Polycystic kidney diseases are a leading cause of end-stagerenal failure and a common indication for dialysis or renaltransplantation. Recent advances have led to insights into mechanismsunderlying the cause and prognosis of these diseases and suggestnew directions for treatment.
Polycystic kidney disease may arise sporadically as a developmentalabnormality or may be acquired in adult life, but most formsare hereditary. Among the acquired forms, simple cysts can developin kidneys as a consequence of aging; dialysis, drugs, and hormonescan cause multicystic disease1,2; and renal cysts are oftensecondary manifestations of genetic proliferative syndromes.1The . . . [Full Text of this Article]
Autosomal Dominant Polycystic Kidney Disease
Autosomal Recessive Polycystic Kidney Disease
Familial Nephronophthisis
Medullary Cystic Kidney Disease
Cell Biology
Proliferation and Apoptosis
Secretion
CellMatrix Interactions
Polarity
Signal Transduction
Cilia
Molecular Biology
PKD Genes and Mutations
Polycystic Kidney Disease Proteins
Polycystin-1
Polycystin-2
Fibrocystin
Nephrocystin
Functions of Polycystins
Developmental Regulation and Programming
Prospects for Prognosis and Therapy
Source Information
From the Department of Medicine, Division of Nephrology, Mount Sinai School of Medicine, New York.
Address reprint requests to Dr. Wilson at the Department of Medicine, Division of Nephrology, Mount Sinai School of Medicine, 1425 Madison Ave., East Bldg., Rm. 11-23, New York, NY 10029, or at pat.wilson@mssm.edu.
Lubrano, R., Versacci, P., Guido, G., Bellelli, E., Andreoli, G., Elli, M.
(2009). Might there be an association between polycystic kidney desease and noncompaction of the ventricular myocardium?. Nephrol Dial Transplant
24: 3884-3886
[Abstract][Full Text]
(2009). Conditional Mutation of Pkd2 Causes Cystogenesis and Upregulates {beta}-Catenin. J. Am. Soc. Nephrol.
20: 2556-2569
Tao, B., Bu, S., Yang, Z., Siroky, B., Kappes, J. C., Kispert, A., Guay-Woodford, L. M.
(2009). Cystin Localizes to Primary Cilia via Membrane Microdomains and a Targeting Motif. J. Am. Soc. Nephrol.
20: 2570-2580
[Abstract][Full Text]
Lieberthal, W., Levine, J. S.
(2009). The Role of the Mammalian Target Of Rapamycin (mTOR) in Renal Disease. J. Am. Soc. Nephrol.
20: 2493-2502
[Abstract][Full Text]
Wu, M., Arcaro, A., Varga, Z., Vogetseder, A., Le Hir, M., Wuthrich, R. P., Serra, A. L.
(2009). Pulse mTOR inhibitor treatment effectively controls cyst growth but leads to severe parenchymal and glomerular hypertrophy in rat polycystic kidney disease. Am. J. Physiol. Renal Physiol.
297: F1597-F1605
[Abstract][Full Text]
Chen, Z., Kastaniotis, A. J., Miinalainen, I. J., Rajaram, V., Wierenga, R. K., Hiltunen, J. K.
(2009). 17{beta}-Hydroxysteroid dehydrogenase type 8 and carbonyl reductase type 4 assemble as a ketoacyl reductase of human mitochondrial FAS. FASEB J.
23: 3682-3691
[Abstract][Full Text]
Casuscelli, J., Schmidt, S., DeGray, B., Petri, E. T., Celic, A., Folta-Stogniew, E., Ehrlich, B. E., Boggon, T. J.
(2009). Analysis of the cytoplasmic interaction between polycystin-1 and polycystin-2. Am. J. Physiol. Renal Physiol.
297: F1310-F1315
[Abstract][Full Text]
Prasad, S., McDaid, J. P., Tam, F. W. K., Haylor, J. L., Ong, A. C. M.
(2009). Pkd2 Dosage Influences Cellular Repair Responses following Ischemia-Reperfusion Injury. Am. J. Pathol.
175: 1493-1503
[Abstract][Full Text]
Maisonneuve, C., Guilleret, I., Vick, P., Weber, T., Andre, P., Beyer, T., Blum, M., Constam, D. B.
(2009). Bicaudal C, a novel regulator of Dvl signaling abutting RNA-processing bodies, controls cilia orientation and leftward flow. Development
136: 3019-3030
[Abstract][Full Text]
Zafar, I., Belibi, F. A., He, Z., Edelstein, C. L.
(2009). Long-term rapamycin therapy in the Han:SPRD rat model of polycystic kidney disease (PKD). Nephrol Dial Transplant
24: 2349-2353
[Abstract][Full Text]
Happe, H., Leonhard, W. N., van der Wal, A., van de Water, B., Lantinga-van Leeuwen, I. S., Breuning, M. H., de Heer, E., Peters, D. J.M.
(2009). Toxic tubular injury in kidneys from Pkd1-deletion mice accelerates cystogenesis accompanied by dysregulated planar cell polarity and canonical Wnt signaling pathways. Hum Mol Genet
18: 2532-2542
[Abstract][Full Text]
Fischer, D.-C., Jacoby, U., Pape, L., Ward, C. J., Kuwertz-Broeking, E., Renken, C., Nizze, H., Querfeld, U., Rudolph, B., Mueller-Wiefel, D. E., Bergmann, C., Haffner, D.
(2009). Activation of the AKT/mTOR pathway in autosomal recessive polycystic kidney disease (ARPKD). Nephrol Dial Transplant
24: 1819-1827
[Abstract][Full Text]
Wan, R. K., Kipgen, D., Morris, S., Rodger, R. S. C.
(2009). A rare cause of nephrotic syndrome in autosomal-dominant polycystic kidney disease. NDT Plus
2: 136-138
[Abstract][Full Text]
Bae, K. T., Tao, C., Zhu, F., Bost, J. E., Chapman, A. B., Grantham, J. J., Torres, V. E., Guay-Woodford, L. M., Meyers, C. M., Bennett, W. M., and Consortium for Radiologic Imaging Studies Poly,
(2009). MRI-based Kidney Volume Measurements in ADPKD: Reliability and Effect of Gadolinium Enhancement. CJASN
4: 719-725
[Abstract][Full Text]
Cook, S. A., Collin, G. B., Bronson, R. T., Naggert, J. K., Liu, D. P., Akeson, E. C., Davisson, M. T.
(2009). A Mouse Model for Meckel Syndrome Type 3. J. Am. Soc. Nephrol.
20: 753-764
[Abstract][Full Text]
Park, E. Y., Sung, Y. H., Yang, M. H., Noh, J. Y., Park, S. Y., Lee, T. Y., Yook, Y. J., Yoo, K. H., Roh, K. J., Kim, I., Hwang, Y.-H., Oh, G. T., Seong, J. K., Ahn, C., Lee, H.-W., Park, J. H.
(2009). Cyst Formation in Kidney via B-Raf Signaling in the PKD2 Transgenic Mice. J. Biol. Chem.
284: 7214-7222
[Abstract][Full Text]
Hogan, M. C., Manganelli, L., Woollard, J. R., Masyuk, A. I., Masyuk, T. V., Tammachote, R., Huang, B. Q., Leontovich, A. A., Beito, T. G., Madden, B. J., Charlesworth, M. C., Torres, V. E., LaRusso, N. F., Harris, P. C., Ward, C. J.
(2009). Characterization of PKD Protein-Positive Exosome-Like Vesicles. J. Am. Soc. Nephrol.
20: 278-288
[Abstract][Full Text]
Montesano, R., Ghzili, H., Carrozzino, F., Rossier, B. C., Feraille, E.
(2009). cAMP-dependent chloride secretion mediates tubule enlargement and cyst formation by cultured mammalian collecting duct cells. Am. J. Physiol. Renal Physiol.
296: F446-F457
[Abstract][Full Text]
Miller, C. S., McGarity, G. J.
(2009). Tetracycline-induced renal failure after dental treatment. Journal of the American Dental Association
140: 56-60
[Abstract][Full Text]
Li, H. Y., Cosgrove, G. P., Swigris, J. J.
(2009). Dyspnea in a 43-Year-Old Woman With Polycystic Kidney Disease. Chest
135: 238-243
[Full Text]
Hartman, T. R., Liu, D., Zilfou, J. T., Robb, V., Morrison, T., Watnick, T., Henske, E. P.
(2009). The tuberous sclerosis proteins regulate formation of the primary cilium via a rapamycin-insensitive and polycystin 1-independent pathway. Hum Mol Genet
18: 151-163
[Abstract][Full Text]
Romaker, D., Puetz, M., Teschner, S., Donauer, J., Geyer, M., Gerke, P., Rumberger, B., Dworniczak, B., Pennekamp, P., Buchholz, B., Neumann, H.P.H., Kumar, R., Gloy, J., Eckardt, K.-U., Walz, G.
(2009). Increased Expression of Secreted Frizzled-Related Protein 4 in Polycystic Kidneys. J. Am. Soc. Nephrol.
20: 48-56
[Abstract][Full Text]
Islam, M. R., Puri, S., Rodova, M., Magenheimer, B. S., Maser, R. L., Calvet, J. P.
(2008). Retinoic acid-dependent activation of the polycystic kidney disease-1 (PKD1) promoter. Am. J. Physiol. Renal Physiol.
295: F1845-F1854
[Abstract][Full Text]
Kim, I., Li, C., Liang, D., Chen, X.-Z., Coffy, R. J., Ma, J., Zhao, P., Wu, G.
(2008). Polycystin-2 Expression Is Regulated by a PC2-binding Domain in the Intracellular Portion of Fibrocystin. J. Biol. Chem.
283: 31559-31566
[Abstract][Full Text]
Feng, S., Okenka, G. M., Bai, C.-X., Streets, A. J., Newby, L. J., DeChant, B. T., Tsiokas, L., Obara, T., Ong, A. C. M.
(2008). Identification and Functional Characterization of an N-terminal Oligomerization Domain for Polycystin-2. J. Biol. Chem.
283: 28471-28479
[Abstract][Full Text]
Lal, M., Song, X., Pluznick, J. L., Di Giovanni, V., Merrick, D. M., Rosenblum, N. D., Chauvet, V., Gottardi, C. J., Pei, Y., Caplan, M. J.
(2008). Polycystin-1 C-terminal tail associates with {beta}-catenin and inhibits canonical Wnt signaling. Hum Mol Genet
17: 3105-3117
[Abstract][Full Text]
Okada, S., Misaka, T., Tanaka, Y., Matsumoto, I., Ishibashi, K., Sasaki, S., Abe, K.
(2008). Aquaporin-11 knockout mice and polycystic kidney disease animals share a common mechanism of cyst formation. FASEB J.
22: 3672-3684
[Abstract][Full Text]
Park, F., Sweeney, W. E., Jia, G., Roman, R. J., Avner, E. D.
(2008). 20-HETE Mediates Proliferation of Renal Epithelial Cells in Polycystic Kidney Disease. J. Am. Soc. Nephrol.
19: 1929-1939
[Abstract][Full Text]
Battini, L., Macip, S., Fedorova, E., Dikman, S., Somlo, S., Montagna, C., Gusella, G. L.
(2008). Loss of polycystin-1 causes centrosome amplification and genomic instability. Hum Mol Genet
17: 2819-2833
[Abstract][Full Text]
Bergmann, C., Bruchle, N. O., Frank, V., Rehder, H., Zerres, K.
(2008). Perinatal Deaths in a Family with Autosomal Dominant Polycystic Kidney Disease and a PKD2 Mutation. NEJM
359: 318-319
[Full Text]
Edelstein, C. L.
(2008). Mammalian Target of Rapamycin and Caspase Inhibitors in Polycystic Kidney Disease. CJASN
3: 1219-1226
[Abstract][Full Text]
Sweeney, W. E. Jr, von Vigier, R. O., Frost, P., Avner, E. D.
(2008). Src Inhibition Ameliorates Polycystic Kidney Disease. J. Am. Soc. Nephrol.
19: 1331-1341
[Abstract][Full Text]
Yang, B., Sonawane, N. D., Zhao, D., Somlo, S., Verkman, A. S.
(2008). Small-Molecule CFTR Inhibitors Slow Cyst Growth in Polycystic Kidney Disease. J. Am. Soc. Nephrol.
19: 1300-1310
[Abstract][Full Text]
Cadieux, C., Harada, R., Paquet, M., Cote, O., Trudel, M., Nepveu, A., Bouchard, M.
(2008). Polycystic Kidneys Caused by Sustained Expression of Cux1 Isoform p75. J. Biol. Chem.
283: 13817-13824
[Abstract][Full Text]
Burtey, S., Riera, M., Ribe, E., Pennekamp, P., Passage, E., Rance, R., Dworniczak, B., Fontes, M.
(2008). Overexpression of PKD2 in the mouse is associated with renal tubulopathy. Nephrol Dial Transplant
23: 1157-1165
[Abstract][Full Text]
Tao, Y., Zafar, I., Kim, J., Schrier, R. W., Edelstein, C. L.
(2008). Caspase-3 Gene Deletion Prolongs Survival in Polycystic Kidney Disease. J. Am. Soc. Nephrol.
19: 749-755
[Full Text]
Rohatgi, R., Battini, L., Kim, P., Israeli, S., Wilson, P. D., Gusella, G. L., Satlin, L. M.
(2008). Mechanoregulation of intracellular Ca2+ in human autosomal recessive polycystic kidney disease cyst-lining renal epithelial cells. Am. J. Physiol. Renal Physiol.
294: F890-F899
[Abstract][Full Text]
Kim, I., Fu, Y., Hui, K., Moeckel, G., Mai, W., Li, C., Liang, D., Zhao, P., Ma, J., Chen, X.-Z., George, A. L. Jr., Coffey, R. J., Feng, Z.-P., Wu, G.
(2008). Fibrocystin/Polyductin Modulates Renal Tubular Formation by Regulating Polycystin-2 Expression and Function. J. Am. Soc. Nephrol.
19: 455-468
[Abstract][Full Text]
Lantinga-van Leeuwen, I. S., Leonhard, W. N., van der Wal, A., Breuning, M. H., de Heer, E., Peters, D. J.M.
(2007). Kidney-specific inactivation of the Pkd1 gene induces rapid cyst formation in developing kidneys and a slow onset of disease in adult mice. Hum Mol Genet
16: 3188-3196
[Abstract][Full Text]
Ibraghimov-Beskrovnaya, O.
(2007). Molecular pathogenesis of ADPKD and development of targeted therapeutic options. Nephrol Dial Transplant
22: 3367-3370
[Full Text]
Lechler, P., Wu, X., Bernhardt, W., Campean, V., Gastiger, S., Hackenbeck, T., Klanke, B., Weidemann, A., Warnecke, C., Amann, K., Engehausen, D., Willam, C., Eckardt, K.-U., Rodel, F., Wiesener, M. S.
(2007). The Tumor Gene Survivin Is Highly Expressed in Adult Renal Tubular Cells: Implications for a Pathophysiological Role in the Kidney. Am. J. Pathol.
171: 1483-1498
[Abstract][Full Text]
Giorgio, G., Alfieri, M., Prattichizzo, C., Zullo, A., Cairo, S., Franco, B.
(2007). Functional Characterization of the OFD1 Protein Reveals a Nuclear Localization and Physical Interaction with Subunits of a Chromatin Remodeling Complex. Mol. Biol. Cell
18: 4397-4404
[Abstract][Full Text]
Sankaran, D., Bankovic-Calic, N., Ogborn, M. R., Crow, G., Aukema, H. M.
(2007). Selective COX-2 inhibition markedly slows disease progression and attenuates altered prostanoid production in Han:SPRD-cy rats with inherited kidney disease. Am. J. Physiol. Renal Physiol.
293: F821-F830
[Abstract][Full Text]
Kuehn, E. W., Walz, G.
(2007). Prime time for polycystic kidney disease: does one shot of roscovitine bring the cure?. Nephrol Dial Transplant
22: 2133-2135
[Full Text]
Condac, E., Silasi-Mansat, R., Kosanke, S., Schoeb, T., Towner, R., Lupu, F., Cummings, R. D., Hinsdale, M. E.
(2007). Polycystic disease caused by deficiency in xylosyltransferase 2, an initiating enzyme of glycosaminoglycan biosynthesis. Proc. Natl. Acad. Sci. USA
104: 9416-9421
[Abstract][Full Text]
Chapman, A. B.
(2007). Autosomal Dominant Polycystic Kidney Disease: Time for a Change?. J. Am. Soc. Nephrol.
18: 1399-1407
[Abstract][Full Text]
Bernhardt, W. M., Wiesener, M. S., Weidemann, A., Schmitt, R., Weichert, W., Lechler, P., Campean, V., Ong, A. C. M., Willam, C., Gretz, N., Eckardt, K.-U.
(2007). Involvement of Hypoxia-Inducible Transcription Factors in Polycystic Kidney Disease. Am. J. Pathol.
170: 830-842
[Abstract][Full Text]
Chang, M.-Y., Parker, E., El Nahas, M., Haylor, J. L., Ong, A. C.M.
(2007). Endothelin B Receptor Blockade Accelerates Disease Progression in a Murine Model of Autosomal Dominant Polycystic Kidney Disease. J. Am. Soc. Nephrol.
18: 560-569
[Abstract][Full Text]
Rapoport, J.
(2007). Autosomal dominant polycystic kidney disease: pathophysiology and treatment. QJM
100: 1-9
[Full Text]
Nguyen, A.-N. T., Wallace, D. P., Blanco, G.
(2007). Ouabain Binds with High Affinity to the Na,K-ATPase in Human Polycystic Kidney Cells and Induces Extracellular Signal-Regulated Kinase Activation and Cell Proliferation. J. Am. Soc. Nephrol.
18: 46-57
[Abstract][Full Text]
Vagin, O., Tokhtaeva, E., Sachs, G.
(2006). The Role of the beta1 Subunit of the Na,K-ATPase and Its Glycosylation in Cell-Cell Adhesion. J. Biol. Chem.
281: 39573-39587
[Abstract][Full Text]
Battini, L., Fedorova, E., Macip, S., Li, X., Wilson, P. D., Gusella, G. L.
(2006). Stable Knockdown of Polycystin-1 Confers Integrin-{alpha}2beta1-Mediated Anoikis Resistance. J. Am. Soc. Nephrol.
17: 3049-3058
[Abstract][Full Text]
Van Bodegom, D., Saifudeen, Z., Dipp, S., Puri, S., Magenheimer, B. S., Calvet, J. P., El-Dahr, S. S.
(2006). The Polycystic Kidney Disease-1 Gene Is a Target for p53-mediated Transcriptional Repression. J. Biol. Chem.
281: 31234-31244
[Abstract][Full Text]
Xiao, Z., Zhang, S., Mahlios, J., Zhou, G., Magenheimer, B. S., Guo, D., Dallas, S. L., Maser, R., Calvet, J. P., Bonewald, L., Quarles, L. D.
(2006). Cilia-like Structures and Polycystin-1 in Osteoblasts/Osteocytes and Associated Abnormalities in Skeletogenesis and Runx2 Expression. J. Biol. Chem.
281: 30884-30895
[Abstract][Full Text]
Sato, Y., Harada, K., Furubo, S., Kizawa, K., Sanzen, T., Yasoshima, M., Ozaki, S., Isse, K., Sasaki, M., Nakanuma, Y.
(2006). Inhibition of Intrahepatic Bile Duct Dilation of the Polycystic Kidney Rat with a Novel Tyrosine Kinase Inhibitor Gefitinib. Am. J. Pathol.
169: 1238-1250
[Abstract][Full Text]
Joly, D., Berissi, S., Bertrand, A., Strehl, L., Patey, N., Knebelmann, B.
(2006). Laminin 5 Regulates Polycystic Kidney Cell Proliferation and Cyst Formation. J. Biol. Chem.
281: 29181-29189
[Abstract][Full Text]
He, Q.-y., Liu, X.-h., Li, Q., Studholme, D. J., Li, X.-w., Liang, S.-p.
(2006). G8: a novel domain associated with polycystic kidney disease and non-syndromic hearing loss. Bioinformatics
22: 2189-2191
[Abstract][Full Text]
Joly, D., Ishibe, S., Nickel, C., Yu, Z., Somlo, S., Cantley, L. G.
(2006). The Polycystin 1-C-terminal Fragment Stimulates ERK-dependent Spreading of Renal Epithelial Cells. J. Biol. Chem.
281: 26329-26339
[Abstract][Full Text]
Lawson, C. R, Doulton, T. W, MacGregor, G. A
(2006). Review: Autosomal Dominant Polycystic Kidney Disease: Role of the Renin-Angiotensin System in Raised Blood Pressure in Progression of Renal and Cardiovascular Disease. Journal of Renin-Angiotensin-Aldosterone System
7: 139-145
[Abstract]
McGlashan, S. R., Jensen, C. G., Poole, C. A.
(2006). Localization of Extracellular Matrix Receptors on the Chondrocyte Primary Cilium. J. Histochem. Cytochem.
54: 1005-1014
[Abstract][Full Text]
Walz, G.
(2006). Therapeutic approaches in autosomal dominant polycystic kidney disease (ADPKD): is there light at the end of the tunnel?. Nephrol Dial Transplant
21: 1752-1757
[Full Text]
Antiga, L., Piccinelli, M., Fasolini, G., Ene-Iordache, B., Ondei, P., Bruno, S., Remuzzi, G., Remuzzi, A.
(2006). Computed Tomography Evaluation of Autosomal Dominant Polycystic Kidney Disease Progression: A Progress Report. CJASN
1: 754-760
[Abstract][Full Text]
Shannon, M. B., Patton, B. L., Harvey, S. J., Miner, J. H.
(2006). A Hypomorphic Mutation in the Mouse Laminin {alpha}5 Gene Causes Polycystic Kidney Disease. J. Am. Soc. Nephrol.
17: 1913-1922
[Abstract][Full Text]
Wang, W., Mei, C., Tang, B., Zhao, H., Xu, C., Li, Z., Shen, X., Fu, W., Dai, B.
(2006). Aberrant expression of SPARC and its impact on proliferation and apoptosis in ADPKD cyst-lining epithelia. Nephrol Dial Transplant
21: 1278-1288
[Abstract][Full Text]
Shillingford, J. M., Murcia, N. S., Larson, C. H., Low, S. H., Hedgepeth, R., Brown, N., Flask, C. A., Novick, A. C., Goldfarb, D. A., Kramer-Zucker, A., Walz, G., Piontek, K. B., Germino, G. G., Weimbs, T.
(2006). From the Cover: The mTOR pathway is regulated by polycystin-1, and its inhibition reverses renal cystogenesis in polycystic kidney disease. Proc. Natl. Acad. Sci. USA
103: 5466-5471
[Abstract][Full Text]
Gray, M. A.
(2006). Primary cilia and regulation of renal Na+ transport. Focus on "Heightened epithelial Na+ channel-mediated Na+ absorption in a murine polycystic kidney disease model epithelium lacking apical monocilia". Am. J. Physiol. Cell Physiol.
290: C947-C949
[Full Text]
Ross, M. J., Wosnitzer, M. S., Ross, M. D., Granelli, B., Gusella, G. L., Husain, M., Kaufman, L., Vasievich, M., D'Agati, V. D., Wilson, P. D., Klotman, M. E., Klotman, P. E.
(2006). Role of Ubiquitin-Like Protein FAT10 in Epithelial Apoptosis in Renal Disease. J. Am. Soc. Nephrol.
17: 996-1004
[Abstract][Full Text]
Wahl, P. R., Serra, A. L., Le Hir, M., Molle, K. D., Hall, M. N., Wuthrich, R. P.
(2006). Inhibition of mTOR with sirolimus slows disease progression in Han:SPRD rats with autosomal dominant polycystic kidney disease (ADPKD). Nephrol Dial Transplant
21: 598-604
[Abstract][Full Text]
Lee, D. B. N., Huang, E., Ward, H. J.
(2006). Tight junction biology and kidney dysfunction. Am. J. Physiol. Renal Physiol.
290: F20-F34
[Abstract][Full Text]
Bae, K. T., Zhu, F., Chapman, A. B., Torres, V. E., Grantham, J. J., Guay-Woodford, L. M., Baumgarten, D. A., King, B. F. Jr., Wetzel, L. H., Kenney, P. J., Brummer, M. E., Bennett, W. M., Klahr, S., Meyers, C. M., Zhang, X., Thompson, P. A., Miller, J. P., and the Consortium for Radiologic Imaging Studies,
(2006). Magnetic Resonance Imaging Evaluation of Hepatic Cysts in Early Autosomal-Dominant Polycystic Kidney Disease: The Consortium for Radiologic Imaging Studies of Polycystic Kidney Disease Cohort. CJASN
1: 64-69
[Abstract][Full Text]
Jiang, S.-T., Chiou, Y.-Y., Wang, E., Lin, H.-K., Lin, Y.-T., Chi, Y.-C., Wang, C.-K. L., Tang, M.-J., Li, H.
(2006). Defining a Link with Autosomal-Dominant Polycystic Kidney Disease in Mice with Congenitally Low Expression of Pkd1. Am. J. Pathol.
168: 205-220
[Abstract][Full Text]
Vagin, O., Turdikulova, S., Sachs, G.
(2005). Recombinant Addition of N-Glycosylation Sites to the Basolateral Na,K-ATPase beta1 Subunit Results in Its Clustering in Caveolae and Apical Sorting in HGT-1 Cells. J. Biol. Chem.
280: 43159-43167
[Abstract][Full Text]
Winkelbauer, M. E., Schafer, J. C., Haycraft, C. J., Swoboda, P., Yoder, B. K.
(2005). The C. elegans homologs of nephrocystin-1 and nephrocystin-4 are cilia transition zone proteins involved in chemosensory perception. J. Cell Sci.
118: 5575-5587
[Abstract][Full Text]
Quarmby, L. M., Mahjoub, M. R.
(2005). Caught Nek-ing: cilia and centrioles. J. Cell Sci.
118: 5161-5169
[Abstract][Full Text]
Zatti, A., Chauvet, V., Rajendran, V., Kimura, T., Pagel, P., Caplan, M. J.
(2005). The C-Terminal Tail of the Polycystin-1 Protein Interacts with the Na,K-ATPase {alpha}-Subunit. Mol. Biol. Cell
16: 5087-5093
[Abstract][Full Text]
Ferreira, P. A.
(2005). Insights into X-linked retinitis pigmentosa type 3, allied diseases and underlying pathomechanisms. Hum Mol Genet
14: R259-R267
[Abstract][Full Text]
Ma, R., Li, W.-P., Rundle, D., Kong, J., Akbarali, H. I., Tsiokas, L.
(2005). PKD2 Functions as an Epidermal Growth Factor-Activated Plasma Membrane Channel. Mol. Cell. Biol.
25: 8285-8298
[Abstract][Full Text]
Anyatonwu, G. I., Ehrlich, B. E.
(2005). Organic Cation Permeation through the Channel Formed by Polycystin-2. J. Biol. Chem.
280: 29488-29493
[Abstract][Full Text]
Quarmby, L. M., Parker, J. D.K.
(2005). Cilia and the cell cycle?. JCB
169: 707-710
[Abstract][Full Text]
Silberberg, M., Charron, A. J., Bacallao, R., Wandinger-Ness, A.
(2005). Mispolarization of desmosomal proteins and altered intercellular adhesion in autosomal dominant polycystic kidney disease. Am. J. Physiol. Renal Physiol.
288: F1153-F1163
[Abstract][Full Text]
Tao, Y., Kim, J., Faubel, S., Wu, J. C., Falk, S. A., Schrier, R. W., Edelstein, C. L.
(2005). Caspase inhibition reduces tubular apoptosis and proliferation and slows disease progression in polycystic kidney disease. Proc. Natl. Acad. Sci. USA
102: 6954-6959
[Abstract][Full Text]
Moe, O. W., Bonny, O.
(2005). Genetic Hypercalciuria. J. Am. Soc. Nephrol.
16: 729-745
[Abstract][Full Text]
Qian, C.-N., Knol, J., Igarashi, P., Lin, F., Zylstra, U., Teh, B. T., Williams, B. O.
(2005). Cystic Renal Neoplasia Following Conditional Inactivation of Apc in Mouse Renal Tubular Epithelium. J. Biol. Chem.
280: 3938-3945
[Abstract][Full Text]
Tao, Y., Kim, J., Schrier, R. W., Edelstein, C. L.
(2005). Rapamycin Markedly Slows Disease Progression in a Rat Model of Polycystic Kidney Disease. J. Am. Soc. Nephrol.
16: 46-51
[Abstract][Full Text]
Leeuwen, I. S. L.-v., Dauwerse, J. G., Baelde, H. J., Leonhard, W. N., van de Wal, A., Ward, C. J., Verbeek, S., DeRuiter, M. C., Breuning, M. H., de Heer, E., Peters, D. J.M.
(2004). Lowering of Pkd1 expression is sufficient to cause polycystic kidney disease. Hum Mol Genet
13: 3069-3077
[Abstract][Full Text]
Masyuk, T. V., Huang, B. Q., Masyuk, A. I., Ritman, E. L., Torres, V. E., Wang, X., Harris, P. C., LaRusso, N. F.
(2004). Biliary Dysgenesis in the PCK Rat, an Orthologous Model of Autosomal Recessive Polycystic Kidney Disease. Am. J. Pathol.
165: 1719-1730
[Abstract][Full Text]
Mahjoub, M. R., Qasim Rasi, M., Quarmby, L. M.
(2004). A NIMA-related Kinase, Fa2p, Localizes to a Novel Site in the Proximal Cilia of Chlamydomonas and Mouse Kidney Cells. Mol. Biol. Cell
15: 5172-5186
[Abstract][Full Text]
Romio, L., Fry, A. M., Winyard, P. J.D., Malcolm, S., Woolf, A. S., Feather, S. A.
(2004). OFD1 Is a Centrosomal/Basal Body Protein Expressed during Mesenchymal-Epithelial Transition in Human Nephrogenesis. J. Am. Soc. Nephrol.
15: 2556-2568
[Abstract][Full Text]
Vagin, O., Turdikulova, S., Sachs, G.
(2004). The H,K-ATPase {beta} Subunit as a Model to Study the Role of N-Glycosylation in Membrane Trafficking and Apical Sorting. J. Biol. Chem.
279: 39026-39034
[Abstract][Full Text]
Markhardt, B. K., Winter, T. C., Lee, F. T. Jr
(2004). Autosomal Dominant Polycystic Kidney Disease Developing in Transplanted Pediatric Kidneys. J Ultrasound Med
23: 1235-1238
[Full Text]