Epithelial transport in polycystic kidney disease

Physiological Reviews

Volumn 78, Issue 4, 1998, Pages 1165-1191

  Sullivan, Lawrence P ^a   Wallace, Darren P ^a   Grantham, Jared J ^a  

^a UNIVERSITY OF KANSAS MEDICAL CENTER   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

BICARBONATE; CHLORIDE ION; CYCLIC AMP; TRANSMEMBRANE CONDUCTANCE REGULATOR;

ANIMAL CELL; ANIMAL EXPERIMENT; ANIMAL MODEL; AUTOSOMAL DOMINANT INHERITANCE; CELL TRANSPORT; CONTROLLED STUDY; CYST FLUID; DOG; EPITHELIUM CELL; HUMAN; HUMAN CELL; ION EXCHANGE; KIDNEY EPITHELIUM; KIDNEY POLYCYSTIC DISEASE; LIVER DISEASE; LUNG ALVEOLUS EPITHELIUM; NONHUMAN; PRIORITY JOURNAL; RAT; REVIEW;

EID: 0031753210     PISSN: 00319333     EISSN: None     Source Type: Journal    
DOI: 10.1152/physrev.1998.78.4.1165     Document Type: Review

References (191)

1. AL-AWQATI, Q. Puzzling polycystin. Mol. Med. 2: 663-664, 1996.
2. ALPER, S. L., A. K. STUART-TILLEY, D. BIEMESDERFER, B. E. SCMUKLER, AND D. BROWN. Immunolocalization of AE2 anion exchanger in rat kidney. Am. J. Physiol. 273 (Renal Physiol. 42): F601-F614, 1997.
3. ALTON, E. W. F. W. Applied cell biology. In: Cystic Fibrosis, edited by M. E. Hodson and D. M. Geddes. London: Chapman & Hall, 1995, p. 41-73.
4. AVNER, E. D., W. E. SWEENEY, JR., AND W. J. NELSON. Abnormal sodium pump distribution during renal tubulogenesis in congenital murine polycystic kidney disease. Proc. Natl. Acad. Sci. USA 89: 7447-7451, 1992.
5. AVNER, E. D., W. E. SWEENEY, JR., N. P. PIESCO, AND D. ELLIS. A new model of glucocorticoid-induced cystic metanephric maldevelopment. Experientia 40: 489-490, 1984.
6. BACHINSKY, D. R., I. SABOLIC, D. S. EMMANOUEL, D. M. JEFFERSON, F. A. CARONE, D. BROWN, AND R. D. PERRONE. Water channel expression in human ADPKD kidneys. Am. J. Physiol. 268 (Renal Fluid Electrolyte Physiol. 37): F398-F403, 1995.
7. BAERT, L. Hereditary polycystic kidney disease (adult form): a microdissection study of two cases at an early stage of the disease. Kidney Int. 13: 519-525, 1978.
8. BARAC-NIETO, M., AND J. J. COHEN. Nonesterified fatty acid uptake by dog kidney: effects of probenecid and chlorothiazide. Am. J. Physiol. 215: 98-105, 1968.
9. BARISONI, L., M. TRUDEL, N. CHRETIEN, L. WARD, J. VAN ADELSBERG, AND V. D'AGATI. Analysis of the role of membrane polarity in polycystic kidney disease of transgenic SBM mice. Am. J. Pathol. 147: 1728-1735, 1995.
10. BELL, P. E., K. F. HOSSACK, P. A. GABOW, J. A. DURR, A. M. JOHNSON, AND R. W. SCHRIER. Hypertension in autosomal dominant polycystic kidney disease. Kidney Int. 34: 683-690, 1988.
11. BENNETT, W. M., L. ELZINGA, J. P. PULLIAM, A. L. RASHAD, AND J. M. BARRY. Cyst fluid antibiotic concentrations in autosomal dominant polycystic kidney disease. Am. J. Kidney Dis. 6: 400-404, 1985.
12. BERNSTEIN, J. A classification of renal cysts. In: Cystic Disease of the Kidney, edited by K. D. Gardner. New York: Wiley, 1976, p. 7-30.
13. BERNSTEIN, J. Morphology of human renal cystic disease. In: Chronic Renal Disease, edited by N. B. Cummings and S. Klahr. New York: Plenum, 1985, p. 47-54.
14. BIHOREAU, M. T., I. CECCHERINI, J. BROWNE, B. KRANZLIN, G. ROMEO, G. M. LATHROP, M. R. JAMES, AND N. GRETZ. Location of the first genetic locus, PKDr1 controlling autosomal dominant polycystic kidney disease in Han:SPRD cy/+ rat. Hum. Mol. Genet. 4: 609-613, 1997.
15. BIRENBOIM, N., V. S. DONOSO, R. A. HUSEMAN, AND J. J. GRANTHAM. Renal excretion and cyst accumulation of β2microglobulin in polycystic kidney disease. Kidney Int. 31: 85-92, 1987.
16. BJERLE, P., B. LINDQVIST, AND G. MICHAELSON. Pressure measurements in renal cysts. Scand. J. Clin. Lab. Invest. 27: 135-138, 1971.
17. BRASIER, J. L., AND E. P. HENSKE. Loss of the polycystic kidney disease (PKD1) region of chromosome 16p13 in renal cyst cells supports a loss-of-function model for cyst pathogenesis. J. Clin. Invest. 99: 194-199, 1997.
18. BREUER, W. V., E. MACK, AND A. ROTHSTEIN. Activation of K and Cl channels by Ca and cyclic AMP in dissociated kidney epithelial (MDCK) cells. Pflügers Arch. 411: 450-455, 1988.
19. BRICKER, N. S., AND J. F. PATTON. Cystic disease of the kidneys. A study of dynamics and chemical composition of cyst fluid. Am. J. Med. 18: 207-219, 1955.
20. BRILL, S. R., K. E. ROSS, C. J. DAVIDOW, M. YE, J. J. GRANTHAM, AND M. J. CAPLAN. Immunolocalization of ion transport proteins in human autosomal dominant polycystic kidney epithelial cells. Proc. Natl. Acad. Sci. USA 93: 10206-10211, 1996.
21. - secretion in a cultured epithelium of renal origin (MDCK). Q. J. Exp. Physiol. 71: 183-193, 1986.
22. - secretion in MDCK cell epithelium. Biochim. Biophys. Acta 649: 427-435, 1981.
23. BURN, T. C., T. D. CONNORS, W. R. DACKOWSKI, L. R. PETRY, T. J. VAN RAAY, J. M. MILLHOLLAND, M. VENET, G. MILLER, R. M. HAKIM, G. M. LANDES, K. W. KLINGER, F. QIAN, L. F. ONUCHIC, T. WATNICK, G. G. GERMlNO, AND N. A. DOGGETT. Analysis of the genomic sequence for the autosomal dominant polycystic kidney disease (PKD1) gene predicts the presence of a leucine-rich repeat. The American PKD1 consortium. Hum. Mol. Genet. 4: 575-582, 1995.
24. CALVET, J. P. Molecular genetics of polycystic kidney disease. J. Nephrol. 11: 24-34, 1998.
25. CARONE, F. A., S. NAKAMURA, M. CAPUTO, R. BACALLAO, W. J. NELSON, AND Y. S. KANWAR. Cell polarity in human renal cystic disease. Lab. Invest. 70: 648-655, 1994.
26. CARONE, F. A., S. NAKAMURA, P. PUNYARIT, Y. S. KANWAR, AND W. J. NELSON. Sequential tubular cell and basement membrane changes in polycystic kidney disease. J. Am. Soc. Nephrol. 3: 244-253, 1992
27. CARONE, F. A., S. OZONO, S. SAMMA, Y. S. KANWAR, AND R. OYASU. Renal functional changes in experimental cystic disease are tubular in origin. Kidney Int. 33: 8-13, 1988.
28. CARONE, F. A., J. STOLARCZYK, F. A. KRUMLOVSKY, S. G. PERLMAN, T. H. ROBERTS, AND R. G. ROWLAND. The nature of a drug-induced renal concentrating defect in rats. Lab. Invest. 31: 658-664, 1974.
29. CHAPMAN, A. B., A. JOHNSON, P. A. GABOW, AND R. W. SCHRIER. The renin-angiotensin-aldosterone system and autosomal dominant polycystic kidney disease. N. Engl. J. Med. 323: 1091-1096, 1990.
30. CHESTER, A. C., K. GEOLY, G. E. SCHREINER, AND H. G. PREUSS. Early diagnosis of polycystic kidney disease. Am. Family Physician 23: 175-181, 1981.
31. COHN, J. A., T. V. STRONG, M. R. PICCIOTTO, A. C. NAIRN, F. S. COLLINS, AND J. G. FITZ. Localization of the cystic flbrosis transmembrane conductance regulator in human bile duct epithelial cells. Gastroenterology 105: 1857-1864, 1993.
32. CORNELL, S. C. Angiography in polycystic disease of the kidney. J. Urol. 103: 24-26, 1970.
33. COWLEY, B. D., JR., AND V. H. GATTONE II. In vivo models of PKD in non-murine species. In: Polycystic Kidney Disease, edited by M. L. Watson and V. E. Torres. New York: Oxford Univ. Press, 1996, p. 88-110.
34. COWLEY, B. D., JR., S. GUDAPATY, A. L. KRAYBILL, B. D. BARASH, M. A. HARDING, J. P. CALVET, AND V. H. GATTONE II. Autosomal dominant polycystic kidney disease in the rat. Kidney Int. 43: 522-534, 1993.
35. CUPPAGE, F. E., R. A. HUSEMAN, A. CHAPMAN, AND J. J. GRANTHAM. Ultrastructure and function of cysts from human adult polycystic kidneys. Kidney Int. 17: 372-381, 1980.
36. CUTHBERT, A. W., A. M. GEORGE, AND L. MACVINISH. Kinin effects on electrogenic ion transport in primary cultures of pig renal papillary collecting tubule cells. Am. J. Physiol. 249 (Renal Fluid Electrolyte Physiol. 18): F439-F447, 1985.
37. D'ANGELO, A., G. MIONI, E. OSSI, A. LUPO, E. VALVO, AND G. MASCHIO. Alterations in renal tubular sodium and water transport in polycystic kidney diseases. Clin. Nephrol. 3: 99-105, 1975.
38. DANIELSEN, H., A. H. NIELSEN, E. B. PEDERSEN, P. HERLEVSEN, H. J. KORNERUP, AND V. POSBORG. Exaggerated natriuresis in adult polycystic kidney disease. Acta Med. Scand. 219: 59-66, 1986.
39. DAOUST, M. C., D. M. REYNOLDS, D. G. BICHET, AND S. SOMLO. Evidence for a third genetic locus for autosomal dominant polycystic kidney disease. Genomics 25: 733-736, 1995.
40. DAVIDOW, C. J., R. L. MASER, L. A. ROME, J. P. CALVET, AND J. J. GRANTHAM. The cystic fibrosis transmembrane conductance regulator mediates transepithelial fluid secretion by human autosomal dominant polycystic kidney disease epithelium in vitro. Kidney Int. 50: 208-218, 1996.
41. DELPIRE, E., M. I. RAUCHMAN, D. R. BEIER, S. C. HEBERT, AND S. R. GULLANS. Molecular cloning and chromosome localization of a putative basolateral Na-K-2Cl cotransporter from mouse inner medullary collecting duct (mIMCD-3) cells. J. Biol. Chem. 269: 25677-25683, 1994.
42. DEVUYST, O., C. R. BURROW, B. L. SMITH, P. AGRE, M. A. KNEPPER, AND P. D. WILSON. Expression of aquaporins-1 and -2 during nephrogenesis and in autosomal dominant polycystic kidney disease. Am. J. Physiol. 271 (Renal Fluid Electrolyte Physiol. 40): F169-F183, 1996.
43. DOUSA, T. P., R. G. ROWLAND, AND F. A. CARONE. Renal medullary adenylate cyclase in drug-induced nephrogenic diabetes insipidus. Proc. Soc. Exp. Biol. Med. 142: 720-772, 1973.
44. ECKARDT, K. U., M. MOLLMANN, R. NEUMANN, R. BRUNKHORST, H. U. BURGER, G. LONNEMANN, H. SCHOLZ, G. KEUSCH, B. BUCHHOLZ, U. FREI, C. BAUER, AND A. KURTZ. Erythropoietin in polycystic kidneys. J. Clin. Invest. 84: 1160-1166, 1989.
45. - secretion but no cation inward conductance. Pflügers Arch. 431: 427-434, 1996.
46. ETTINGER, A., P. C. KAHN, AND H. M. WISE, JR. The importance of selective renal angiography in the diagnosis of polycystic disease. J. Urol. 102: 156-161, 1969.
47. EUROPEAN POLYCYSTIC KIDNEY DISEASE CONSORTIUM. The polycystic kidney disease 1 gene encodes a 14 kb transcript and lies within a duplicated region on chromosome 16. Cell 77: 881-894, 1994.
48. EVAN, A. P., AND K. D. GARDNER, JR. Nephron obstruction in nordihydroguaiaretic acid-induced renal cystic disease. Kidney Int. 15: 7-19 1979.
49. EVAN, A. P., AND K. D. GARDNER, JR. Morphology of polycystic kidney disease in man and experimental models. In: Chronic Renal Disease, edited by N. B. Cummings and S. Klahr. New York: Plenum, 1985, p. 55-68.
50. EVAN, A. P., K. D. GARDNER, JR., AND J. BERNSTEIN. Polypoid and papillary epithelial hyperplasia: a potential cause of ductal obstruction in adult polycystic disease. Kidney Int. 16: 743-750, 1979.
51. EVERSON, G. T., M. EMMETT, W. R. BROWN, P. REDMOND, AND D. THICKMAN. Functional similarities of hepatic cystic and biliary epithelium: studies of fluid constituents and in vivo secretion in response to secretin. Hepatology 11: 557-565, 1990.
52. FICK, G. M., AND P. A. GABOW. Natural history of autosomal dominant polycystic kidney disease. Annu. Rev. Med. 45: 23-29, 1994.
53. FORBUSH, B., III, M. HAAS, AND C. LYTLE. Na-K-Cl cotransport in the shark rectal gland. I. Regulation in the intact perfused gland. Am. J. Physiol 262 (Cell Physiol. 31): C1000-C1008, 1992.
54. GABOW, P. A., A. B. CHAPMAN, A. M. JOHNSON, D. J. TANGEL, I. T. DULEY, W. D. KAEHNY, M. MANCO-JOHNSON, AND R. W. SCHRIER. Renal structure and hypertension in autosomal dominant polycystic kidney disease. Kidney Int. 38: 1177-1180, 1990.
55. GABOW, P. A., AND J. J. GRANTHAM. Polycystic kidney disease. In: Diseases of the Kidney (6th ed.), edited by R. W. Schrier and C. W. Gottschalk. Boston: Little, Brown, 1996, p. 521-560.
56. GABOW, P. A., A. M. JOHNSON, W. D. KAEHNY, W. J. KIMBERLING, D. C. LEZOTTE, I. T. DULEY, AND R. H. JONES. Factors affecting the progression of renal disease in autosomal dominant polycystic kidney disease. Kidney Int. 41: 1311-1319, 1992.
57. GABOW, P. A., A. M. JOHNSON, W. D. KAEHNY, M. L. MANCO-JOHNSON, I. T. DULEY, AND G. T. EVERSON. Risk factors for the development of hepatic cysts in autosomal dominant polycystic kidney disease. Hepatology 11: 1033-1037, 1990.
58. GABOW, P. A., W. D. KAEHNY, A. M. JOHNSON, I. T. DULEY, M. MANCO-JOHNSON, D. C. LEZOTTE, AND R. W. SCHRIER. The clinical utility of renal concentrating capacity in polycystic kidney disease. Kidney Int. 35: 675-680, 1989.
59. GARDNER, K. D., JR. Composition of fluid in twelve cysts of a polycystic kidney. N. Engl. J. Med. 281: 985-988, 1969.
60. GARDNER, K. D., JR., J. S. BURNSIDE, B. J. SKIPPER, S. K. SWAN, W. M. BENNETT, B. A. CONNORS, AND A. P. EVAN. On the probability that kidneys are different in autosomal dominant polycystic disease. Kidney Int. 42: 1199-1206, 1992.
61. GARDNER, K. D., JR., R. H. GLEW, A. P. EVAN, J. A. McATEER, AND J. BERNSTEIN. Why renal cysts grow. Am. J. Physiol. 266 (Renal Fluid Electrolyte Physiol. 35): F353-F359, 1994.
62. GATTONE, V. H., II, J. P. CALVET, B. D. COWLEY, JR., A. P. EVAN, T. S. SHAVER, K. HELMSTADTER, AND J. J. GRANTHAM. Autosomal recessive polycystic kidney disease in a murine model. A gross and microscopic description. Lab. Invest. 59: 231-238, 1988.
63. GATTONE, V. H., II, B. D. COWLEY, JR., B. D. BARASH, S. NAGAO, H. TAKAHASHI, T. YAMAGUCHI, AND J. J. GRANTHAM. Methylprednisolone retards the progression of inherited polycystic kidney disease in rodents. Am. J. Kidney Dis. 25: 302-313, 1995.
64. GATTONE, V. H., II, AND J. J. GRANTHAM. Understanding human cystic disease through experimental models. Semin. Nephrol. 11: 617-631, 1991.
65. GENG, L., Y. SEGAL, B. PEISSEL, N. DENG, Y. PEI, F. CARONE, H. G. RENNKE, A. M. GLUCKSMANN-KUIS, M. C. SCHNEIDER, M. ERICSSON, S. T. REEDERS, AND J. ZHOU. Identification and localization of polyoystin, the PKD1 gene product. J. Clin. Invest. 98: 2674-2682, 1996.
66. GRAHAM, P. C., AND G. B. M. LINDOP. The anatomy of the renin-secreting cell in adult polycystic kidney disease. Kidney Int. 33: 1084-1090, 1988.
67. GRANTHAM, J. J. Fluid secretion, cellular proliferation, and the pathogenesis of renal epithelial cysts. J. Am. Soc. Nephrol. 3: 1841-1857, 1993.
68. GRANTHAM, J. J. The etiology, pathogenesis, and treatment of autosomal dominant polycystic kidney disease: recent advances. Am. J. Kidney Dis. 28: 788-803, 1996.
69. GRANTHAM, J. J., V. S. DONOSO, A. P. EVAN, F. A. CARONE, AND K. D. GARDNER, JR. Viscoelastic properties of tubule basement membranes in experimental renal cystic disease. Kidney Int. 32: 187-197, 1987.
70. GRANTHAM, J. J., J. L. GEISER, AND A. P. EVAN. Cyst formation and growth in autosomal dominant polycystic kidney disease. Kidney Int. 31: 1145-1152, 1987.
71. GRANTHAM, J. J., P. B. QUALIZZA, AND R. L. IRWIN. Net fluid secretion in proximal straight renal tubules in vitro: role of PAH. Am. J. Physiol. 226: 191-197, 1974.
72. GRANTHAM, J. J., G. F. SCHREINER, L. ROME, L. GRENZ, AND A. JOLY. Evidence for inflammatory and secretagogue lipids in cyst fluids from patients with autosomal dominant polycystic kidney disease. Proc. Assoc. Am. Phys. 109: 397-408, 1997.
73. GRANTHAM, J. J., M. UCHIC, E. J. CRAGOE, JR., J. KORNHAUS, J. A. GRANTHAM, V. DONOSO, R. MANGOO-KARIM, A. EVAN, AND J. McATEER. Chemical modification of cell proliferation and fluid secretion in renal cysts. Kidney Int. 35: 1379-1389, 1989.
74. GRANTHAM, J. J., M. YE, C. DAVIDOW, B. HOLUB, AND M. SHARMA. Evidence for a potent lipid secretagogue in the cyst fluids of patients with autosomal dominant polycystic kidney disease. J. Am. Soc. Nephrol. 6: 1242-1249, 1995.
75. GRANTHAM, J. J., M. YE, V. H. GATTONE II, AND L. P. SULLIVAN. In vitro fluid secretion by epithelium from polycystic kidneys. J. Clin. Invest. 95: 195-202, 1995.
76. GREGER, R., M. BLEICH, J. LEIPZIGER, D. ECKE, M. MALL, AND K. KUNZELMANN. Regulation of ion transport in colonie crypts. News Physiol. Sci. 12: 62-66, 1997.
77. GREGER, R. E., AND F. SCHLATTER. Mechanism of NaCl secretion in rectal gland tubules of spiny dogfish (Squalus acanthias). 2. Effect of inhibitors. Pflügers Arch. 402: 364-375, 1984.
78. GRIFFIN, M. D., V. E. TORRES, J. P. GRANDE, AND R. KUMAR. Immunolocalization of polycystin in human tissues and cultured cells. Proc. Assoc. Am. Phys. 108: 185-197, 1996.
79. GRIFFIN, M. D., V. E. TORRES, AND R. KUMAR. Cystic kidney diseases. Curr. Opin. Nephrol. Hypertens. 6: 276-283, 1997.
80. GRIMM, P. C., J. F. CROCKER, D. A. MALATJALIAN, AND M. R. OGBORN. The microanatomy of the intracepatic bile duct in polycystic disease: comparison of the cpk mouse and human. J. Exp. Pathol. 71: 119-131, 1990.
81. GRUBMAN, S. A., R. D. PERRONE, D. W. LEE, S. L. MURRAY, L. C. ROGERS, L. I. WOLKOFF, A. E. MULBERG, V. CHERINGTON, AND D. M. JEFFERSON. Regulation of intracellular pH by immortalized human intrahepatic biliary epithelial cell lines. Am. J. Physiol. 266 (Gastrointest. Liver Physiol. 29): G1060-G1070, 1994.
82. HAAS, M. The Na-K-Cl cotransporters. Am. J. Physiol. 267 (Cell Physiol. 36): C869-C885, 1994.
83. 3H]bumetanide binding study. Am. J. Physiol. 259 (Cell Physiol. 28): C557-C569, 1990.
84. HANAOKA, K., O. DEVUYST, E. M. SCHWIEBERT, P. D. WILSON, AND W. B. GUGGINO. A role for CFTR in human autosomal dominant polycystic kidney disease. Am. J. Physiol. 270 (Cell Physiol. 39): C389-C399, 1996.
85. HAYASHI, M., Y. YAMAJI, T. MONKAWA, T. YOSHIDA, H. TSUGANEZAWA, H. SASAMURA, W. KITAJIMA, S. SASAKI, K. ISHIBASHI, F. MAURMO, AND T. SARUTA. Expression and localization of the water channels in human autosomal dominant polycystic kidney disease. Nephron 75: 321-326, 1997.
86. HEGGO, O. A microdissection study of cystic disease of the kidneys in adults. J. Pathol. Bacteriol. 91: 311-315, 1966.
87. HUGHES, J., C. J. WARD, B. PERAL, R. ASPINWALL, K. CLARK, J. L. SAN-MILLAN, V. GAMBLE, AND P. C. HARRIS. The polycystic kidney disease 1 (PKD1) gene encodes a novel protein with multiple cell recognition domains. Nature Genet. 10: 151-160, 1995.
88. HUSEMAN, R., A. GRADY, D. WELLING, AND J. GRANTHAM. Macropuncture study of polycystic disease in adult human kidneys. Kidney Int. 18: 375-385, 1980.
89. HUSTED, R. F., K. A. VOLK, R. D. SIGMUND, AND J. B. STOKES. Anion secretion by the inner medullary collecting duct. Evidence for involvement of the cystic flbrosis transmembrane conductance regulator. J. Clin. Invest. 95: 644-650, 1995.
90. IBRAGHIMOV-BESKROVNAYA, O., W. R. DACKOWSKI, L. FOGGENSTEINER, N. COLEMAN, S. THIRU, L. R. PETRY, T. C. BURN, T. D. CONNORS, T. VANRAAY, J. BRADLEY, F. QIAN, L. F. ONUCHIC, T. J. WATNICK, K. PIONTEK, R. M. HAKIM, G. M. LANDES, G. GERMINO, R. SANDFORD, AND K. W. KLINGER. Polycystin: in vitro synthesis, in vivo tissue expression, and subcellular localization identifies a large membrane associated protein. Proc. Natl. Acad. Sci. USA 94: 6397-6402, 1997.
91. INTERNATIONAL POLYCYSTIC KIDNEY DISEASE CONSORTIUM. Polycystic kidney disease: the complete structure of the PKD1 gene and its protein. Cell 81: 289-298, 1995.
92. JACOBSSON, L., B. LINDQVlST, G. MICHAELSON, AND P. BJERLE. Fluid turnover in renal cysts. Acta Med. Scand. 202: 327-329, 1977.
93. KAPLAN, M. R., D. B. MOUNT, E. DELPIRE, G. GAMBA, AND S. C. HEBERT. Molecular mechanisms of NaCl cotransport. Annu. Rev. Physiol. 58: 649-668, 1996.
94. KAPLAN, M. R., M. D. PLOTKIN, D. BROWN, S. C. HEBERT, AND E. DELPIRE. Expression of the mouse Na-K-2Cl cotransporter, mBSC2, in the terminal inner medullary collecting duct, the glomerular and extraglomerular mesangium, and the glomerular afferent arteriole. J. Clin. Invest. 98: 723-730 1996.
95. KASPAREIT-RITTINGHAUSEN, J., F. DEERBERG, AND A. WCISLO. Hereditary polycystic kidney disease: adult kidney disease associated with renal hypertension, renal osteodystrophy, and uremic enteritis in SPRD rats. Am. J. Pathol. 139: 693-696, 1991.
96. KASPAREIT-RITTINGHAUSEN, J., K. RAPP, F. DEERBERG, A. WCISLO, AND C. MESSOW. Hereditary polycystic kidney disease associated with osteorenal syndrome in rats. Vet. Pathol. 26: 195-201, 1989.
97. KAWA G., S. NAGAO, A. YAMAMOTO, K. OMORI, Y. KOMATZ, H. TAKAHASHI, AND Y. TASHIRO. Sodium pump distribution is not reversed in the DBA/2FG-pcy, polycystic kidney disease model mouse. J. Am. Soc. Nephrol. 4: 2040-2049, 1994.
98. KEES-FOLTS, D., J. L. SADOW, AND G. F. SCHRIENER. Tubular catabolism of albumin is associated with the release of an inflammatory lipid. Kidney Int. 45: 1697-1709, 1994.
99. KIMBERLING, W. J., S. KUMAR, P. A. GABOW, J. B. KENYON, C. J. CONOLLY, AND S. SOMLO. Autosomal dominant polycystic kidney disease: localization of the second gene to chromosome 4q13-123. Genomics 18: 467-472, 1993.
100. KIZER, N. L., B. LEWIS, AND B. A. STANTON. Electrogenic sodium absorption and chloride secretion by an inner medullary collecting duct cell line (mIMCD-K2). Am. J. Physiol. 268 (Renal Fluid Electrolyte Physiol. 37): F347-F355, 1995.
101. KIZER, N. L., D. VANDORPE, B. LEWIS, B. BUNTING, J. RUSSELL, AND B. A. STANTON. Vasopressin and cAMP stimulate electrogenic chloride secretion in an IMCD cell line. Am. J. Physiol. 268 (Renal Fluid Electrolyte Physiol. 37): F854-F861, 1995.
102. KOLB, H. A., C. D. A. BROWN, AND H. MURER. Identification of a voltage-dependent anion channel in the apical membrane of a Cl-secretory epithelium (MDCK). Pflügers Arch. 403: 262-265, 1985.
103. LAMBERT, P. P. Polycystic disease of the kidney. A review. Arch. Pathol. 44: 34-58, 1947.
104. LANG, F., M. DEFREGGER, AND M. PAULMICHL. Apparent chloride conductance of subconfluent Madin Darby canine kidney cells. Pflügers Arch. 407: 158-162, 1986.
105. LENZEN, R., A. GIANFRANCO, AND N. TAVOLONI. Secretin stimulates bile ductular secretory activity through the cAMP system. Am. J. Physiol. 263 (Gastrointest. Liver Physiol. 26): G527-G532, 1992.
106. LIEDTKE, C. M. Regulation of chloride transport in epithelia. Annu. Rev. Physiol. 51: 143-160, 1989.
107. LYTLE, C., AND B. FORBUSH III. Na-K-Cl cotransport in the shark rectal gland. II. Regulation in isolated tubules. Am. J. Physiol. 262 (Cell Physiol. 31): C1009-C1017, 1992.
108. MACIAS, W. L., J. A. McATEER, G. A. TANNER, A. L. FRITZ, AND W. M. ARMSTRONG. NaCl transport by Madin Darby canine kidney cyst epithelial cells. Kidney Int. 42: 308-319, 1992.
109. MACLEOD, R. J., J. R. HAMILTON, H. KOPELMAN, AND N. B. SWEEZEY. Developmental differences of cystic flbrosis transmembrane conductance regulator functional expression in isolated rat fetal distal airway epithelial cells. Pediatr. Res. 35: 45-49, 1994.
110. MANGOO-KARIM, R., L. P. SULLIVAN, M. YE, AND J. J. GRANTHAM. In vitro models in the study of renal cystogenesis. In: Polycystic Kidney Disease, edited by M. L. Watson and V. E. Torres New York: Oxford Univ. Press, 1996, p. 39-61.
111. MANGOO-KARIM, R., M. E. UCHIC, M. GRANT, W. A. SHUMATE, J. P. CALVET, C. H. PARK, AND J. J. GRANTHAM. Renal epithelial fluid secretion and cyst growth: the role of cyclic AMP. FASEB J. 3: 2629-2632, 1989.
112. MANGOO-KARIM, R., M. UCHIC, C. LECHENE, AND J. J. GRANTHAM. Renal epithelial cyst formation and enlargement in vitro: dependence on cAMP. Proc. Natl. Acad. Sci. USA 86: 6007-6011, 1989.
113. MANGOO-KARIM, R., M. YE, D. P. WALLACE, J. J. GRANTHAM, AND L. P. SULLIVAN. Anion secretion drives fluid secretion by monolayers of cultured human polycystic cells. Am. J. Physiol. 269 (Renal Fluid Electrolyte Physiol 38): F381-F388, 1995.
114. MARTINEZ-ANSO, E., J. E. CASTILLO, J. DIEZ, J. F. MEDINA, AND J. PRIETO. Immunohistochemical detection of chloride/bicarbonate anion exchangers in human liver. Hepatology 19: 1400-1406, 1994.
115. MARTINEZ-MALDONADO, M. Functional aspects: electrolyte and uric acid excretion. In: Problems in Diagnosis and Management of Polycystic Kidney Disease, edited by K. D. Gardner, Jr., and J. J. Grantham. Kansas City, KS: PKR Found., 1985, p. 70-80.
116. MARTINEZ-MALDONADO, M., J. J. YIUM, G. EKNOYAN, AND W. N. SUKI. Adult polycystic kidney disease: studies of the defect in urine concentration. Kidney Int. 2: 107-113, 1972.
117. McATEER, J. A., F. A. CARONE, J. J. GRANTHAM, S. A. KEMPSON, K. D. GARDNER, JR., AND A. P. EVAN. Explant culture of human polycystic kidney. Lab. Invest. 59: 126-136, 1988.
118. McATEER, J. A., A. P. EVAN, AND E. L. GARDNER. Morphogenetic clonal growth of kidney epithelial cell line, MDCK. Anat. Rec. 217: 229-239, 1987.
119. McDONALD, R. A., AND E. D. AVNER. Mouse models of polycystic kidney disease. In: Polycystic Kidney Disease, edited by M. L. Watson and V. E. Torres. New York: Oxford Univ. Press, 1996, p. 64-87.
120. MILUTINOVIC, J., L. C. AGODOA, R. E. CUTLER, AND G. E. STRIKER. Autosomal dominant polycystic kidney disease. Early diagnosis and consideration of pathogenesis. Am. J. Clin. Pathol. 73: 740-747, 1980.
121. MOCHIZUKI, T., G. WU, T. HAYASHI, S. L. XENOPHONTOS, B. VELDHUISEN, J. J. SARIS, D. M. REYNOLDS, Y. CAI, P. A. GABOW, A. PIERIDES, W. J. KIMBERLlNG, M. H. BREUNING, C. C. DELTAS, D. J. PETERS, AND S. SOMLO. PKD2, a gene for polycystic kidney disease that encodes an integral membrane protein. Science 272: 1339-1342, 1996.
122. NAUTA, J., M. A. GOEDBLOED, T. M. LUIDER, A. T. HOOGEVEEN, A. M. VAN DEN OUWELAND, AND D. J. HALLEY. The Han:SPRD rat is not a genetic model of human autosomal dominant polycystic kidney disease type 1. Lab. Anim. 31: 241-247, 1997.
123. NEUFELD, T. K., D. DOUGLASS, M. GRANT, M. YE, F. SILVA, T. NADASDY, AND J. J. GRANTHAM. In vitro formation and expansion of cysts derived from human renal cortex epithelial cells. Kidney Int. 41: 1222-1236, 1992.
124. NEUFELD, T. K., M. E. GRANT, AND J. J. GRANTHAM. A method to measure the rate of net fluid secretion by monolayers of cultured renal epithelial cells. J. Tissue Culture Methods 13: 229-234, 1991.
125. NEUFELD, T. K., AND J. J. GRANTHAM. Epidermal growth factor promotes cyst formation by human renal epithelial cells in vitro. Trans. Assoc. Am. Phys. 80: 48-52, 1990.
126. + channel expression is one of the pathogenetic mechanisms leading to human neonatal respiratory distress syndrome. Proc. Assoc. Am. Phys. 108: 345-355, 1996.
127. OGBORN, M. R., S. SAREEN, AND P. C. GRIMM. Renal tubule Na,K-ATPase polarity in glucocorticoid-induced polycystic kidney disease. J. Histochem. Cytochem. 41: 555-558, 1993.
128. OGBORN, M. R., S. SAREEN, J. PRYCHITKO, R. BUIST, AND J. PEELING. Altered organic anion and osmolyte content and excretion in rat polycystic kidney disease: an NMR study. Am. J. Physiol. 272 (Renal Physiol. 41): F63-F69, 1997.
129. OGBORN, M. R., S. SAREEN, K. TOMOBE, H. TAKAHASHI, AND J. F. CROCKER. Renal tubule Na,K-ATPase polarity in different animal models of polycystic kidney disease. J. Histochem. Cytochem. 43: 785-790, 1995.
130. PALSSON, R., C. P. SHARMA, K. KIM, M. McLAUGHLIN, D. BROWN, AND M. A. ARNAOUT. Characterization and cell distribution of polycystin, the product of autosomal dominant polycystic kidney disease gene 1. Mol. Med. 2: 702-711, 1996.
131. PARFREY, P. S., J. C. BEAR, J. MORGAN, B. C. CRAMER, P. J. McMANAMON, M. H. GAULT, D. N. CHURCHILL, M. SINGH, R. HEWITT, S. SOMLO, AND S. T REEDERS. The diagnosis and prognosis of autosomal dominant polycystic kidney disease. N. Engl. J. Med. 323: 1085-1090, 1990.
132. PATTERSON, M., J. C. GONZALEZ-VITALE, AND C. J. FAGAN. Polycystic liver disease: a study of cyst fluid constituents. Hepatology 2: 475-478, 1982.
133. PERRONE, R. D. In vitro function of cyst epithelium from human polycystic kidney. J. Clin. Invest. 76: 1688-1691, 1985.
134. PERRONE, R. D., S. A. GRUBMAN, S. L. MURRAY, D. W. LEE, S. L. ALPER, AND D. M. JEFFERSON. Autosomal dominant polycystic kidney disease decreases anion exchanger activity. Am. J. Physiol. 272 (Cell Physiol. 41): C1748-C1756, 1997.
135. PERRONE, R. D., S. A. GRUBMAN, L. C. ROGERS, D. W. LEE, E. MOY, S. L. MURRAY, V. E. TORRES, AND D. M. JEFFERSON. Continuous epithelial cell lines from ADPKD liver cysts exhibit characteristics of intrahepatic biliary epithelium. Am. J. Physiol. 269 (Gastrointest. Liver Physiol. 32): G335-G345, 1995.
136. PERRONE, R. D., AND M. L. McLAUGHLIN. Cyst function in polycystic kidney disease: nongradient cysts. Clin. Nephrol. 32: 113-118, 1989.
137. PETER, K. Rekonstruktionsmethoden. In: Handbuch der biologischen Arbeits-methoden, edited by E. Abderhalden. Vienna: Urban & Schwarzenberg, 1921, vol. 9, p. 1.
138. POTTER, E. L. Normal and Abnormal Development of the Kidney. Chicago, IL: Year Book, 1972, p. 182-208.
139. PREUSS, H., K. GEOLY, M. JOHNSON, A. CHESTER, A. KLIGER, AND G. SCHREINER. Tubular function in adult polycystic kidney disease. Nephron 24: 198-204, 1979.
140. QIAN, F., F. J. GERMINO, Y. CAI, X. ZHANG, S. SOMLO, AND G. G. GERMINO. PKD1 interacts with PKD2 through a probable coiled-coil domain. Nature Genet. 16: 179-183, 1997.
141. QIAN, F., T. J. WATNICK, L. F. ONUCHIC, AND G. G. GERMINO. The molecular basis of focal cyst formation in human autosomal dominant polycystic kidney disease type I. Cell 87: 979-987, 1996.
142. RAMOS, A., V. E. TORRES, K. E. HOLLEY, K. P. OFFORD, J. RAKELA, AND E. HOLLEY. The liver in autosomal dominant polycystic kidney disease. Arch. Pathol. Lab. Med. 114: 180-184, 1990.
143. REEDERS, S. T., M. H. BREUNING, K. E. DAVIES, R. D. NICHOLS, A. P. JARMAN, D. R. HIGGS, P. L. PEARSON, AND D. J. WEATHERALL. A highly polymorphic DNA marker linked to adult polycystic kidney disease on chromosome 16. Nature 317: 542-544, 1985.
144. RIORDAN, J. R. The cystic flbrosis transmembrane conductance regulator. Annu. Rev. Physiol. 55: 609-630, 1993.
145. ROCHA, A. S., AND L. H. KUDO. Factors governing sodium and chloride transport across the inner medullary collecting duct. Kidney Int. 38: 654-667, 1990.
146. SCHAEFER, K., N. GRETZ, M. BADER, I. OBERBAEUMER, K. U. ECKARDT, W. KRIZ, AND S. BACHMANN. Characterization of the Han:SPRD rat model for hereditary polycystic kidney disease. Kidney Int. 46: 134-152, 1994.
147. SCHMID, M., J. F. E. MANN, G. STEIN, M. HERTER, J. NUSSBERGER, A. KLINGBEIL, AND E. RITZ. Natriuresis-pressure relationship in polycystic kidney disease. J. Hypertens. 8: 277-283, 1990.
148. SCHNERMANN, J., J. BRIGGS, AND G. SCHUBERT. In situ studies of the distal convoluted tubule in the rat. I. Evidence for NaCl secretion. Am. J. Physiol. 243 (Renal Fluid Electrolyte Physiol. 12): F160-F106, 1982.
149. SCHREINER, G. F. Renal toxicity of albumin and other lipoproteins. Curr. Opin. Nephrol. Hypertens. 4: 369-373, 1995.
150. SCHWAB, S., D. HINTHORN, D. DIEDERICH, F. CUPPAGE, AND J. GRANTHAM. pH-dependent accumulation of clindamycin in a polycystic kidney. Am. J. Kidney Dis. 3: 63-66, 1983.
151. - secretion by exogenous ATP in cultured MDCK epithelial monolayers. Biochim. Biophys. Acta 646: 231-242, 1981.
152. 1 and by forskolin in a canine renal epithelial cell line. J. Physiol. (Lond.) 432: 459-472, 1991.
153. - secretion. Exp. Physiol. 78: 117-137, 1993.
154. SONNENBERG, H. Secretion of salt and water into the medullary collecting duct of Ringer-infused rats. Am. J. Physiol. 228: 565-568, 1975.
155. STANTON, B. A. Cystic flbrosis transmembrane conductance regulator (CFTR) and renal function. Wien. Klin. Wochenschr. 109: 457-464, 1997.
156. STEIN, J. H., R. W. OSGOOD, AND R. T. KUNAU. Direct measurement of papillary collecting duct sodium transport in the rat. Evidence for heterogeneity of nephron function during Ringer loading. J. Clin. Invest. 58: 767-773, 1976.
157. SULLIVAN, L. P., AND J. J. GRANTHAM. Specificity of basolateral organic anion exchange in proximal tubule for cellular and extracellular solutes. J. Am. Soc. Nephrol. 2: 1192-1200, 1992.
158. SULLIVAN, L. P., D. P. WALLACE, AND J. J. GRANTHAM. Coupling of cell volume and membrane potential changes to fluid secretion in a model of renal cysts. Kidney Int. 45: 1369-1380, 1994.
159. SULLIVAN, L. P., D. P. WALLACE, AND J. J. GRANTHAM. Chloride and fluid secretion in polycystic kidney disease. J. Am. Soc. Nephrol. 9: 903-916, 1998.
160. TAKAHASHI, H., J. P. CALVET, D. DITTEMORE-HOOVER, K. YOSHIDA, J. J. GRANTHAM, AND V. H. GATTONE II. A hereditary model of slowly progressive polycystic kidney disease in the mouse. J. Am. Soc. Nephrol. 1: 980-981, 1991.
161. TAKAHASHI, M., K. TSUCHIYA, Y. KOMATSU, AND H. NIHEI. A role for Na/K adenosine triphosphatase in the pathogenesis of cyst formation in experimental polycystic kidney disease. J. Lab. Clin. Med. 129: 517-526, 1997.
162. TANNER, G. A., N. GRETZ, B. A. CONNORS, A. P. EVAN, AND M. STEINHAUSEN. Role of obstruction in autosomal dominant polycystic kidney disease in rats. Kidney Int. 50: 873-886, 1996.
163. TANNER, G. A., N. GRETZ, Y. Z. SHAO, A. P. EVAN, AND M. STEINHAUSEN. Organic anion secretion in polycystic kidney disease. J. Am. Soc. Nephrol. 8: 1222-1231, 1997.
164. TANNER, G. A., M. R. MAXWELL, AND J. A. McATEER. Fluid transport in a cultured cell model of kidney epithelial cyst enlargement. J. Am. Soc. Nephrol. 2: 1208-1218, 1991.
165. TANNER, G. A., AND J. A. McATEER. Measurement of cell volume and fluid transport in MDCK cysts by video microscopy. J. Tissue Culture Methods 13: 235-240, 1991.
166. TANNER, G. A., P. F. McQUILLAN, M. R. MAXWELL, J. K. KECK, AND J. A. McATEER. An in vitro test of the cell stretch-proliferation hypothesis of renal cyst enlargement. J. Am. Soc. Nephrol. 6: 1230-1241, 1995.
167. TAUB, M., L. CHUMAN, M. H. SAIER, AND G. SATO. Growth of Madin-Darby canine kidney epithelial cell (MDCK) line in hormone-supplemented, serum-free medium. Proc. Natl. Acad. Sci. USA 76: 3338-3342, 1979.
168. TORRES, V. E., D. S. KEITH, K. P. OFFORD, S. P. KON, AND D. M. WILSON. Renal ammonia in autosomal dominant polycystic kidney disease. Kidney Int. 45: 1745-1753, 1994.
169. TORRES, V. E., D. M. WILSON, J. C. BURNETT, JR., C. M. JOHNSON, AND K. P. OFFORD. Effect of inhibition of converting enzyme on renal hemodynamics and sodium management in polycystic kidney disease. Mayo Clin. Proc. 66: 1010-1017, 1991.
170. TORRES, V. E., D. M. WILSON, K. P. OFFORD, J. C. BURNETT, JR., AND J. C. ROMERO. Natriuretic response to volume expansion in polycystic kidney disease. Mayo Clin. Proc. 64: 509-515, 1989.
171. TRIMBLE, M. E. Mediated transport of long-chain fatty acids by rat renal basolateral membranes. Am. J. Physiol. 257 (Renal Fluid Electrolyte Physiol. 26): F539-F546, 1989.
172. TSIOKAS, L., E. KIM, T. ARNOULD, Y. P. SUKHATME, AND G. WALZ. Homo and hetero-dimeric interactions between the gene products of Pkd1 and Pkd2. Proc. Natl. Acad. Sci. USA 94: 6965-6970, 1997.
173. ULLRICH, K. J., G. RUMRICH, AND S. KLOSS. Contraluminal para-aminohippurate transport in the proximal tubule of the rat kidney. III. Specificity: monocarboxylic acids. Pflügers Arch. 409: 547-554, 1987.
174. VAN ADELSBERG, J., S. CHAMBERLAIN, AND V. D'AGATI. Polycystin expression is temporally and spatially regulated during renal development. Am. J. Physiol. 272 (Renal Physiol. 41): F602-F609, 1997.
175. VAN ADELSBERG, J. S., AND D. FRANK. The PKD1 gene produces a developmentally regulated protein in mesenchyme and vasculature. Nature Med. 1: 359-364, 1995.
176. WAITMAN, A. M., W. P. DYCK, AND H. D. JANOWITZ. Effect of secretin and acetazolamide on the volume and electrolyte composition of hepatic bile in man.
177. WALL, S. M., J. M. SANDS, M. F. FLESSNER, H. NONOGUCHI, K. R. SPRING, AND M. A. KNEPPER. Net acid transport by isolated perfused inner medullary collecting ducts. Am. J. Physiol. 258 (Renal Fluid Electrolyte Physiol. 27): F75-F84, 1990.
178. WALLACE, D. P., J. J. GRANTHAM, AND L. P. SULLIVAN. Chloride and fluid secretion by cultured human polycystic kidney cells. Kidney Int. 50: 1327-1336, 1996.
179. WELLING, L. W., AND J. J. GRANTHAM. Physical properties of isolated perfused renal tubules and tubular basement membranes. J. Clin. Invest. 51: 1063-1075, 1972.
180. WELLING, L. W., AND D. J. WELLING. Kinetics of cyst development in cystic renal disease. In: Chronic Renal Disease, edited by N. B. Cummings and S. Klahr. New York: Plenum, 1985, p. 95-103.
181. WELSH, M. Barium inhibition of basolateral membrane potassium conductance in tracheal epithelium. Am. J. Physiol, 244 (Renal Fluid Electrolyte Physiol. 13): F639-F645, 1983.
182. WICKRE, C. G., AND W. M. BENNETT. Renal cyst epithelial transport in non-uremic polycystic kidney disease. Kidney Int. 23: 514-518, 1983.
183. WILSON, P. D. Pathogenesis of polycystic kidney disease: altered cell function. In: Polycystic Kidney Disease, edited by M. L. Watson and V. E. Torres. New York: Oxford Univ. Press, 1996, p. 125-166.
184. WILSON, P. D. Epithelial cell polarity and disease. Am. J. Physiol. 272 (Renal Physiol. 41): F434-F442, 1997.
185. WILSON, P. D., R. W. SCHRIER, R. D. BRECKON, AND P. A. GABOW. A new method for studying human polycystic kidney disease epithelia in culture. Kidney Int. 30: 371-378, 1986.
186. +-ATPase: mislocation to apical plasma membranes in polycystic kidney disease epithelia. Am. J. Physiol. 260 (Renal Fluid Electrolyte Physiol. 29): F420-F430, 1991.
187. WINGO, C. S. Active and passive chloride transport by the rabbit cortical collecting duct. Am. J. Physiol. 258 (Renal Fluid Electrolyte Physiol. 27): F1388-F1393, 1990.
188. YAMAGUCHI, T., S. NAGAO, H. TAKAHASHI, M. YE, AND J. J. GRANTHAM. Cyst fluid from a murine model of polycystic kidney disease stimulates fluid secretion, cyclic adenosine monophosphate accumulation, and cell proliferation by Madin-Darby canine kidney cells in vitro. Am. J. Kidney Dis. 25: 471-477, 1995.
189. YE, M., M. GRANT, M. SHARMA, L. ELZINGA, S. SWAN, V. E. TORRES, AND J. J. GRANTHAM. Cyst fluid from human autosomal dominant polycystic kidneys promotes cyst formation and expansion by renal epithelial cells in vitro. J. Am. Soc. Nephrol. 3: 984-994, 1992.
190. YE, M., AND J. J. GRANTHAM. The secretion of fluid by renal cysts from patients with autosomal dominant polycystic kidney disease. N. Engl. J. Med. 329: 310-313 1993.
191. ZHANG, C., R. F. HUSTED, AND J. B. STOKES. Effect of cAMP agonists on cell pH and anion transport by cultured rat inner medullary collecting duct cells. Am. J. Physiol. 270 (Renal Fluid Electrolyte Physiol. 39): F131-F140, 1996.