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Science 31 May 1996:
Vol. 272. no. 5266, pp. 1339 - 1342
DOI: 10.1126/science.272.5266.1339
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Reports

PKD2, a Gene for Polycystic Kidney Disease That Encodes an Integral Membrane Protein

Toshio Mochizuki, Guanqing Wu, * Tomohito Hayashi, * Stavroulla L. Xenophontos, Barbera Veldhuisen, Jasper J. Saris, David M. Reynolds, Yiqiang Cai, Patricia A. Gabow, Alkis Pierides, William J. Kimberling, Martijn H. Breuning, C. Constantinou Deltas, Dorien J. M. Peters, Stefan Somlo dagger

A second gene for autosomal dominant polycystic kidney disease was identified by positional cloning. Nonsense mutations in this gene (PKD2) segregated with the disease in three PKD2 families. The predicted 968-amino acid sequence of the PKD2 gene product has six transmembrane spans with intracellular amino- and carboxyl-termini. The PKD2 protein has amino acid similarity with PKD1, the Caenorhabditis elegans homolog of PKD1, and the family of voltage-activated calcium (and sodium) channels, and it contains a potential calcium-binding domain.

T. Mochizuki, G. Wu, T. Hayashi, D. M. Reynolds, Y. Cai, S. Somlo, Renal Division, Department of Medicine and Molecular Genetics, Albert Einstein College of Medicine, 1300 Morris Park Avenue, Bronx, NY 10461, USA.
S. L. Xenophontos and C. C. Deltas, Cyprus Institute of Neurology and Genetics, 6 International Airport Avenue, 2012 Agios Dhomentios, Nicosia, Cyprus.
B. Veldhuisen, J. J. Saris, M. H. Breuning, D. J. M. Peters, Department of Human Genetics, Sylvius Laboratories, Leiden University, Wassenaarseweg 72, 2333 AL, Leiden, Netherlands.
P. A. Gabow, Department of Internal Medicine, Colorado University Health Sciences Center, Denver, CO 80220, USA.
A. Pierides, Department of Nephrology, Nicosia General Hospital, 1311 Nicosia, Cyprus.
W. J. Kimberling, Departments of Pathology and Otolaryngology, Boys Town National Research Hospital, Omaha, NE 68131, USA.
* These authors contributed equally to this work.
dagger To whom correspondence should be addressed.


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TRP channels and lipids: from Drosophila to mammalian physiology.
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L. A. Smith, N. O. Bukanov, H. Husson, R. J. Russo, T. C. Barry, A. L. Taylor, D. R. Beier, and O. Ibraghimov-Beskrovnaya (2006)
J. Am. Soc. Nephrol. 17, 2821-2831
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A Truncated Polycystin-2 Protein Causes Polycystic Kidney Disease and Retinal Degeneration in Transgenic Rats.
A. R. Gallagher, S. Hoffmann, N. Brown, A. Cedzich, S. Meruvu, D. Podlich, Y. Feng, V. Konecke, U. de Vries, H.-P. Hammes, et al. (2006)
J. Am. Soc. Nephrol. 17, 2719-2730
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Development 133, 3859-3870
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Laminin 5 Regulates Polycystic Kidney Cell Proliferation and Cyst Formation.
D. Joly, S. Berissi, A. Bertrand, L. Strehl, N. Patey, and B. Knebelmann (2006)
J. Biol. Chem. 281, 29181-29189
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The Polycystin 1-C-terminal Fragment Stimulates ERK-dependent Spreading of Renal Epithelial Cells.
D. Joly, S. Ishibe, C. Nickel, Z. Yu, S. Somlo, and L. G. Cantley (2006)
J. Biol. Chem. 281, 26329-26339
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Diagnostic Approach in Autosomal Dominant Polycystic Kidney Disease.
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Clin. J. Am. Soc. Nephrol. 1, 1108-1114
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The Intraflagellar Transport Protein IFT20 Is Associated with the Golgi Complex and Is Required for Cilia Assembly.
J. A. Follit, R. A. Tuft, K. E. Fogarty, and G. J. Pazour (2006)
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Increased Water Intake Decreases Progression of Polycystic Kidney Disease in the PCK Rat.
S. Nagao, K. Nishii, M. Katsuyama, H. Kurahashi, T. Marunouchi, H. Takahashi, and D. P. Wallace (2006)
J. Am. Soc. Nephrol. 17, 2220-2227
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E.-F. Bui-Xuan, Q. Li, X.-Z. Chen, C. A. Boucher, R. Sandford, J. Zhou, and N. Basora (2006)
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Identification of an N-terminal glycogen synthase kinase 3 phosphorylation site which regulates the functional localization of polycystin-2 in vivo and in vitro.
A. J. Streets, D. J. Moon, M. E. Kane, T. Obara, and A. C.M. Ong (2006)
Hum. Mol. Genet. 15, 1465-1473
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Casein Kinase II and Calcineurin Modulate TRPP Function and Ciliary Localization.
J. Hu, Y.-K. Bae, K. M. Knobel, and M. M. Barr (2006)
Mol. Biol. Cell 17, 2200-2211
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Polycystin-2 traffics to cilia independently of polycystin-1 by using an N-terminal RVxP motif.
L. Geng, D. Okuhara, Z. Yu, X. Tian, Y. Cai, S. Shibazaki, and S. Somlo (2006)
J. Cell Sci. 119, 1383-1395
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Inhibition of mTOR with sirolimus slows disease progression in Han:SPRD rats with autosomal dominant polycystic kidney disease (ADPKD).
P. R. Wahl, A. L. Serra, M. Le Hir, K. D. Molle, M. N. Hall, and R. P. Wuthrich (2006)
Nephrol. Dial. Transplant. 21, 598-604
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Nonspecific Cation Current Associated with Native Polycystin-2 in HEK-293 Cells.
B. Pelucchi, G. Aguiari, A. Pignatelli, E. Manzati, R. Witzgall, L. del Senno, and O. Belluzzi (2006)
J. Am. Soc. Nephrol. 17, 388-397
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Loss of polycystin-1 or polycystin-2 results in dysregulated apolipoprotein expression in murine tissues via alterations in nuclear hormone receptors.
E. Allen, K. B. Piontek, E. Garrett-Mayer, M. Garcia-Gonzalez, K. L. Gorelick, and G. G. Germino (2006)
Hum. Mol. Genet. 15, 11-21
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Defining a Link with Autosomal-Dominant Polycystic Kidney Disease in Mice with Congenitally Low Expression of Pkd1.
S.-T. Jiang, Y.-Y. Chiou, E. Wang, H.-K. Lin, Y.-T. Lin, Y.-C. Chi, C.-K. L. Wang, M.-J. Tang, and H. Li (2006)
Am. J. Pathol. 168, 205-220
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Calcium Restores a Normal Proliferation Phenotype in Human Polycystic Kidney Disease Epithelial Cells.
T. Yamaguchi, S. J. Hempson, G. A. Reif, A.-M. Hedge, and D. P. Wallace (2006)
J. Am. Soc. Nephrol. 17, 178-187
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Polycystin 2 Interacts with Type I Inositol 1,4,5-Trisphosphate Receptor to Modulate Intracellular Ca2+ Signaling.
Y. Li, J. M. Wright, F. Qian, G. G. Germino, and W. B. Guggino (2005)
J. Biol. Chem. 280, 41298-41306
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Overview of Molecular Relationships in the Voltage-Gated Ion Channel Superfamily.
F. H. Yu, V. Yarov-Yarovoy, G. A. Gutman, and W. A. Catterall (2005)
Pharmacol. Rev. 57, 387-395
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NIMA-Related Kinases Defective in Murine Models of Polycystic Kidney Diseases Localize to Primary Cilia and Centrosomes.
M. R. Mahjoub, M. L. Trapp, and L. M. Quarmby (2005)
J. Am. Soc. Nephrol. 16, 3485-3489
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A splice form of polycystin-2, lacking exon 7, does not interact with polycystin-1.
K. Hackmann, A. Markoff, F. Qian, N. Bogdanova, G. G. Germino, P. Pennekamp, B. Dworniczak, J. Horst, and V. Gerke (2005)
Hum. Mol. Genet. 14, 3249-3262
   Abstract »    Full Text »    PDF »
Increased Activity of Activator Protein-1 Transcription Factor Components ATF2, c-Jun, and c-Fos in Human and Mouse Autosomal Dominant Polycystic Kidney Disease.
N. Hang Le, A. van der Wal, P. van der Bent, I. S. Lantinga-van Leeuwen, M. H. Breuning, H. van Dam, E. de Heer, and D. J.M. Peters (2005)
J. Am. Soc. Nephrol. 16, 2724-2731
   Abstract »    Full Text »    PDF »
Expression of polycystin-1 enhances endoplasmic reticulum calcium uptake and decreases capacitative calcium entry in ATP-stimulated MDCK cells.
K. M. Hooper, A. Boletta, G. G. Germino, Q. Hu, R. C. Ziegelstein, and M. Sutters (2005)
Am J Physiol Renal Physiol 289, F521-F530
   Abstract »    Full Text »    PDF »
Organic Cation Permeation through the Channel Formed by Polycystin-2.
G. I. Anyatonwu and B. E. Ehrlich (2005)
J. Biol. Chem. 280, 29488-29493
   Abstract »    Full Text »    PDF »
Androgen Receptor Pathway in Rats with Autosomal Dominant Polycystic Kidney Disease.
S. Nagao, M. Kusaka, K. Nishii, T. Marunouchi, H. Kurahashi, H. Takahashi, and J. Grantham (2005)
J. Am. Soc. Nephrol. 16, 2052-2062
   Abstract »    Full Text »    PDF »
Alpha-actinin associates with polycystin-2 and regulates its channel activity.
Q. Li, N. Montalbetti, P. Y. Shen, X.-Q. Dai, C. I. Cheeseman, E. Karpinski, G. Wu, H. F. Cantiello, and X.-Z. Chen (2005)
Hum. Mol. Genet. 14, 1587-1603
   Abstract »    Full Text »    PDF »
Kinesin Family Member 12 Is a Candidate Polycystic Kidney Disease Modifier in the cpk Mouse.
M. Mrug, R. Li, X. Cui, T. R. Schoeb, G. A. Churchill, and L. M. Guay-Woodford (2005)
J. Am. Soc. Nephrol. 16, 905-916
   Abstract »    Full Text »    PDF »
Molecular physiology and pathology of Ca2+-conducting channels in the plasma membrane of mammalian sperm.
R. Felix (2005)
Reproduction 129, 251-262
   Abstract »    Full Text »    PDF »
The TRP Superfamily of Cation Channels.
C. Montell (2005)
Sci. STKE 2005, re3
   Abstract »    Full Text »    PDF »
Genomic Organization and Functional Analysis of Murine PKD2L1.
M. Murakami, T. Ohba, F. Xu, S. Shida, E. Satoh, K. Ono, I. Miyoshi, H. Watanabe, H. Ito, and T. Iijima (2005)
J. Biol. Chem. 280, 5626-5635
   Abstract »    Full Text »    PDF »
PGD for autosomal dominant polycystic kidney disease type 1.
M. De Rycke, I. Georgiou, K. Sermon, W. Lissens, P. Henderix, H. Joris, P. Platteau, A. Van Steirteghem, and I. Liebaers (2005)
Mol. Hum. Reprod. 11, 65-71
   Abstract »    Full Text »    PDF »
TRP channels at a glance.
R. Padinjat and S. Andrews (2004)
J. Cell Sci. 117, 5707-5709
   Full Text »    PDF »
The VGL-Chanome: A Protein Superfamily Specialized for Electrical Signaling and Ionic Homeostasis.
F. H. Yu and W. A. Catterall (2004)
Sci. STKE 2004, re15
   Abstract »    Full Text »    PDF »
Intraflagellar Transport and Cilia-Dependent Renal Disease: The Ciliary Hypothesis of Polycystic Kidney Disease.
G. J. Pazour (2004)
J. Am. Soc. Nephrol. 15, 2528-2536
   Abstract »    Full Text »    PDF »
The intrarenal renin-angiotensin system in autosomal dominant polycystic kidney disease.
M. Loghman-Adham, C. E. Soto, T. Inagami, and L. Cassis (2004)
Am J Physiol Renal Physiol 287, F775-F788
   Abstract »    Full Text »    PDF »
Inaugural Article: Identification of 315 genes essential for early zebrafish development.
A. Amsterdam, R. M. Nissen, Z. Sun, E. C. Swindell, S. Farrington, and N. Hopkins (2004)
PNAS 101, 12792-12797
   Abstract »    Full Text »    PDF »
A genetic screen in zebrafish identifies cilia genes as a principal cause of cystic kidney.
Z. Sun, A. Amsterdam, G. J. Pazour, D. G. Cole, M. S. Miller, and N. Hopkins (2004)
Development 131, 4085-4093
   Abstract »    Full Text »    PDF »
PIGEA-14, a Novel Coiled-coil Protein Affecting the Intracellular Distribution of Polycystin-2.
S. Hidaka, V. Konecke, L. Osten, and R. Witzgall (2004)
J. Biol. Chem. 279, 35009-35016
   Abstract »    Full Text »    PDF »
Cystic Kidney Diseases: All Roads Lead to the Cilium.
Q. Zhang, P. D. Taulman, and B. K. Yoder (2004)
Physiology 19, 225-230
   Abstract »    Full Text »    PDF »
PKD2 Interacts and Co-localizes with mDia1 to Mitotic Spindles of Dividing Cells: ROLE OF mDia1 IN PKD2 LOCALIZATION TO MITOTIC SPINDLES.
D. R. Rundle, G. Gorbsky, and L. Tsiokas (2004)
J. Biol. Chem. 279, 29728-29739
   Abstract »    Full Text »    PDF »
The Transient Receptor Potential Superfamily of Ion Channels.
C.-L. Huang (2004)
J. Am. Soc. Nephrol. 15, 1690-1699
   Abstract »    Full Text »    PDF »
The N-terminal Extracellular Domain Is Required for Polycystin-1-dependent Channel Activity.
V. Babich, W.-Z. Zeng, B.-I. Yeh, O. Ibraghimov-Beskrovnaya, Y. Cai, S. Somlo, and C.-L. Huang (2004)
J. Biol. Chem. 279, 25582-25589
   Abstract »    Full Text »    PDF »
Regulation of calcium signaling by polycystin-2.
H. F. Cantiello (2004)
Am J Physiol Renal Physiol 286, F1012-F1029
   Abstract »    Full Text »    PDF »


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