems in Xenopus laevis oocytes. Functional characterization and molecular identification. J. Biol. Chem. 269, 3022-3026
34. Markovich, D. (2001) Physiological roles and regulation of mammalian sulfate transporters. Physiol. Rev. 81, 1499-1533
35. Mount, D. B. and Romero, M. F. (2003) The SLC26 gene family of multifunctional anion exchangers. Pflugers Arch. 447, 710-721
36. Ko, S. B., Shcheynikov, N., Choi, J. Y., Luo, X., Ishibashi, K., Thomas, P. J., Kim, J. Y., Kim, K. H., Lee, M. G., Naruse, S. and Muallem, S. (2002) A molecular mechanism for aberrant CFTR-dependent HCO3- transport in cystic fibrosis. EMBO J. 21, 5662-5672
37. 3- exchange by slc26a3 and slc26a6. J. Gen. Physiol. 127, 511-524
38. 3- exchanger in the upper villous epithelium of the murine duodenum. Am. J. Physiol. Gastrointest. Liver Physiol. 292, G1079-G1088
39. Chernova, M. N., Jiang, L., Friedman, D. J., Darman, R. B., Lohi, H., Kere, J., Vandorpe, D. H. and Alper, S. L. (2005) Functional comparison of mouse slc26a6 anion exchanger with human SLC26A6 polypeptide variants: differences in anion selectivity, regulation, and electrogenicity. J. Biol. Chem. 280, 8564-8580
40. 3- secretion. Acta Physiol. 193, 357-365
41. - secretion and in regulation of CFTR in the parotid duct. J. Physiol. 586, 3813-3824
42. - exchangers SLC26A7 and AE1 in kidney outer medullary collecting duct. J. Am. Soc. Nephrol. 15, 2002-2011
43. - exchanger specific to gastric parietal cells. Am. J. Physiol. Gastrointest. Liver Physiol. 284, G1093-G1103
44. Morgan, P. E., Pastorekov'a, S., Stuart-Tilley, A. K., Alper, S. L. and Casey, J. R. (2007) Interactions of transmembrane carbonic anhydrase, CAIX, with bicarbonate transporters. Am. J. Physiol. Cell Physiol. 293, C738-C744
45. - channel regulated by intracellular pH. J. Biol. Chem. 280, 6463-6470
46. Kosiek, O., Busque, S. M., Foller, M., Shcheynikov, N., Kirchhoff, P., Bleich, M., Muallem, S. and Geibel, J. P. (2007) SLC26A7 can function as a chloride-loading mechanism in parietal cells. Pflugers Arch. 454, 989-998
47. +. Am. J. Physiol. Cell Physiol. 289, C493-C505
48. - channel regulated by the WNK kinases. J. Physiol. 584, 333-345
49. Loriol, C., Dulong, S., Avella, M., Gabillat, N., Boulukos, K., Borgese, F. and Ehrenfeld, J. (2008) Characterization of SLC26A9, facilitation of Cl transport by bicarbonate. Cell. Physiol. Biochem. 22, 15-30
50. Alvarez, B. V., Vilas, G. L. and Casey, J. R. (2005) Metabolon disruption: a mechanism that regulates bicarbonate transport. EMBO J. 24, 2499-2511
51. Ko, S. B., Zeng, W., Dorwart, M. R., Luo, X., Kim, K. H., Millen, L., Goto, H., Naruse, S., Soyombo, A., Thomas, P. J. and Muallem, S. (2004) Gating of CFTR by the STAS domain of SLC26 transporters. Nat. Cell Biol. 6, 343-350
52. Dorwart, M. R., Shcheynikov, N., Baker, J. M., Forman-Kay, J. D., Muallem, S. and Thomas, P. J. (2008) Congenital chloride-losing diarrhea causing mutations in the stas domain result in misfolding and mistrafficking of SLC26A3. J. Biol. Chem. 283, 8711-8722
53. Vandenberg, J. I., Metcalfe, J. C. and Grace, A. A. (1993) Mechanisms of pHi recovery after global ischemia in the perfused heart. Circ. Res. 72, 993-1003
54. Yang, B., Fukuda, N., van Hoek, A., Matthay, M. A., Ma, T. and Verkman, A. S. (2000) Carbon dioxide permeability of aquaporin-1 measured in erythrocytes and lung of aquaporin-1 null mice and in reconstituted proteoliposomes. J. Biol. Chem. 275, 2686-2692
55. 2 transport across the human erythrocyte membrane. FASEB J. 20, 1974-1981
56. Bruce, L. J., Beckmann, R., Ribeiro, M. L., Peters, L. L., Chasis, J. Delauney, J., Mohandas, N., Anstee, D. J. and Tanner, M. J. (2003) A band 3-based macrocomplex of integral and peripheral proteins in the red cell membrane. Blood 101, 4180-4188
57. 2 permeability in lung and kidney. J. Physiol. 542, 63-69
58. Wang, Y., Cohen, J., Boron, W. F., Schulten, K. and Tajkhorshid, E. (2007) Exploring gas permeability of cellular membranes and membrane channels with molecular dynamics. J. Struct. Biol. 157, 534-544
59. 2 channel in the human red cell membrane. FASEB J. 22, 64-73
60. Girijashanker, K., He, L., Soleimani, M., Reed, J. M., Li, H., Liu, Z., Wang, B., Dalton, T. P. and Nebert, D. W. (2008) Slc39a14 gene encodes ZIP14, a metal/bicarbonate symporter: similarities to the ZIP8 transporter. Mol. Pharmacol. 73, 1413-1423
61. He, L., Girijashanker, K., Dalton, T. P., Reed, J., Li, H., Soleimani, M. and Nebert, D. W. (2006) ZIP8, member of the solute-carrier-39 (SLC39) metal-transporter family: characterization of transporter properties. Mol. Pharmacol. 70, 171-180
62. Hug, M. J., Tamada, T. and Bridges, R. J. (2003) CFTR and bicarbonate secretion by epithelial cells. News Physiol. Sci. 18, 38-42
63. Wang, X. F., Zhou, C. X., Shi, Q. X., Yuan, Y. Y., Yu, M. K., Ajonuma, L. C., Ho, L. S., Lo, P. S., Tsang, L. L., Liu, Y. et al. (2003) Involvement of CFTR in uterine bicarbonate secretion and the fertilizing capacity of sperm. Nat. Cell Biol. 5, 902-906
64. - secretion. Am. J. Physiol. Gastrointest. Liver Physiol. 285, G887-G897
65. Bormann, J., Hamill, O. P. and Sakmann, B. (1987) Mechanism of anion permeation through channels gated by glycine and γ-aminobutyric acid in mouse cultured spinal neurones. J. Physiol. 385, 243-286
66. -. Am. J. Physiol. Cell Physiol. 294, C1371-C1377
67. Detro-Dassen, S., Schanzler, M., Lauks, H., Martin, I., Meyer Zu Berstenhorst, S., Nothmann, D., Torres-Salazar, D., Hidalgo, P., Schmalzing, G. and Fahlke, C. (2007) Conserved dimeric subunit stoichiometry of SLC26 multifunctional anion exchangers. J. Biol. Chem. 283, 4177-4188
68. Casey, J. R. and Reithmeier, R. A. (1991) Analysis of the oligomeric state of Band 3, the anion transport protein of the human erythrocyte membrane, by size exclusion high performance liquid chromatography. Oligomeric stability and origin of heterogeneity. J. Biol. Chem. 266, 15726-15737
69. Kao, L., Sassani, P., Azimov, R., Pushkin, A., Abuladze, N., Peti-Peterdi, J., Liu, W., Newman, D. and Kurtz, I. (2008) Oligomeric structure and minimal functional unit of the electrogenic sodium bicarbonate cotransporter NBCe1-A. J. Biol. Chem. 283, 26782-26794
70. Wieth, J. O., Andersen, O. S., Brahm, J., Bjerrum, P. J. and Borders, Jr, C. L. (1982) Chloride-bicarbonate exchange in red blood cells: physiology of transport and chemical modification of binding sites. Philos. Trans. R. Soc. London Ser B 299, 383-399
71. McMurtrie, H. L., Cleary, H. J., Alvarez, B. V., Loiselle, F. B., Sterling, D., Morgan, P. E., Johnson, D. E. and Casey, J. R. (2004) The bicarbonate transport metabolon. J. Enzyme Inhib. Med. Chem. 19, 231-236
72. - exchanger. J. Biol. Chem. 273, 28430-28437
73. Sterling, D., Reithmeier, R. A. and Casey, J. R. (2001) A transport metabolon. functional interaction of carbonic anhydrase II and chloride/ bicarbonate exchangers. J. Biol. Chem. 276, 47886-47894
74. Chang, S. H. and Low, P. S. (2003) Identification of a critical ankyrin-binding loop on the cytoplasmic domain of erythrocyte membrane band 3 by crystal structure analysis and site-directed mutagenesis. J. Biol. Chem. 278, 6879-6884
75. Low, P. S. (1986) Structure and function of the cytoplasmic domain of Band 3: center of erythrocyte membrane-peripheral protein interactions. Biochim. Biophys. Acta 864, 145-167
76. Perrotta, S., Borriello, A., Scaloni, A., De Franceschi, L., Brunati, A. M., Turrini, F., Nigro, V., del Giudice, E. M., Nobili, B., Conte, M. L. et al. (2005) The N-terminal 11 amino acids of human erythrocyte band 3 are critical for aldolase binding and protein phosphorylation: implications for band 3 function. Blood 106, 4359-4366
77. Delaunay, J. (2007) The molecular basis of hereditary red cell membrane disorders. Blood Rev. 21, 1-20
78. Bruce, L. J., Robinson, H. C., Guizouarn, H., Borgese, F., Harrison, P., King, M. J., Goede, J. S., Coles, S. E., Gore, D. M., Lutz, H. U. et al. (2005) Monovalent cation leaks in human red cells caused by single amino-acid substitutions in the transport domain of the band 3 chloride-bicarbonate exchanger, AE1. Nat. Genet. 37, 1258-1263
79. Ganesan, J., Lie-Injo, L. E. and Ong Beng, P. (1975) Abnormal hemoglobins, glucose-6-phosphate dehydrogenase deficiency and hereditary ovalocytosis in the Dayaks of Sarawak. Hum. Hered. 25, 258-262
80. Lie-Injo, L. E., Fix, A., Bolton, J. M. and Gilman, R. H. (1972) Haemoglobin E-hereditary elliptocytosis in Malayan aborigines. Acta Haematol. 47, 210-216
81. Eber, S. and Lux, S. E. (2004) Hereditary spherocytosis-defects in proteins that connect the membrane skeleton to the lipid bilayer. Semin. Hematol. 41, 118-141
82. Alloisio, N., Texier, P., Vallier, A., Ribeiro, M. L., Morle, L., Bozon, M., Bursaux, E., Maillet, P., Goncalves, P., Tanner, M. J. et al. (1997) Modulation of clinical expression and band 3 deficiency in hereditary spherocytosis. Blood 90, 414-420
83. Ribeiro, M. L., Alloisio, N., Almeida, H., Gomes, C., Texier, P., Lemos, C., Mimoso, G., Morle, L., Bey-Cabet, F., Rudigoz, R. C. et al. (2000) Severe hereditary spherocytosis and distal renal tubular acidosis associated with the total absence of band 3. Blood 96, 1602-1604
84. Toye, A. M., Williamson, R. C., Khanfar, M., Bader-Meunier, B., Cynober, T., Thibault, M., Tchernia, G., Dechaux, M., Delaunay, J. and Bruce, L. J. (2008) Band 3 Courcouronnes (Ser667Phe): a trafficking mutant differentially rescued by wild type band 3 and glycophorin A. Blood 111, 5380-5389
85. Southgate, C. D., Chishti, A. H., Mitchell, B., Yi, S. J. and Palek, J. (1996) Targeted disruption of the murine erythroid band 3 gene results in spherocytosis and severe haemolytic anaemia despite a normal membrane skeleton. Nat. Genet. 14, 227-230
86. Peters, L. L., Shivdasani, R. A., Liu, S. C., Hanspal, M., John, K. M., Gonzalez, J. M., Brugnara, C., Gwynn, B., Mohandas, N., Alper, S. L. et al. (1996) Anion exchanger 1 (band 3) is required to prevent erythrocyte membrane surface loss but not to form the membrane skeleton. Cell 86, 917-927
87. Jarolim, P., Palek, J., Amato, D., Hassan, K., Sapak, P., Nurse, G. T., Rubin, H. L., Zhai, S., Sahr, K. E. and Liu, S. C. (1991) Deletion in erythrocyte band 3 gene in malaria-resistant Southeast Asian ovalocytosis. Proc. Natl. Acad. Sci. U.S.A. 88, 11022-11026
88. Schofield, A. E., Tanner, M. J. A., Pinder, J. C., Clough, B., Bayley, P. M., Nash, G. B., Dluzewski, A. R., Reardon, D. M., Cox, T. M., Wilson, R. J. M. and Gratzer, W. B. (1992) Basis of unique red cell membrane properties in hereditary ovalocytosis. J. Mol. Biol. 223, 949-958
89. Mohandas, N., Lie-Injo, L. E., Friedman, M. and Mak, J. W. (1984) Rigid membranes of Malayan ovalocytes: a likely genetic barrier against malaria. Blood 63, 1385-1392
90. Vasuvattakul, S., Yenchitsomanus, P. T., Vachuanichsanong, P., Thuwajit, P., Kaitwatcharachai, C., Laosombat, V., Malasit, P., Wilairat, P. and Nimmannit, S. (1999) Autosomal recessive distal renal tubular acidosis associated with Southeast Asian ovalocytosis. Kidney Int. 56, 1674-1682
91. Genton, B., al-Yaman, F., Mgone, C. S., Alexander, N., Paniu, M. M., Alpers, M. P. and Mokela, D. (1995) Ovalocytosis and cerebral malaria. Nature 378, 564-565
92. Jennings, M. L. and Gosselink, P. G. (1995) Anion exchange protein in Southeast Asian ovalocytes: heterodimer formation between normal and variant subunits. Biochemistry 34, 3588-3595
93. Moriyama, R., Ideguchi, H., Lombardo, C. R., Van Dort, H. M. and Low, P. S. (1992) Structural and functional characterization of band 3 from Southeast Asian ovalocytes. J. Biol. Chem. 267, 25792-25797
94. Sarabia, V. E., Casey, J. R. and Reithmeier, R. A. (1993) Molecular characterization of the band 3 protein from Southeast Asian ovalocytes. J. Biol. Chem. 268, 10676-10680
95. Liu, S. C., Jarolim, P., Rubin, H. L., Palek, J., Amato, D., Hassan, K., Zaik, M. and Sapak, P. (1994) The homozygous state for the band 3 protein mutation in Southeast Asian Ovalocytosis may be lethal. Blood 84, 3590-3591
96. Cheung, J. C., Cordat, E. and Reithmeier, R. A. (2005) Trafficking defects of the Southeast Asian ovalocytosis deletion mutant of anion exchanger 1 membrane proteins. Biochem. J. 392, 425-434
97. Daniels, G. L., Fletcher, A., Garratty, G., Henry, S., Jorgensen, J., Judd, W. J., Levene, C., Lomas-Francis, C., Moulds, J. J., Moulds, J. M. et al. (2004) Blood group terminology 2004: from the International Society of Blood Transfusion committee on terminology for red cell surface antigens. Vox Sang. 87, 304-316
98. Bruce, L. J., Ring, S. M., Anstee, D. J., Reid, M. E., Wilkinson, S. and Tanner, M. J. (1995) Changes in the blood group Wright antigens are associated with a mutation at amino acid 658 in human erythrocyte band 3: a site of interaction between band 3 and glycophorin A under certain conditions. Blood 85, 541-547
99. - co-transporter. Biochemistry 42, 12321-12329
100. - cotransporter in human corneal endothelial cells. Pflugers Arch. 438, 458-462
101. Dinour, D., Chang, M. H., Satoh, J., Smith, B. L., Angle, N., Knecht, A., Serban, I., Holtzman, E. J. and Romero, M. F. (2004) A novel missense mutation in the sodium bicarbonate cotransporter (NBCe1/SLC4A4) causes proximal tubular acidosis and glaucoma through ion transport defects. J. Biol. Chem. 279, 52238-52246
102. 3- cotransporter gene (SLC4A4) in a patient with permanent isolated proximal renal tubular acidosis and bilateral glaucoma. J. Am. Soc. Nephrol. 12, 713-718
103. Igarashi, T., Inatomi, J., Sekine, T., Cha, S. H., Kanai, Y., Kunimi, M., Tsukamoto, K., Satoh, H., Shimadzu, M., Tozawa, F. et al. (1999) Mutations in SLC4A4 cause permanent isolated proximal renal tubular acidosis with ocular abnormalities. Nat. Genet. 23, 264-266
104. Inatomi, J., Horita, S., Braverman, N., Sekine, T., Yamada, H., Suzuki, Y., Kawahara, K., Moriyama, N., Kudo, A., Kawakami, H. et al. (2004) Mutational and functional analysis of SLC4A4 in a patient with proximal renal tubular acidosis. Pflugers Arch. 448, 438-444
105. Toye, A. M., Parker, M. D., Daly, C. M., Lu, J., Virkki, L. V., Pelletier, M. F. and Boron, W. F. (2006) The human NBCe1-A mutant R881C, associated with proximal renal tubular acidosis, retains function but is mistargeted in polarized renal epithelia. Am. J. Physiol. Cell Physiol. 291, C788-C801
106. Horita, S., Yamada, H., Inatomi, J., Moriyama, N., Sekine, T., Igarashi, T., Endo, Y., Dasouki, M., Ekim, M., Al-Gazali, L. et al. (2005) Functional analysis of NBC1 mutants associated with proximal renal tubular acidosis and ocular abnormalities. J. Am. Soc. Nephrol. 16, 2270-2278
107. Demirci, F. Y., Chang, M. H., Mah, T. S., Romero, M. F. and Gorin, M. B. (2006) Proximal renal tubular acidosis and ocular pathology: a novel missense mutation in the gene (SLC4A4) for sodium bicarbonate cotransporter protein (NBCe1). Mol. Vis. 12, 324-330
108. - cotransporter NBC1 show abnormal trafficking in polarized kidney cells: a basis of proximal renal tubular acidosis. Am. J. Physiol. Renal Physiol. 289, F61-F71
109. Jiang, Z., Asplin, J. R., Evan, A. P., Rajendran, V. M., Velazquez, H., Nottoli, T. P., Binder, H. J. and Aronson, P. S. (2006) Calcium oxalate urolithiasis in mice lacking anion transporter Slc26a6. Nat. Genet. 38, 474-478
110. Shayakul, C. and Alper, S. L. (2000) Inherited renal tubular acidosis. Curr. Opin. Nephrol. Hypertens. 9, 541-546
111. - exchanger (slc4a1). J. Am. Soc. Nephrol. 18, 1408-1418
112. Cordat, E. (2006) Unraveling trafficking of the kidney anion exchanger 1 in polarized MDCK epithelial cells. Biochem. Cell Biol. 84, 949-959
113. Cordat, E. and Reithmeier, R. (2006) Expression and interaction of two compound heterozygous distal renal tubular acidosis mutants of kidney anion exchanger 1 in epithelial cells. Am. J. Physiol. Renal Physiol. 291, F1354-F1361
114. Quilty, J. A., Cordat, E. and Reithmeier, R. A. (2002) Impaired trafficking of human kidney anion exchanger (kAE1) caused by hetero-oligomer formation with a truncated mutant associated with distal renal tubular acidosis. Biochem. J. 13, 895-903
115. Kittanakom, S., Keskanokwong, T., Akkarapatumwong, V., Yenchitsomanus, P. T. and Reithmeier, R. A. (2004) Human kanadaptin and kidney anion exchanger 1 (kAE1) do not interact in transfected HEK 293 cells. Mol. Membr. Biol. 21, 395-402
116. Devonald, M. A., Smith, A. N., Poon, J. P., Ihrke, G. and Karet, F. E. (2003) Non-polarized targeting of AE1 causes autosomal dominant distal renal tubular acidosis. Nat. Genet. 33, 125-127
117. Toye, A. M., Banting, G. and Tanner, M. J. (2004) Regions of human kidney anion exchanger 1 (kAE1) required for basolateral targeting of kAE1 in polarised kidney cells: mis-targeting explains dominant renal tubular acidosis (dRTA). J. Cell Sci. 117, 1399-1410
118. - exchanger specific to intercalated cells of the outer medullary collecting duct. Am. J. Physiol. Renal Physiol. 286, F161-F169
119. 3 loading. Am. J. Physiol. Renal Physiol. 284, F584-F593
120. -/anion exchanger pendrin in mouse kidney by acid-base status. Kidney Int. 62, 2109-2117
121. Verlander, J. W., Hassell, K. A., Royaux, I. E., Glapion, D. M., Wang, M. E., Everett, L. A., Green, E. D. and Wall, S. M. (2003) Deoxycorticosterone upregulates PDS (Slc26a4) in mouse kidney: role of pendrin in mineralocorticoid-induced hypertension. Hypertension 42, 356-362
122. - transporter expression is reduced in Slc26a4 null mice. Am. J. Physiol. Renal Physiol. 289, F1262-F1272
123. Wall, S. M., Kim, Y. H., Stanley, L., Glapion, D. M., Everett, L. A., Green, E. D. and Verlander, J. W. (2004) NaCl restriction upregulates renal Slc26a4 through subcellular redistribution: role in Cl- conservation. Hypertension 44, 982-987
124. Royaux, I. E., Suzuki, K., Mori, A., Katoh, R., Everett, L. A., Kohn, L. D. and Green, E. D. (2000) Pendrin, the protein encoded by the Pendred syndrome gene (PDS), is an apical porter of iodide in the thyroid and is regulated by thyroglobulin in FRTL-5 cells. Endocrinology 141, 839-845
125. - secretion. Acta Physiol. 193, 309
126. - secretion. Gastroenterology 130, 349-358
127. - cotransporter. J. Biol. Chem. 282, 9042-9052
128. Kawamata, K., Hayashi, H. and Suzuki, Y. (2007) Propionate absorption associated with bicarbonate secretion in vitro in the mouse cecum. Pflugers Arch. 454, 253-262
129. Lecona, E., Olmo, N., Turnay, J., Santiago-Gomez, A., Lopez de Silanes, I., Gorospe, M. and Lizarbe, M. A. (2008) Kinetic analysis of butyrate transport in human colon adenocarcinoma cells reveals two different carrier-mediated mechanisms. Biochem. J. 409, 311-320
130. - exchanger are achlorhydric. J. Biol. Chem. 279, 30531-30539
131. Smith, C. E., Chong, D. L., Bartlett, J. D. and Margolis, H. C. (2005) Mineral acquisition rates in developing enamel on maxillary and mandibular incisors of rats and mice: implications to extracellular acid loading as apatite crystals mature. J. Bone Miner. Res. 20, 240-249
132. Lyaruu, D. M., Bronckers, A. L., Mulder, L., Mardones, P., Medina, J. F., Kellokumpu, S., Oude Elferink, R. P. and Everts, V. (2007) The anion exchanger Ae2 is required for enamel maturation in mouse teeth. Matrix Biol. 27, 119-127
133. Paine, M. L., Snead, M. L., Wang, H. J., Abuladze, N., Pushkin, A., Liu, W., Kao, L. Y., Wall, S. M., Kim, Y. H. and Kurtz, I. (2008) Role of NBCe1 and AE2 in secretory ameloblasts. J. Dent. Res. 87, 391-395
134. Chesler, M. (2003) Regulation and modulation of pH in the brain. Physiol. Rev. 83, 1183-1221
135. Sander, T., Toliat, M. R., Heils, A., Leschik, G., Becker, C., Ruschendorf, F., Rohde, K., Mundlos, S. and Nurnberg, P. (2002) Association of the 867Asp variant of the human anion exchanger 3 gene with common subtypes of idiopathic generalized epilepsy. Epilepsy Res. 51, 249-255
136. - exchanger AE3 display a reduced seizure threshold. Mol. Cell. Biol. 26, 182-191
137. Jacobs, S., Ruusuvuori, E., Sipila, S. T., Haapanen, A., Damkier, H. H., Kurth, I., Hentschke, M., Schweizer, M., Rudhard, Y., Laatikainen, L. M. et al. (2008) Mice with targeted Slc4a10 gene disruption have small brain ventricles and show reduced neuronal excitability. Proc. Natl. Acad. Sci. U.S.A. 105, 311-316
138. Thiry, A., Dogne, J. M., Supuran, C. T. and Masereel, B. (2008) Anticonvulsant sulfonamides/sulfamates/sulfamides with carbonic anhydrase inhibitory activity: drug design and mechanism of action. Curr. Pharm. Des. 14, 661-671
139. Brown, P. D., Davies, S. L., Speake, T. and Millar, I. D. (2004) Molecular mechanisms of cerebrospinal fluid production. Neuroscience 129, 957-970
140. Praetorius, J. (2007) Water and solute secretion by the choroid plexus. Pflugers Arch. 454, 1-18
141. Kong, C. W., Li, K. K. and To, C. H. (2006) Chloride secretion by porcine ciliary epithelium: new insight into species similarities and differences in aqueous humor formation. Invest. Ophthalmol. Visual Sci. 47, 5428-5436
142. Bonanno, J. A. (2003) Identity and regulation of ion transport mechanisms in the corneal endothelium. Prog. Retin. Eye Res. 22, 69-94
143. Bok, D., Schibler, M. J., Pushkin, A., Sassani, P., Abuladze, N., Naser, Z. and Kurtz, I. (2001) Immunolocalization of electrogenic sodium-bicarbonate cotransporters pNBC1 and kNBC1 in the rat eye. Am. J. Physiol. Renal Physiol. 281, F920-F935
144. Salas, J. T., Banales, J. M., Sarvide, S., Recalde, S., Ferrer, A., Uriarte, I., Oude Elferink, R. P., Prieto, J. and Medina, J. F. (2008) Ae2a,b-deficient mice develop antimitochondrial antibodies and other
145. Hejny, C., Edelhauser, H. F. and Ubels, J. L. (2003) Cornea and schlera. In Alder's Physiology of the Eye: Clinical Application (Kaufman, P. L. and Alm, A., eds), pp. 47-114, Mosby, St Louis, U.S.A.
146. Jiao, X., Sultana, A., Garg, P., Ramamurthy, B., Vemuganti, G. K., Gangopadhyay, N., Hejtmancik, J. F. and Kannabiran, C. (2007) Autosomal recessive corneal endothelial dystrophy (CHED2) is associated with mutations in SLC4A11. J. Med. Genet. 44, 64-68
147. Winkler, B. S. (1981) Glycolytic and oxidative metabolism in relation to retinal function. J. Gen. Physiol. 77, 667-692
148. Kobayashi, S., Morgans, C. W., Casey, J. R. and Kopito, R. R. (1994) AE3 anion exchanger isoforms in the vertebrate retina: developmental regulation and differential expression in neurons and glia. J. Neurosci. 14, 6266-6279
149. Alvarez, B. V., Gilmour, G., Mema, S. C., Shull, G. E., Casey, J. R. and Sauvé, Y. (2007) Blindness results from deficiency in AE3 chloride/bicarbonate exchanger. PloS ONE 2, e839
150. Bok, D., Galbraith, G., Lopez, I., Woodruff, M., Nusinowitz, S., BeltrandelRio, H., Huang, W., Zhao, S., Geske, R., Montgomery, C. et al. (2003) Blindness and auditory impairment caused by loss of the sodium bicarbonate cotransporter NBC3. Nat. Genet. 34, 313-319
151. Everett, L. A., Morsli, H., Wu, D. K. and Green, E. D. (1999) Expression pattern of the mouse ortholog of the Pendred's syndrome gene (Pds) suggests a key role for pendrin in the inner ear. Proc. Natl. Acad. Sci. U.S.A. 96, 9727-9732
152. Everett, L. A., Belyantseva, I. A., Noben-Trauth, K., Cantos, R., Chen, A., Thakkar, S. I., Hoogstraten-Miller, S. L., Kachar, B., Wu, D. K. and Green, E. D. (2001) Targeted disruption of mouse Pds provides insight about the inner-ear defects encountered in Pendred syndrome. Hum. Mol. Genet. 10, 153-161
153. 2+] by inhibition of acid-sensitive TRPV5 and TRPV6 channels. Am. J. Physiol. Renal Physiol. 292, F1314-F1321
154. 2+ reabsorption in a Pendred syndrome mouse model. Am. J. Physiol. Renal Physiol. 292, F1345-F1353
155. Harboyan, G., Mamo, J., der Kaloustian, V. and Karam, F. (1971) Congenital corneal dystrophy. Progressive sensorineural deafness in a family. Arch. Ophthalmol. 85, 27-32
156. Morris, K. A., Snir, E., Pompeia, C., Koroleva, I. V., Kachar, B., Hayashizaki, Y., Carninci, P., Soares, M. B. and Beisel, K. W. (2005) Differential expression of genes within the cochlea as defined by a custom mouse inner ear microarray. J. Assoc. Res. Otolaryngol. 6, 75-89
157. Orchard, C. H. and Kentish, J. C. (1990) Effects of changes of pH on the contractile function of cardiac muscle. Am. J. Physiol. 27, C967-C981
158. Hermans, M. M., Kortekaas, P., Jongsma, H. J. and Rook, M. B. (1995) pH sensitivity of the cardiac gap junction proteins, connexin 45 and 43. Pflugers Arch. 431, 138-140
159. Vaughan-Jones, R. D. (1982) Chloride activity and its control in skeletal and cardiac muscle. Phil. Trans. R. Soc. London Ser B 299, 537-548
160. Alvarez, B. V., Kieller, D. M., Quon, A. L., Markovich, D. and Casey, J. R. (2004) Slc26a6: a cardiac chloride/hydroxyl exchanger and predominant chloride/bicarbonate exchanger of the heart. J. Physiol. 561, 721-734
161. Alvarez, B. V., Kieller, D. M., Quon, A. L., Robertson, M. and Casey, J. R. (2007) Cardiac hypertrophy in anion exchanger 1-null mutant mice with severe hemolytic anemia. Am. J. Physiol. Heart Circ. Physiol. 292, H1301-H1312
162. Cingolani, H. E. and Ennis, I. L. (2007) Sodium-hydrogen exchanger, cardiac overload, and myocardial hypertrophy. Circulation 115, 1090-1100
163. + exchanger. Curr. Cardiol. Rev. 3, 149-164
164. Thomas, R. (1989) Cell growth factors: bicarbonate and pHi response. Nature 337, 601
165. Ganz, M. B., Boyarsky, G., Boron, W. F. and Sterzel, R. B. (1988) Effects of angiotensin II and vasopressin on intracellular pH of glomerular mesangial cells. Am. J. Physiol. 254, F787-F794
166. + exchanger. Circ. Res. 77, 1192-1200
167. + exchangers. J. Biol. Chem. 275, 1-4
168. 2+ exchange after myocardial stretch: underlying mechanism of the slow force response. Circ. Res. 88, 376-382
169. + exchanger inhibition: a new antihypertrophic tool. Circ. Res. 90, 751-753
170. Alvarez, B. V., Fujinaga, J. and Casey, J. R. (2001) Molecular basis for angiotensin II-induced increase of chloride/bicarbonate exchange in the myocardium. Circ. Res. 89, 1246-1253
171. Alvarez, B. V., Xia, Y., Sowah, D., Soliman, D., Light, P., Karmazyn, M. and Casey, J. R. (2007) A carbonic anhydrase inhibitor prevents and reverts cardiomyocyte hypertrophy. J. Physiol. 579, 127-145
172. - cotransport modulates resting membrane potential and action potential duration in cat ventricular myocytes. J. Physiol. 578, 819-829
173. Khandoudi, N., Albadine, J., Robert, P., Krief, S., Berrebi-Bertrand, I., Martin, X., Bevensee, M. O., Boron, W. F. and Bril, A. (2001) Inhibition of the cardiac electrogenic sodium bicarbonate cotransporter reduces ischemic injury. Cardiovasc. Res. 52, 387-396
174. -. Nat. Med. 7, 292-293
175. Wine, J. J. (2001) Cystic fibrosis: the 'bicarbonate before chloride' hypothesis. Curr. Biol. 11, R463-R466
176. Rosenstein, B. J. and Zeitlin, P. L. (1998) Cystic fibrosis. Lancet 351, 277-282
177. Scheele, G. A., Fukuoka, S. I., Kern, H. F. and Freedman, S. D. (1996) Pancreatic dysfunction in cystic fibrosis occurs as a result of impairments in luminal pH, apical trafficking of zymogen granule membranes, and solubilization of secretory enzymes. Pancreas 12, 1-9
178. Sutton, K. A., Jungnickel, M. K. and Florman, H. M. (2003) Of fertility, cystic fibrosis and the bicarbonate ion. Nat. Cell Biol. 5, 857-859
179. - transport in mutations associated with cystic fibrosis. Nature 410, 94-97
180. Song, Y., Salinas, D., Nielson, D. W. and Verkman, A. S. (2006) Hyperacidity of secreted fluid from submucosal glands in early cystic fibrosis. Am. J. Physiol. Cell Physiol. 290, C741-C749
181. - cotransport isoform 3. J. Biol. Chem. 277, 50503-50509
182. - secretion: relevance to cystic fibrosis. EMBO J. 25, 5049-5057
183. Steward, M. C., Ishiguro, H. and Case, R. M. (2005) Mechanisms of bicarbonate secretion in the pancreatic duct. Annu. Rev. Physiol. 67, 377-409
184. Hess, K. C., Jones, B. H., Marquez, B., Chen, Y., Ord, T. S., Kamenetsky, M., Miyamoto, C., Zippin, J. H., Kopf, G. S., Suarez, S. S. et al. (2005) The 'soluble' adenylyl cyclase in sperm mediates multiple signaling events required for fertilization. Dev. Cell 9, 249-259
185. Medina, J. F., Recalde, S., Prieto, J., Lecanda, J., Saez, E., Funk, C. D., Vecino, P., van Roon, M. A., Ottenhoff, R., Bosma, P. J. et al. (2003) Anion exchanger 2 is essential for spermiogenesis in mice. Proc. Natl. Acad. Sci. U.S.A. 100, 15847-15852
186. -cotransporter in mouse sperm capacitation. J. Biol. Chem. 278, 7007-7009
187. Meizel, S. and Deamer, D. W. (1978) The pH of the hamster sperm acrosome. J. Histochem. Cytochem. 26, 98-105
188. Zeng, Y., Clark, E. N. and Florman, H. M. (1995) Sperm membrane potential: hyperpolarization during capacitation regulates zona pellucida-dependent acrosomal secretion. Dev. Biol. 171, 554-563
189. Chen, Y., Cann, M. J., Litvin, T. N., Iourgenko, V., Sinclair, M. L., Levin, L. R. and Buck, J. (2000) Soluble adenylyl cyclase as an evolutionarily conserved bicarbonate sensor. Science 289, 625-628
190. Xu, W. M., Shi, Q. X., Chen, W. Y., Zhou, C. X., Ni, Y., Rowlands, D. K., Yi Liu, G., Zhu, H., Ma, Z. G., Wang, X. F. et al. (2007) Cystic fibrosis transmembrane conductance regulator is vital to sperm fertilizing capacity and male fertility. Proc. Natl. Acad. Sci. U.S.A. 104, 9816-9821
191. Schulz, S., Jakubiczka, S., Kropf, S., Nickel, I., Muschke, P. and Kleinstein, J. (2006) Increased frequency of cystic fibrosis transmembrane conductance regulator gene mutations in infertile males. Fertil. Steril. 85, 135-138
192. Kopito, L. E., Kosasky, H. J. and Shwachman, H. (1973) Water and electrolytes in cervical mucus from patients with cystic fibrosis. Fertil. Steril. 24, 512-516
193. Oppenheimer, E. A., Case, A. L., Esterly, J. R. and Rothberg, R. M. (1970) Cervical mucus in cystic fibrosis: a possible cause of infertility. Am. J. Obstet. Gynecol. 108, 673-674
194. - exchanger in the mouse oocyte is inactivated during first meiotic metaphase and reactivated after egg activation via the MAP kinase pathway. Mol. Biol. Cell 13, 3800-3810
195. Fitzharris, G. and Baltz, J. M. (2006) Granulosa cells regulate intracellular pH of the murine growing oocyte via gap junctions: development of independent homeostasis during oocyte growth. Development 133, 591-599
196. - cotransporter. J. Biol. Chem. 272, 19111-19114
197. - cotransporter. Nature 387, 409-413
198. Abuladze, N., Lee, I., Newman, D., Hwang, J., Boorer, K., Pushkin, A. and Kurtz, I. (1998) Molecular cloning, chromosomal localization, tissue distribution, and functional expression of the human pancreatic sodium bicarbonate cotransporter. J. Biol. Chem. 273, 17689-17695
199. 3- cotransporter isoform (hhNBC). Am. J. Physiol. 276, C576-C584
200. - cotransporter (NBC) with a novel COOH-terminus, cloned from rat brain. Am. J. Physiol. Cell Physiol. 278, C1200-C1211
201. - cotransporter (NBC) in human corneal endothelium. Exp. Eye Res. 77, 287-295
202. Pushkin, A., Abuladze, N., Newman, D., Lee, I., Xu, G. and Kurtz, I. (2000) Cloning, characterization and chromosomal assignment of NBC4, a new member of the sodium bicarbonate cotransporter family. Biochim. Biophys. Acta 1493, 215-218
203. Sassani, P., Pushkin, A., Gross, E., Gomer, A., Abuladze, N., Dukkipati, R., Carpenito, G. and Kurtz, I. (2002) Functional characterization of NBC4: a new electrogenic sodium-bicarbonate cotransporter. Am. J. Physiol. Cell Physiol. 282, C408-C416
204. - cotransporter (NBCe2). Am. J. Physiol. Cell Physiol. 282, C1278-C1289
205. - cotransporter NBC4 in rat kidney and characterization of a novel NBC4 variant. Am. J. Physiol. Renal Physiol. 284, F41-F50
206. Ishibashi, K., Sasaki, S. and Marumo, F. (1998) Molecular cloning of a new sodium bicarbonate cotransporter cDNA from human retina. Biochem. Biophys. Res. Commun. 246, 535-538
207. Pushkin, A., Abuladze, N., Lee, I., Newman, D., Hwang, J. and Kurtz, I. (1999) Cloning, tissue distribution, genomic organization, and functional characterization of NBC3, a new member of the sodium bicarbonate cotransporter family. J. Biol. Chem. 274, 16569-16575
208. Choi, I., Aalkjaer, C., Boulpaep, E. L. and Boron, W. F. (2000) An electroneutral sodium/bicarbonate cotransporter NBCn1 and associated sodium channel. Nature 405, 571-575
209. - transporter superfamily is an apical anion exchanger of beta-intercalated cells in the kidney. J. Biol. Chem. 276, 8180-8189
210. + driven anion exchanger (NDAE1): a new bicarbonate transporter. J. Biol. Chem. 275, 24552-24559
211. - cotransporter isoform NBC-3 (kNBC-3): cloning, expression, and renal distribution. Kidney Int. 59, 1405-1414
212. Praetorius, J., Nejsum, L. N. and Nielsen, S. (2004) A SCL4A10 gene product maps selectively to the basolateral plasma membrane of choroid plexus epithelial cells. Am. J. Physiol. Cell Physiol. 286, C601-C610
213. Schweinfest, C. W., Spyropoulos, D. D., Henderson, K. W., Kim, J. H., Chapman, J. M., Barone, S., Worrell, R. T., Wang, Z. and Soleimani, M. (2006) Slc26a3 (dra)-deficient mice display chloride-losing diarrhea, enhanced colonic proliferation, and distinct up-regulation of ion transporters in the colon. J. Biol. Chem. 281, 37962-37971
214. + exchanger. J. Biol. Chem. 274, 22855-22861
215. Barmeyer, C., Ye, J. H., Sidani, S., Geibel, J., Binder, H. J. and Rajendran, V. M. (2007) Characteristics of rat downregulated in adenoma (rDRA) expressed in HEK 293 cells. Pflugers Arch. 454, 441-450
216. Scott, D. A., Wang, R., Kreman, T. M., Sheffield, V. C. and Karnishki, L. P. (1999) The Pendred syndrome gene encodes a chloride-iodide transport protein. Nat. Genet. 21, 440-443
217. - exchanger in the kidney cortex. Am. J. Physiol. Renal Physiol. 280, F356-F364
218. Waldegger, S., Moschen, I., Ramirez, A., Smith, R. J., Ayadi, H., Lang, F. and Kubisch, C. (2001) Cloning and characterization of SLC26A6, a novel member of the solute carrier 26 gene family. Genomics 72, 43-50
219. - exchanger in the small intestine. Am. J. Physiol. Gastrointest. Liver Physiol. 282, G573-G579
220. Xie, Q., Welch, R., Mercado, A., Romero, M. F. and Mount, D. B. (2002) Molecular characterization of the murine Slc26a6 anion exchanger: functional comparison with Slc26a1. Am. J. Physiol. Renal Physiol. 283, F826-F838
221. Vincourt, J. B., Jullien, D., Kossida, S., Amalric, F. and Girard, J. P. (2002) Molecular cloning of SLC26A7, a novel member of the SLC26 sulfate/ anion transporter family, from high endothelial venules and kidney. Genomics 79, 249-256
222. Bruce, L. J., Cope, D. L., Jones, G. K., Schofield, A. E., Burley, M., Povey, S., Unwin, R. J., Wrong, O. and Tanner, M. J. (1997) Familial distal renal tubular acidosis is associated with mutations in the red cell anion exchanger (Band 3, AE1) gene. J. Clin. Invest. 100, 1693-1707
223. Gallagher, P. G. and Forget, B. G. (1997) Hematologically important mutations: band 3 and protein 4.2 variants in hereditary spherocytosis. Blood Cells Mol. Dis. 23, 417-421
224. Schofield, A. E., Reardon, D. M. and Tanner, M. J. (1992) Defective anion transport activity of the abnormal band 3 in hereditary ovalocytic red blood cells. Nature 355, 836-838
225. Inaba, M., Yawata, A., Koshino, I., Sato, K., Takeuchi, M., Takakuwa, Y., Manno, S., Yawata, Y., Kanzaki, A., Sakai, J. et al. (1996) Defective anion transport and marked spherocytosis with membrane instability caused by hereditary total deficiency of red cell band 3 in cattle due to a nonsense mutation. J. Clin. Invest. 97, 1804-1817
226. Hunt, S. C., Xin, Y., Wu, L. L., Cawthon, R. M., Coon, H., Hasstedt, S. J. and Hopkins, P. N. (2006) Sodium bicarbonate cotransporter polymorphisms are associated with baseline and 10-year follow-up blood pressures. Hypertension 47, 532-536
227. Barkley, R. A., Chakravarti, A., Cooper, R. S., Ellison, R. C., Hunt, S. C., Province, M. A., Turner, S. T., Weder, A. B. and Boerwinkle, E. (2004) Positional identification of hypertension susceptibility genes on chromosome 2. Hypertension 43, 477-482
228. Hoglund, P., Haila, S., Socha, J., Tomaszewski, L., Saarialho-Kere, U., Karjalainen-Lindsberg, M. L., Airola, K., Holmberg, C., de la Chapelle, A. and Kere, J. (1996) Mutations of the down-regulated in adenoma (DRA) gene cause congenital chloride diarrhoea. Nat. Genet. 14, 316-319
229. Zhu, Q., Lee, D. W. K. and Casey, J. R. (2003) Novel topology in C-terminal region of the human plasma membrane anion exchanger, AE1. J. Biol. Chem. 278, 3112-3120