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PKD Core Center
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Selected Publications by Baltimore PKD Center Investigators

Cebotaru V, Cebotaru L, Kim H, Chiaravalli M, Boletta A, Qian F, Guggino WB. Polycystin-1 Negatively Regulates Polycystin-2 Expression via the Aggresome/Autophagosome Pathway. J Biol Chem. 289: 6404-6414, 2014. PMID: 24459142, PMCID: PMC3945307.

Chatterjee S, Alsaeedi N, Hou J, Bandaru VV, Wu L, Halushka MK, Pili R, Ndikuyeze G, Haughey NJ. Use of a glycolipid inhibitor to ameliorate renal cancer in a mouse model. PLoS One.;8(5):e63726. doi: 10.1371/journal.pone.0063726, 2013. PMID: 23671696

Coxam B, Sabine A, Bower NI, Smith KA, Pichol-Thievend C, Skoczylas R, Astin JW, Frampton E, Jaquet M, Crosier PS, Parton RG, Harvey NL, Petrova TV, Schulte-Merker S, Francois M, Hogan BM. Pkd1 regulates lymphatic vascular morphogenesis during development. Cell Rep. May 8;7(3):623-33. doi: 10.1016/j.celrep.2014.03.063, 2014. PMID: 24767999.

Dalagiorgou G, Piperi C, Georgopoulou U, Adamopoulos C, Basdra EK, Papavassiliou AG. Mechanical stimulation of polycystin-1 induces human osteoblastic gene expression via potentiation of the calcineurin/NFAT signaling axis. Cell Mol Life Sci. 7:167-80, 2013. PMID: 23014991. DID NOT CITE CENTER USED OUR REAGENTS

DeRose R, Pohlmeyer C, Umeda N, Ueno T, Nagano T, Kuo S, Inoue T. Spatio-temporal manipulation of small GTPase activity at subcellular level and on timescale of seconds in living cells. J Vis Exp. 2012 Mar 9;(61). pii: 3794. doi: 10.3791/3794 PMID: 22433289, PMCID: PMC3460574

Ferreira FM, Oliveira LC, Germino GG, Onuchic JN and Onuchic LF. Macromolecular assembly of polycystin-2 intracytosolic C-terminal domain. Proc Natl Acad Sci U S A. 108: 9833-9838, 2011. PMID: 21622852 PMCID: PMC3116388 [PDF]

Fogelgren, B., Lin, S.Y., Zuo, X., Jaffe, K.M., Park, K.M., Reichert, R.J., Bell, P.D., Burdine, R.D., and Lipschutz, J.H. The Exocyst Protein Sec10 Interacts with Polycystin-2 and Knockdown Causes PKD-Phenotypes. PLoS Genet 7: e1001361. doi: 10.1371/journal.pgen.1001361. PMID: 21490950 PMCID: PMC3072367 [PDF]

Fonseca JM, Bastos AP, Am aral AG, Sousa MF, Souza LE, Malheiros DM, Piontek K, Irigoyen MC, Watnick TJ, Onuchic LF. Renal cyst growth is the main determinant for hypertension and concentrating deficit in Pkd1 - deficient mice. Kidney Int 85:1137 - 1150, 2014. PMID: 24429399.

Foy RL, Chitalia VC, Panchenko MV, Zeng L, Lopez D, Lee JW, Rana SV, Boletta A, Qian F, Tsiokas L, Piontek KB, Germino GG, Zhou MI, Cohen HT. Polycystin-1 regulates the stability and ubiquitination of transcription factor Jade-1. Hum Mol Genet 15:5456-71, 2012. PMID: 23001567 PMCID: PMC3516131.

Garcia-Gonzalez, M.A., Outeda, P., Zhou, Q., Zhou, F., Menezes, L.F., Qian, F., Huso, D.L., Germino, G.G., Piontek, K.B., and Watnick, T. Pkd1 and Pkd2 are required for normal placental development. PLoS One 5: e12821. doi:10.1371/journal.pone.0012821, 2010. PMID: 20862291 [PDF]

Hofherr A, Wagner C, Fedeles S, Somlo S, Köttgen M. N-glycosylation determines the abundance of the transient receptor potential channel TRPP2. J Biol Chem. 289:14854-67, 2014. PMID: 24719335 PMCID: PMC4031537.

Kim H, Xu H, Yao Q, Li W, Huang Q, Outeda P, Cebotaru V, Chiaravalli M, Boletta A, Weinman EJ, Piontek K, Germino GG, Watnick T and Qian F. Ciliary membrane proteins traffic through the Golgi via a Rabep1/GGA1/Arl3-dependent mechanism. Nat Commun 5: 5482, 2014. PMID: 25405894

Kleene, S.J. and Van Houten, J.L. (2014) Electrical signaling in motile and primary cilia. BioScience, In Press.

Kobayashi T, Kim S, Lin YC, Inoue T, Dynlacht BD. The CP110-interacting proteins Talpid3 and Cep290 play overlapping and distinct roles in cilia assembly. J Cell Biol. 204: 215-29, 2014. PMID: 24421332, PMCID:PMC3897186. (Cites P30 number in acknowledgments)

Köttgen M, Hofherr A, Li W, Chu K, Cook S, Montell C, Watnick T. Drosophila Sperm Swim Backwards in the Female Reproductive Tract and Are Activated via TRPP2 Ion Channels. PLoS One 6(5): e20031, 2011. PMID: 2162549 4 [PMCID: PMC3098850 [PDF]

Kurbegovic A, Kim H, Xu H, Yu S, Cruanes J, Maser RL, Boletta A, Trudel M, Qian F. Novel functional complexity of polycystin-1 by GPF cleavage in vivo: role in polycystic kidney disease. Moll Cell Biol 34:3341-53, 2014. PMID: 24958103 [PubMed - in process] PMCID: PMC4135549

Kwon M, Pavlov TS, Nozu K, Rasmussen S, Ilatovskaya DV, Lerch-Gaggl A, North LM, Kim H, Qian F, Sweeney, WE Jr. Avner ED, Blumer JB, Staruschenko A, Park F. G protein signaling modulator 1 deficiency accelerates cystic disease in an orthologous mouse model of ADPKD. Proc Natl Acad Sci USA 109: 21462-7, 2012. PMID: 23236168 PMCID: PMC3535663 [PDF]

Lan Z, Wang K, Garcia-Gonzalez M, He N, Dicks E, Parfrey P, Germino G, Watnick T. A missense mutation in PKD1 attenuates the severity of renal disease. Kidney Int. 81: 412-7, 2012. [PDF]

Lee, Y., Akitake, B., Woodward, O.M., Guggino, W.B., and Montell, C. Drosophila TRPA1 channel mediates chemical avoidance in gustatory receptor neurons. Proc Natl Acad Sci USA 107: 8440-8445, 2010. [PDF]

Li Y, Zhang Q, Wei Q, Zhang Y, Ling K, Hu J. SUMOylation of smal GTPase Arl-13 Promotes Ciliary Targeting of Sensory Receptors. Journal of Cell Biology 199: 589-98, 2012. PMCID: PMC3494855 ( Cited our Core in the Erratum ) [PDF]

Lin YC , Niewiadomski P, Lin B, Nakamura H, Phua SC, Jiao J, Levchenko A, Inoue T, Rohatgi R, Inoue T. Chemically inducible diffusion trap at cilia reveals molecular sieve - like barrier. Nat Chem Biol . 9 :437 - 43 , 2013 . PMID: 23666116 PMCID: PMC3870470

Liu D, Wang CJ, Judge DP, Halushka MK, Ni J, Habashi JP, Moslehi J, Bedja D, Gabrielson KL, Xu H, QianF, Huso D, Dietz HC, Germino GG, Watnick T. A Pkd1-Fbn1 genetic interaction implicates TGF-β signaling in the pathogenesis of vascular complications in autosomal dominant polycystic kidney disease. J Am Soc Nephrol. 25: 81-91, 2014. PMID: 24071006

McNeal CJ, Chatterjee S, Hou J, Worthy LS, Larner CD, Macfarlane RD, Alaupovic P, Brocia RW. Human HDL containing a novel apoC-I isoform induces smooth muscle cell apoptosis. Cardiovasc Res. 98:83-93, 2013. PMID: 23354389

Miyamoto T, DeRose R, Suarez A, Ueno T, Chen M, Sun TP, Wolfgang MJ, Mukherjee C, Meyers DJ, Inoue T. Rapid and orthogonal logic gating with a gibberellin-induced dimerization system. Nat Chem Biol : 8:465-70, 2012. PMID: 22446836, PMCID: PMC3368803.

Nadella, R., Blumer, J.B., Jia, G., Kwon, M., Akbulut, T., Qian, F., Sedlic, F., Wakatsuki, T., Sweeney, W.E., Jr., Wilson, P.D., et al. Activator of G protein signaling 3 promotes epithelial cell proliferation in PKD. J Am Soc Nephrol 21: 1275-1280. PMID: 20488951 PMCID: PMC2938587 [PDF]

Narayanan D, Bulley S, Leo MD, Burris SK, Gabrick KS, Boop FA, Jaggar JH.Smooth muscle cell transient receptor potential polycystin-2 (TRPP2) channels contribute to the myogenic response in cerebral arteries. J Physiol 591:5031-46, 2013. PMID: 23858011 PMCID: PMC3810807

Nie X, Arend LJ. Pkd1 is required for male reproductive tract development. Mech Dev. 130: 567-76, 2013. PMID: 23933588.

Nie X, Arend LJ. Novel roles of Pkd2 in male reproductive system development. Differentiation 87:161-71, 2014. PMID: 24951251

Outeda P, Huso DL, Fisher SA, Halushka MK, Kim H, Qian F, Germino GG, Watnick T. Polycystin Signaling Is Required for Directed Endothelial Cell Migration and Lymphatic Development. Cell Rep. May 8;7(3):634 - 44. doi: 10.1016/j.celrep.2014.03.064, 2014. PMID: 24767998 [PubMed - in process] PMCID: PMC4040350

Pei, Y., and Watnick, T. Diagnosis and screening of autosomal dominant polycystic kidney disease. Adv Chronic Kidney Dis 17: 140-152, 2010. PMID: 22031115 [PDF]

Rowe I, Chiaravalli M, Mannella V, Ulisse V, Quilici G, Pema M, Song XW, Xu H, Mari S, Qian F, Pei Y, Musco G, Boletta A. Defective glucose metabolism in polycystic kidney disease identifies a new therapeutic strategy. Nature Med 19: 488 - 93, 2013. PMID: 23524344

Santoso NG, Cebotaru L and GugginoWB. Polycystin-1, 2, and STIM1 interact with IP(3)R to modulate ER Ca release through the PI3K/Akt pathway. Cell Physiol Biochem 27: 715-26, 2011. PMID: 21691089 PMCID: PMC3221273 [PDF]

Santoso, N.G., Yu, S., Woodward, O.M., Qian, F., and Guggino, W.B. (2009). Polycystin-1 interacts with inositol 1,4,5-trisphosphate receptor to modulate intracellular Ca2+ signaling with implications for polycystic kidney disease. J Biol Chem 284: 36431-36441, 2009. [PDF]

Schroder, S., Fraternali, F., Quan, X., Scott, D., Qian, F., and Pfuhl, M. When a module is not a domain: the case of the REJ module and the redefinition of the architecture of polycystin-1. Biochem J 435: 651-660. PMID: 21314639. [PDF]

Segal, Y., Flinter, F., Makanjuola, D., Gan, J.S., and Watnick, T. Aortic abnormalities in males with Alport syndrome. Nephrol Dial Transplant 25: 3554-3560, 2010. [PDF]

Steigelman KA, Lelli A, Wu X, Gao J, Lin S, Piontek K, Wodarczyk C, Boletta A, Kim H, Qian F, Germino G, Géléoc GS, Holt JR, Zuo J. Polycystin-1 is required for stereocilia structure but not for mechanotransduction in inner ear hair cells J Neurosci. 31: 12241-50, 2011. PMID: 21865467 [PDF]

Su S, Phua SC, DeRose R, Chiba S, Narita K, Kalugin PN, Katada T, Kontani K, Takeda S,Inoue T. Genetically encoded calcium indicator illuminates calcium dynamics in cilia, Nat Methods 10: 1105-1107, 2013. PMID: 24056873, PMCID: PMC3860264

Tan M, Wettersten HI, Chu K, Huso DL, Watnick T, Friedlander S, Landesman Y, Weiss RH. Novel inhibitors of nuclear transport cause cell cycle arrest and decrease cyst growth in ADPKD associated with decreased cdk4 levels. Am J Physiol Renal Physiol. In Press 2014. PMID: 25234309

Talbot JJ, Shillingford JM, Vasanth S, Doerr N, Mukherjee S, Kinter MT, Watnick T, Weimbs T. Polycystin-1 regulates STAT activity by a dual mechanism. Proc Natl Acad Sci U S A 108: 7985-90, 2011. P MID: 21518865 PMCID: PMC309351 [PDF]

Ueno T, Falkenburger BH, Pohlmeyer C, Inoue T. Triggering actin comets versus membrane ruffles: distinctive effects of phosphoinositides on actin reorganization. Sci Signal 4:ra87. doi:10.1126/scisignal.2002033, 2011. PMID: 22169478, PMCID: PMC3372792

Umeda N1, Ueno T, Pohlmeyer C, Nagano T, Inoue T. A photocleavable rapamycin conjugate for spatiotemporal control of small GTPase activity. J Am Chem Soc 13:12-14, 2011. PMID: 21142151, PMCID:PMC3850177.

Watnick, T., and Atta, M.G. Not all renal cysts are created equal. Lancet 376: 1024, 2010. [PDF]

Watnick, T., and Germino, G.G. mTOR inhibitors in polycystic kidney disease. N Engl J Med 363: 879-881, 2010. [PDF]

Watnick, TJ and Germino GG. Polycystic kidney disease: Polycystin - 1 and polycystin - 2 — it's complicated. Nat Rev Nephrol 9 : 249 - 50, 2013. PMID: 23567275

Woodward OM1, Li Y, Yu S, Greenwell P, Wodarczyk C, Boletta A, Guggino WB, Qian F. Identification of a polycystin - 1 cleavage product, P100, that regulates store operated Ca entry through interactions with STIM1. PLoS One 5(8):e12305. doi: 10.1371/journal.pone.0012305, 2010. PMID: 20808796 [PubMed - indexed for MEDLINE] PMCID: PMC2925899

Woodward, O.M., Li, Y., Yu, S., Greenwell, P., Wodarczyk, C., Boletta, A., Guggino, W.B., and Qian, F. Identification of a polycystin-1 cleavage product, P100, that regulates store operated Ca entry through interactions with STIM1. PLoS One 5: e12305, 2010. [PDF]

Xu M, Ma L, Bujalowski PJ, Qian F, Sutton RB, Oberhauser AF. Analysis of the REJ module of Polycystin-1 using Molecular Modeling and Force-Spectroscopy Techniques. Journal of Biophysics, 2013. PMID:23762046, PMCID: PMC3677617.

Zuo X, Fogelgren B, Lipschutz JH. (2011) The small GTPase Cdc42 is necessary for primary ciliogenesis in renal tubular epithelial cells. J Biol Chem 286: 22469-77, 2011. PMID: 21543338 PMCID: PMC3121392 [PDF]

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The University of Maryland Medical Center and the Division of Nephrology provide multidisciplinary care for patients with PKD. For Appointments call 410-328-5720 and ask for Karleen Schuhart. Make an appointment.

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