From structure to disease: The evolving tale of aquaporin biology

Nature Reviews Molecular Cell Biology

Volumn 5, Issue 9, 2004, Pages 687-698

  King, Landon S ^a   Kozono, David ^a   Agre, Peter ^a  

^a NONE

Author keywords

[No Author keywords available]

Indexed keywords

AQUAPORIN; MEMBRANE PROTEIN; MONOMER; TETRAMER;

CELL MEMBRANE; CHANNEL GATING; EYE; HOMEOSTASIS; MEMBRANE CHANNEL; MEMBRANE PERMEABILITY; PRIORITY JOURNAL; PROTEIN EXPRESSION; PROTEIN LOCALIZATION; PROTEIN STRUCTURE; REVIEW; WATER HOMEOSTASIS; WATER PERMEABILITY; WATER TRANSPORT;

ANIMALS; AQUAPORINS; ASTROCYTES; CELL MEMBRANE; EYE; HOMEOSTASIS; HUMANS; KIDNEY; PERMEABILITY; PHYLOGENY; PROTEIN CONFORMATION; PROTEIN ISOFORMS; RESPIRATORY SYSTEM; TISSUE DISTRIBUTION; WATER;

EID: 4444221676     PISSN: 14710072     EISSN: None     Source Type: Journal    
DOI: 10.1038/nrm1469     Document Type: Review

References (142)

1. Agre, P. et al. Aquaporin CHIP: the archetypal molecular water channel. Am. J. Physiol. 265, F463-F476 (1993).
2. Preston, G. M., Carroll, T. P., Guggino, W. B. & Agre, P. Appearance of water channels in Xenopus oocytes expressing red cell CHIP28 protein. Science 256, 385-387 (1992). This is the initial report of the discovery and biophysical characterization of aquaporin-1, the first molecular water channel to be identified.
3. Loo, D. D., Wright, E. M. & Zeuthen, T. Water pumps. J. Physiol. 542, 53-60 (2002).
4. Borgnia, M., Nielsen, S., Engel, A. & Agre, P. Cellular and molecular biology of the aquaporin water channels. Annu. Rev. Biochem. 68, 425-458 (1999).
5. Koyama, Y. et al. Molecular cloning of a new aquaporin from rat pancreas and liver. J. Biol. Chem. 272, 30329-30333 (1997).
6. Yasui, M. et al. Rapid gating and anion permeability of an intracellular aquaporin. Nature 402, 184-187 (1999). This report highlights the unusual features of AQP6, which, in contrast to most members of the aquaporin family, is an intracellular channel, is gated and is permeated by anions as well as by water.
7. Ishibashi, K. et al. Cloning and functional expression of a second new aquaporin abundantly expressed in testis. Biochem. Biophys. Res. Commun. 237, 714-718 (1997).
8. Ma. T., Yang, B. & Verkman, A. S. Cloning of a novel water and urea-permeable aquaporin from mouse expressed strongly in colon, placenta, liver, and heart. Biochem. Biophys. Res. Commun. 240, 324-328 (1997).
9. Smith, B. L. & Agre, P. Erythrocyte Mr 28,000 transmembrane protein exists as a multisubunit oligomer similar to channel proteins. J. Biol. Chem. 266, 6407-6415 (1991).
10. Verbavatz, J. M. et al. Tetrameric assembly of CHIP28 water channels in liposomes and cell membranes: a freeze-fracture study. J. Cell Biol. 123, 605-618 (1993).
11. Preston, G. M. & Agre, P. Isolation of the cDNA for erythrocyte integral membrane protein of 28 kilodaitons: member of an ancient channel family. Proc. Natl Acad. Sci. USA 88, 11110-11114 (1991).
12. Jung, J. S., Preston, G. M., Smith, B. L., Guggino, W. B. & Agre, P. Molecular structure of the water channel through aquaporin CHIP. The hourglass model. J. Biol. Chem. 269, 14648-14654 (1994).
13. Murata, K. et al. Structural determinants of water permeation through aquaporin-1. Nature 407, 599-605 (2000).
14. Ren, G., Reddy, V. S., Cheng, A., Melnyk, P. & Mitra, A. K. Visualization of a water-selective pore by electron crystallography in vitreous ice. Proc. Natl Acad. Sci. USA 98, 1398-1403 (2001).
15. Fu, D. et al. Structure of a glycerol-conducting channel and the basis for its selectivity. Science 290, 481-486 (2000).
16. Sui, H., Han, B. G., Lee, J. K., Walian, P. & Jap, B. K. Structural basis of water-specific transport through the AQP1 water channel. Nature 414, 872-878 (2001). References 15 and 16 define the atomic structures of GlpF and AQP1, respectively, which are both members of the aquaporin family of proteins.
17. Kozono, D., Yasui, M., King, L. S. & Agre, P. Aquaporin water channels: atomic structure and molecular dynamics meet clinical medicine. J. Clin. Invest 109, 1395-1399 (2002).
18. de Groot, B. L. & Grubmuller, H. Water permeation across biological membranes: mechanism and dynamics of aquaporin-1 and GlpF. Science 294, 2353-2357 (2001).
19. Tajkhorshid, E. et al. Control of the selectivity of the aquaporin water channel family by global orientational tuning. Science 296, 525-530 (2002).
20. Nemeth-Cahalan, K. L. & Hall, J. E. pH and calcium regulate the water permeability of aquaporin O. J. Biol. Chem. 275, 6777-6782 (2000).
21. Gonen, T., Sliz, P., Kistler, J., Cheng, Y. & Walz, T. Aquaporin-O membrane junctions reveal the structure of a closed water pore. Nature 429, 193-197 (2004).
22. -. J. Biol. Chem. 274, 21631-21636 (1999).
23. Zelenina, M., Bondar, A. A., Zelenin, S. & Aperia, A. Nickel and extracellular acidification inhibit the water permeability of human aquaporin-3 in lung epithelial cells. J. Biol. Chem. 278, 30037-30043 (2003).
24. Han, H., Wax, M. B. & Patil, R. V. Regulation of aquaporin-4 water channels by phorbol ester-dependent protein phosphorylation. J. Biol. Chem. 273, 6001-6004 (1998).
25. Zelenia, M., Zelenin, S., Bondar, A. A., Brismar, H. & Aperia. A. Water permeability of aquaporin-4 is deceased by protein kinase C and dopamine. Am. J. Physiol. 283, F309-F318 (2002).
26. Ikeda, M. et al. Characterization of aquaporin-6 as a nitrate channel in mammalian cells. J. Biol. Chem. 277, 39873-39879 (2002).
27. Anthony, T. L. et al. Cloned human aquaporin-1 is a cyclic GMP-gated ion channel. Mol. Pharm. 57, 576-588 (2000).
28. Saparov, S. M., Kozono, D., Rothe, U., Agre, P. & Pohl, P. Water and ion permeation of aquaporin-1 in planar lipid bilayers. J. Biol. Chem. 276, 31515-31520 (2001).
29. 2 permeability of Xenopus oocytes. Am. J. Physiol. 274, C543-548(1998).
30. 2 permeability of Xenopus oocytes expressing aquaporin-1 or its C189S mutant. Am. J. Physiol. 275, C1481-C1486 (1998).
31. 2 across membranes. J. Biol. Chem. 273, 33123-33126 (1998).
32. Yang, B. et al. Carbon dioxide permeability of aquaporin-1 measured in erthrocytes and lung of aquaporin-1 null mice and in reconstituted liposomes. J. Biol. Chem. 275, 2686-2692 (2000).
33. 2 permeability in lung and kidney. J. Physiol. 542, 63-69 (2002).
34. Cooper, G. J., Zhou, Y., Bouyer, P., Grichtchenko, I. I. & Boron, W. F. Transport of volatile solutes through AQP1. J. Physiol. 542, 17-29 (2002).
35. Verkman, A. S. Does aquaporin-1 pass gas? An opposing view. J. Physiol. 542, 31 (2002).
36. 2 permeability on photosynthesis and leaf growth in plants.
37. Nielsen, S. et al. Aquaporins in the kidney: from molecules to medicine. Physiol. Rev. 82, 205-244 (2002).
38. Ma, T. et al. Severely impaired urinary concentrating ability in transgenic mice lacking aquaporin-1 water channels. J. Biol. Chem. 273, 4296-4299 (1998). This description of renal function in Aqp1-null mice provided convincing evidence for a physiological role for AQP1 in the kidney.
39. Schneemann, J. et al. Defective proximal tubular fluid reabsorption in transgenic aquaporin-1 null mice. Proc. Natl Acad. Sci. USA 95, 9660-9664 (1998).
40. Pallone, T. L., Edwards, A., Ma, T., Silldorff, E. P. & Verkman, A. S. Requirement of aquaporin-1 for NaCl-driven water transport across descending vasa recta. J. Clin. Invest. 105, 215-222 (2000).
41. Smith, B. L., Preston, G. M., Spring, F. A., Anstee, D. J. & Agre, P. Human red cell aquaporin CHIP. I. Molecular characterization of ABH and Colton blood group antigens. J. Clin. Invest. 94, 1043-1049 (1994).
42. Preston, G. M., Smith, B. L., Zeidel, M. L., Moulds, J. J. & Agre, P. Mutations in aquaporin-1 in phenotypically normal humans without functional CHIP water channels. Science 265, 1585-1587 (1994).
43. King, L. S., Choi, M., Fernandez, P. C., Cartron, J. P. & Agre, P. Defective urinary-concentrating ability due to a complete deficiency of aquaporin-1. N. Engl. J. Med. 345, 175-179 (2001).
44. Robertson, G. L. Differential diagnosis of polyuria. Annu. Rev. Med. 39, 425-442 (1988).
45. Brown, D. The ins and outs of aquaporin-2 trafficking. Am. J. Physiol. 284, F893-F901 (2003).
46. Wade, J. B., Stetson, D. L. & Lewis, S. A. ADH action: evidence for a membrane shuttle mechanism. Ann. NY Acad. Sci. 372, 106-117 (1981).
47. van Balkom, B. W. et al. Hypertonicity is involved in redirecting the aquaporin-2 water channel into the basolateral, instead of the apical, plasma membrane of renal epithelial cells. J. Biol. Chem. 278, 1101-1107 (2003).
48. Edwards, R. M., Jackson, B. A. & Dousa, T. P. ADH-sensitive cAMP system in papillary collecting duct: effect of osmolality and PGE2. Am. J. Physiol. 240, F311-F318 (1981).
49. Fushimi, K., Sasaki, S. & Marumo, F. Phosphorylation of serine 256 is required for cAMP-dependent regulatory exocytosis of the aquaporin-2 water channel. J. Biol. Chem. 272, 14800-14804 (1997).
50. Katsura, T., Gustafson, C. E., Ausiello, D. A. & Brown, D. Protein kinase A phosphorylation is involved in regulated exocytosis of aquaporin-2 in transfected LLC-PK1 cells. Am. J. Physiol. 272, F817-F822 (1997).
51. Marples, D. et al. Dynein and dynactin colocalize with AQP2 water channels in intracellular vesicles from kidney collecting duct. Am. J. Physiol 274, F384-F394 (1998).
52. Nielsen, S. et al. Expression of VAMP-2-like protein in kidney collecting duct intracellular vesicles. J. Clin. Invest. 96, 1834-1844 (1995).
53. Inoue, T. et al. SNAP-23 in rat kidney: colocalization with aquaporin-2 in collecting duct vesicles. Am. J. Physiol. 275, F752-F760 (1998).
54. Gouraud, S. et al. Functional involvement of VAMP/synaptobrevin-2 in cAMP-stimulated aquaporin 2 translocation in renal collecting duct cells. J. Cell Sci. 115, 3667-3674 (2002).
55. van Balkom, V. W. M. et al. The role of putative phosphorylation sites in the targeting and shuttling of the aquaporin-2 water channel. J. Biol. Chem. 277, 41473-41479 (2003).
56. 1 family is required for cAMP-triggered trafficking of aquaporin 2 in kidney epithelial cells. J. Biol. Chem. 273, 22627-22634 (1998).
57. Klussmann, E. et al. An inhibitory role of rho in the vasopressin-mediated translocation of aquaporin-2 into cell membranes of renal principal cells. J. Biol. Chem. 276, 20451-20457 (2001).
58. Bouley R. et al. Nitric oxide and atrial natriuretic factor stimulate cGMP-dependent membrane insertion of aquaporin 2 in renal epithelial cells. J. Clin. Invest. 106, 1115-1126 (2000).
59. Deen, P. M. et al. Requirement of human renal water channel aquaporin-2 for vasopressin-dependent concentration of urine. Science 264, 92-95 (1994). These investigators provided the first example of an aquaporin mutation that has physiological consequences in humans.
60. Mulders, S. M. et al. An aquaporin-2 water channel mutant which causes autosomal dominant nephrogenic diabetes insipidus is retained in the Golgi complex. J. Clin. Invest. 102, 57-66 (1998).
61. Kamsteeg, E. J., Wormhoudt, T. A., Rijss, J. P., van Os, C. H. & Deen, P. M. An impaired routing of wild-type aquaporin-2 after tetramerization with an aquaporin-2 mutant explains dominant nephrogenic diabetes insipidus. EMBO J. 18, 2394-2400 (1999).
62. Marples, D., Christensen. S., Christensen, E. I., Ottosen, P. D. & Nielsen, S. Lithium-induced downregulation of aquaporin-2 water channel expression in rat kidney medulla. J. Clin. Invest. 95, 1838-1845 (1995).
63. Frøkiær, J., Marples, D., Knepper, M. A. & Nielsen, S. Bilateral ureteral obstruction downregulates expression of vasopressin-sensitive AQP-2 water channel in rat kidney. Am. J. Physiol. 270, F657-F668 (1996).
64. Marples, D., Frøkiær, J., Dorup, J., Knepper, M. A. & Nielsen, S. Hypokalemia-induced downregulation of aquaporin-2 water channel expression in rat kidney medulla and cortex. J. Clin. Invest. 97, 1960-1968 (1996).
65. Nielsen, S. et al. Congestive heart failure in rats is associated with increased expression and targeting of aquaporin-2 water channel in collecting duct. Proc. Natl Acad. Sci. USA 94, 5450-5455 (1997).
66. Schrier, R. W. & Martin, P. Y. Recent advances in the understanding of water metabolism in heart failure. Adv. Exp. Med. Biol. 449, 415-426 (1998).
67. Ma, T. et al. Generation and phenotype of a transgenic knockout mouse lacking the mercurial-insensitive water channel aquaporin-4. J. Clin. Invest. 100, 957-962 (1997).
68. Ma, T. et al. Nephrogenic diabetes insipidus in mice lacking aquaporin-3 water channels. Proc. Natl Acad. Sci. USA 97, 4386-4391 (2000).
69. Roudler, N. et al. AQP3 deficiency in humans and the molecular basis of a novel blood group system, GIL. J. Biol. Chem. 277, 45854-45859 (2002).
70. Nielsen, S., King, L. S., Christensen, B. M. & Agre, P. Aquaporins in complex tissues. II. Subcellular distribution in respiratory and glandular tissues of rat. Am. J. Physiol. 273, C1549-1561 (1997).
71. Kreda, S. M., Gynn, M. C., Fenstermacher, D. A., Boucher, R. C. & Gabriel, S. E. Expression and localization of epithelial aquaporins in the adult human lung. Am. J. Respir. Cell Mol. Biol. 24, 224-234 (2001).
72. Krane, C. M. et al. Aquaporin 5-deficient mouse lungs are hyperresponsive to cholinergic stimulation. Proc. Natl Acad. Sci. USA 98, 14114-14119 (2001).
73. King, L. S. & Agre, P. Man is not a rodent: aquaporins in the airways. Am. J. Respir. Cell Mol. Biol. 24, 221-223 (2001).
74. Borok, Z. et al. Keratinocyte growth factor modulates alveolar epithelial cell phenotype in vitro: expression of aquaporin 5. Am. J. Respir. Cell Mol. Biol. 18, 554-561 (1998).
75. King, L. S., Nielsen, S. & Agre, P. Aquaporin-1 water channel protein in lung: ontogeny, steroid-induced expression, and distribution in rat. J. Clin. Invest. 97, 2183-2191 (1996).
76. King, L. S., Nielsen, S. & Agre, P. Aquaporins in complex tissues. I. Developmental patterns in respiratory and glandular tissues of rat. Am. J. Physiol. 273, C1541-C1548 (1997).
77. Moon, C., King, L. S. & Agre, P. Aqp1 expression in erythroleukemia cells: genetic regulation of glucocorticoid and chemical induction. Am. J. Physiol. 273, C1562-C1570 (1997).
78. Leitch, V., Agre, P. & King, L. S. Altered ubiquitination and stability of aquaporin-1 in hypertonic stress. Proc. Natl Acad. Sci. USA 98, 2894-2898 (2001).
79. Towne, J. E., Krane, C. N., Bachurski, C. J. & Menon, A. G. Tumor necrosis factor-α inhibits aquaporin 5 expression in mouse lung epithelial cells. J. Biol. Chem. 276, 18657-18664 (2001).
80. Yang, F., Kawedia, J. D. & Menon, A. G. Cyclic AMP regulates aquaporin 5 expression at both transcriptional and post-transcriptional levels through a protein kinase A pathway. J. Biol. Chem. 278, 32173-32180 (2003).
81. Hoffert, J. D., Leitch, V., Agre, P. &. King, L. S. Hypertonic induction of aquaporin-5 expression through an ERK-dependent pathway. J. Biol. Chem. 275, 9070-9077 (2000).
82. Jayaraman, S., Song, Y., Vetrivel, L., Shankar, L. & Verkman, A. S. Noninvasive in vivo fluorescence measurement of airway-surface liquid depth, salt concentration, and pH. J. Clin. Invest. 107, 317-324 (2001).
83. Hunt, J. F. et al. Endogenous airway acidification: implication for asthma pathophysiology. Am. J. Respir. Crit. Care Med. 161, 694-699 (2000).
84. Ballard, S. T., Trout. L., Bebok, Z., Sorscher, E. J. & Crews, A. CFTR involvement in chloride, bicarbonate, and liquid secretion by airway submucosal glands. Am. J. Physiol. 277, L694-L699 (1999).
85. Song, Y. & Verkman, A. S. Aquaporin-5 dependent fluid secretion in airway submucosal glands. J. Biol. Chem. 276 41288-41292 (2001).
86. James, A. L., Hogg, J. C., Dunn, L. A. & Pare, P. D. The use of the internal perimeter to compare airway size and to calculate smooth muscle shortening. Am. Rev. Respir. Dis. 138, 136-139 (1988).
87. Anderson, S. D. & Daviskas, E. The mechanism of exercise-induced asthma is... J. Allergy Clin. Immunol. 106, 453-459 (2000).
88. Bai, C. et al. Lung fluid transport in aquaporin-1 and aquaporin-4 knockout mice. J. Clin. Invest 103, 555-561 (1999).
89. King, L. S., Nielsen, S., Agre, P. & Brown, R. H. Decreased pulmonary vascular permeability in aquaporin-1-null humans. Proc. Natl Acad. Sci. USA 99, 1059-1063 (2002).
90. Brown, R. H., Zerhouni, E. A. & Mitzner, W. Visualization of airway obstruction in vivo during pulmonary vascular engorgement and edema. J. Appl. Physiol. 78, 1070-1078 (1995).
91. Dobbs, L. G. et al. Highly water-permeable type I alveolar epithelial cells confer high water permeability between the airspace and vasculature in rat lung. Proc. Natl Acad. Sci. USA 95, 2991-2996 (1998).
92. Bai, C. et al. Lung fluid transport in aquaporin-1 and aquaporin-5 knockout mice. J. Clin. Invest. 103, 555-561 (1999).
93. Ma, T., Fukuda, N., Song, Y., Matthay, M. A. & Verkman, A. S. Lung fluid transport in aquaporin-5 knockout mice. J. Clin. Invest 105, 93-100 (2000).
94. Towne, J. E., Harrod, K. S., Krane, C. M. & Menon, A. G. Decreased expression of aquaporin AQP1 and AQP5 in mouse lung after acute viral infection. Am. J. Respir. Cell Mol. Biol. 22, 34-44 (2000).
95. Leikauf, G. D. et al. Pathogenomic mechanisms for particulate matter induction of acute lung injury and inflammation in mice. Res. Resp. Health Eff. Inst. 105, 5-58 (2001).
96. Ma, T., Fukuda, N., Song, Y., Matthay, M. A. & Verkman, A. S. Lung fluid transport in aquaporin-5 knockout mice. J. Clin. Invest. 105, 93-100 (2000).
97. Song, Y., et al. Role of aquaporins in alveolar fluid clearance in neonatal and adult lung, and in oedema formation following acute lung injury. J. Physiol. 525, 771-779 (2000).
98. Hamann, S. et al. Aquaporins in complex tissues: distribution of aquaporins 1-5 in human and rat eye. Am. J. Physiol. 274, C1332-C1345 (1998).
99. Mulders, S. M. et al. Water channel properties of major intrinsic protein of lens. J. Biol. Chem. 270, 9010-9016 (1995).
100. Shiels, A. & Bassnett, S. Mutations in the founder of the MIP gene family underlie cataract development in the mouse. Nature Genet. 12, 212-215 (1996).
101. Berry, V., Francis, P., Kaushal, S., Moore, A. & Bhattacharya, S. Missense mutations in MIP underlie autosomal dominant 'polymorphic' and lamellar cataracts linked to 12q. Nature Genet. 25, 15-17 (2000).
102. Francis, P. et al. Functional impairment of lens aquaporin in two families with dominantly inherited cataracts. Hum. Mol. Genet. 9, 2329-2334 (2000).
103. Thiagarajah, J. R. & Verkman, A. S. Aquaporin deletion in mice reduces comeal water permeability and delays restoration of transparency after swelling. J. Biol. Chem. 277, 19139-19144 (2002).
104. Zhang, D., Vetrivel, L. & Verkman, A. S. Aquaporin deletion in mice reduces intraocular pressure and aqueous fluid production. J. Gen. Physiol. 119, 561-569 (2002).
105. Raina, S., Preston, G. M., Guggino, W. B. & Agre, P. Molecular cloning and characterization of an aquaporin cDNA from salivary, lacrimal, and respiratory issues. J. Biol. Chem. 270, 1908-1912 (1995).
106. Gresz, V. et al. Identification and localization of aquaporin water channels in human salivary glands. Am. J. Physiol. 281, G247-G254 (2001).
107. Steinfeld, S. et al. Abnormal distribution of aquaporin-5 water channel protein in salivary glands from Sjogren's syndrome patients. Lab. Invest. 81, 143-148 (2001).
108. Tsubota, K., Hirai, S., King, L. S., Agre, P. & Ishida, N. Defective cellular trafficking of lacrimal gland aquaporin-5 in Sjogren's syndrome. Lancet 357, 688-689 (2001).
109. Beroukas, D., Hiscock, J., Jonsson, R., Waterman, S. A. & Gordon, T. P. Subcellular distribution of aquaporin 5 in salivary glands in primary Sjogren's syndrome. Lancet 358, 1875-1876 (2001).
110. Ma, T. et al. Defective secretion of saliva in transgenic mice lacking aquaporin-5 water channels. J. Biol. Chem. 274, 20071-20074 (1999).
111. Krane, C. M. et al. Salivary acinar cells from aquaporin 5-deficient mice have decreased membrane water permeability and altered cell volume regulation. J. Biol. Chem. 276, 23413-23420 (2001).
112. Nejsum, L. M. et al. Functional requirement of aquaporin-5 in plasma membranes of sweat glands. Proc. Natl Acad. Sci. USA 99, 511-516 (2002).
113. Song, Y., Sonawane, N. & Verkman, A. S. Localization of aquaporin-5 in sweat glands and functional analysis using knockout mice. J. Physiol. 54, 561-568 (2002).
114. Moore, M., Ma, T., Yang, B. & Verkman, A. S. Tear secretion by lacrimal glands in transgenic mice lacking water channels AQP1, AQP3, AQP4, and AQP5. Exp. Eye Res. 70, 557-562 (2000).
115. Umenishi, F. & Schrier, R. W. Hypertonicity-induced aquaporin-1 (AQP1) expression is mediated by the actuation of MAPK pathway and hypertonicity-responsive element in the AQP1 gene. J. Biol. Chem. 278, 15765-15770 (2003).
116. Badaut, J., Lasbennes, F., Magistretti, P. J. & Regli, L. Aquaporins in brain: distribution, physiology, and pathophysiology. J. Cereb. Blood Flow Metab. 22, 367-378 (2002).
117. Nielsen, S. et al. Specialized membrane domains for water transport in glial cells: high-resolution immunogold cytochemistry of aquaporin-4 in rat brain. J. Neurosci. 17, 171-180 (1997).
118. Amiry-Moghaddam, M. & Otterson, O. P. The molecular basis of water transport in the brain. Nature Rev. Neurosci. 4, 991-1001 (2003).
119. Amiry-Moghaddam, M. et al. α syntrophin deletion removes the perivascular but not the endothelial pool of aquaporin-4 at the blood-brain barrier and delays the development of brain edema in an experimental model of acute hyponatremia. FASEB J. 18, 542-544 (2004).
120. Neely, J. D. et al. Syntrophin-dependent expression and localization of aquaporin-4 water channel protein. Proc. Natl Acad. Sci. USA 98, 14108-14113 (2001).
121. Hung, A. Y. & Sheng, M. PDZ domains: structural modules for protein complex assembly. J. Biol. Chem. 277, 5699-5702 (2002).
122. Madrid, R. et al. Polarized trafficking and surface expression of the AQP4 water channel are coordinated by serial and regulated interactions with different clathrin-adaptor complexes. EMBO J. 20, 7008-7021 (2001).
123. Monaco, A. P. et al. Isolation of candidate cDNAs for portions of the Duchenne muscular dystophy gene. Nature 323, 646-650 (1986).
124. Frigeri, A. et al. Muscle loading modulates aquaporin-4 expression in skeletal muscle. FASEB J. 15, 1282-1284 (2001).
125. Amiry-Moghadam, M. et al. An α-syntrophin dependent pool of AQP4 in astroglial end-feet confers bi-directional water flow between blood and brain. Proc. Natl Acad. Sci. USA 100, 2106-2111 (2003).
126. Manley, G. T. et al. Aquaporin-4 deletion in mice reduces brain edema after acute water intoxication and ischemic stroke. Nature Med. 6, 159-163 (2000). These researchers show that the absence of AQP4 in the brain is protective against oedema formation in two models of brain oedema.
127. Vajde, Z. et al. Delayed onset of brain edema and mislocalization of aquaporin-4 in dystrophin-null transgenic mice. Proc. Natl Acad. Sci. USA 99, 13131-13136 (2002).
128. Nielsen, S., Smith, B. L., Christensen, E. I. & Agre, P. Distribution of the aquaporin CHIP in secretory and resorptive epithelia and capillary endothelia. Proc. Natl Acad. Sci. USA 90, 7275-7279 (1993).
129. Elkjaer, M. et al. Immunolocalization of AQP9 in liver, epididymis, testis, spleen, and bran. Biochem. Biophys. Res. Commun. 276, 1118-1128 (2000).
130. Ma. T, Hara, M., Sougrat, R., Verbavatz, J.-M. & Verkman, A. S. Impaired stratum corneum hydration in mice lacking epidermal water channel aquaporin-3. J. Biol. Chem. 277, 17147-17153 (2002).
131. Hara, M. & Verkman, A. S. Glycerol replacement corrects defective skin hydration, elasticity and barrier function in aquaporin-3-deficient mice. Proc. Natl Acad. Sci. USA 100, 7360-7365 (2003).
132. Burghardt, B. et al. Distribution of aquaporin water channels AQP1 and AQP5 in the ductal system of the human pancreas. Gut 52, 1006-1016 (2003).
133. Beitz, E., Kumagami, H., Krippeit-Drews, P., Ruppersberg, J. P. & Schultz, J. E. Expression pattern of aquaporin water channels in the inner ear of the rat. Hear. Res. 132, 76-84 (1999).
134. Beitz, E., Zenner, H. P. & Schultz, J. E. Aquaporin-mediated fluid regulation in the inner ear. Cell Mol. Neurobiol. 23, 315-329 (2003).
135. Li, J. & Verkman, A. S. Impaired hearing in mice lacking aquaporin-4 water channels. J. Biol. Chem. 276, 31233-31237 (2001).
136. Richard, C., Gao, J., Brown, N. & Reese, J. Aquaporin water channel genes are differentially expressed and regulated by ovarian steroids during the periimplantation period in the mouse. Endocrinology 144, 1533-1541 (2003).
137. Calamita, G., Mazzone, A., Bizzoca, A. Svelto, M. Possible involvement of aquaporin-7 and -8 in rats testis development and spermatogenesis. Biochem. Biophys. Res. Commun. 288, 619-625 (2001).
138. Kishida, K. et al. Aquaporin adipose, a putative glycerol channel in adipocytes. J. Biol. Chem. 275, 20896-20902 (2000).
139. Kondo, H. et al. Human aquaporin adipose (AQPao) gene. Genomic structure, promoter analysis and functional mutation. Eur. J. Biochem. 269, 1814-1826 (2002).
140. Carbrey, J. M. et al. Aquaglyceroporin AQP9: solute permeation and metabolic control of expression in liver. Proc. Natl Acad. Sci. USA 100, 2945-2950 (2003).
141. Liu, Z. et al. Arsenite transport by mammalian aquaglyceroporins AQP7 and AQP9. Proc. Natl Acad. Sci. USA 99, 6053-6058 (2002).
142. King, L. S. & Yasui, M. Aquaporins and disease: lessons from mice to humans. Trends Endocrin. Metab. 13, 355-360 (2002).