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Items: 1 to 50 of 52

1.

Polycystin-1 Regulates Actomyosin Contraction and the Cellular Response to Extracellular Stiffness.

Nigro EA, Distefano G, Chiaravalli M, Matafora V, Castelli M, Pesenti Gritti A, Bachi A, Boletta A.

Sci Rep. 2019 Nov 12;9(1):16640. doi: 10.1038/s41598-019-53061-0.

PMID:
31719603
2.

The N-Terminal Domain of NPHP1 Folds into a Monomeric Left-Handed Antiparallel Three-Stranded Coiled Coil with Anti-apoptotic Function.

Mannella V, Quilici G, Nigro EA, Lampis M, Minici C, Degano M, Boletta A, Musco G.

ACS Chem Biol. 2019 Aug 16;14(8):1845-1854. doi: 10.1021/acschembio.9b00582. Epub 2019 Aug 5.

PMID:
31345020
3.

mTORC1-driven accumulation of the oncometabolite fumarate as a potential critical step in renal cancer progression.

Drusian L, Boletta A.

Mol Cell Oncol. 2018 Nov 20;6(1):1537709. doi: 10.1080/23723556.2018.1537709. eCollection 2019.

PMID:
30788416
4.

Dissection of metabolic reprogramming in polycystic kidney disease reveals coordinated rewiring of bioenergetic pathways.

Podrini C, Rowe I, Pagliarini R, Costa ASH, Chiaravalli M, Di Meo I, Kim H, Distefano G, Tiranti V, Qian F, di Bernardo D, Frezza C, Boletta A.

Commun Biol. 2018 Nov 16;1:194. doi: 10.1038/s42003-018-0200-x. eCollection 2018.

5.

Metabolism and mitochondria in polycystic kidney disease research and therapy.

Padovano V, Podrini C, Boletta A, Caplan MJ.

Nat Rev Nephrol. 2018 Nov;14(11):678-687. doi: 10.1038/s41581-018-0051-1. Review.

PMID:
30120380
6.

mTORC1 Upregulation Leads to Accumulation of the Oncometabolite Fumarate in a Mouse Model of Renal Cell Carcinoma.

Drusian L, Nigro EA, Mannella V, Pagliarini R, Pema M, Costa ASH, Benigni F, Larcher A, Chiaravalli M, Gaude E, Montorsi F, Capitanio U, Musco G, Frezza C, Boletta A.

Cell Rep. 2018 Jul 31;24(5):1093-1104.e6. doi: 10.1016/j.celrep.2018.06.106.

7.

Casein kinase 1ε and 1α as novel players in polycystic kidney disease and mechanistic targets for (R)-roscovitine and (S)-CR8.

Billot K, Coquil C, Villiers B, Josselin-Foll B, Desban N, Delehouzé C, Oumata N, Le Meur Y, Boletta A, Weimbs T, Grosch M, Witzgall R, Saunier S, Fischer E, Pontoglio M, Fautrel A, Mrug M, Wallace D, Tran PV, Trudel M, Bukanov N, Ibraghimov-Beskrovnaya O, Meijer L.

Am J Physiol Renal Physiol. 2018 Jul 1;315(1):F57-F73. doi: 10.1152/ajprenal.00489.2017. Epub 2018 Mar 14.

8.

The centrosomal OFD1 protein interacts with the translation machinery and regulates the synthesis of specific targets.

Iaconis D, Monti M, Renda M, van Koppen A, Tammaro R, Chiaravalli M, Cozzolino F, Pignata P, Crina C, Pucci P, Boletta A, Belcastro V, Giles RH, Surace EM, Gallo S, Pende M, Franco B.

Sci Rep. 2017 Apr 27;7(1):1224. doi: 10.1038/s41598-017-01156-x.

9.

Defective glycolysis and the use of 2-deoxy-D-glucose in polycystic kidney disease: from animal models to humans.

Magistroni R, Boletta A.

J Nephrol. 2017 Aug;30(4):511-519. doi: 10.1007/s40620-017-0395-9. Epub 2017 Apr 7. Review.

PMID:
28390001
10.

mTORC1 signaling and primary cilia are required for brain ventricle morphogenesis.

Foerster P, Daclin M, Asm S, Faucourt M, Boletta A, Genovesio A, Spassky N.

Development. 2017 Jan 15;144(2):201-210. doi: 10.1242/dev.138271. Epub 2016 Dec 19.

11.

The polycystins are modulated by cellular oxygen-sensing pathways and regulate mitochondrial function.

Padovano V, Kuo IY, Stavola LK, Aerni HR, Flaherty BJ, Chapin HC, Ma M, Somlo S, Boletta A, Ehrlich BE, Rinehart J, Caplan MJ.

Mol Biol Cell. 2017 Jan 15;28(2):261-269. doi: 10.1091/mbc.E16-08-0597. Epub 2016 Nov 23.

12.

Deciphering Variability of PKD1 and PKD2 in an Italian Cohort of 643 Patients with Autosomal Dominant Polycystic Kidney Disease (ADPKD).

Carrera P, Calzavara S, Magistroni R, den Dunnen JT, Rigo F, Stenirri S, Testa F, Messa P, Cerutti R, Scolari F, Izzi C, Edefonti A, Negrisolo S, Benetti E, Alibrandi MT, Manunta P, Boletta A, Ferrari M.

Sci Rep. 2016 Aug 8;6:30850. doi: 10.1038/srep30850.

13.

Double inhibition of cAMP and mTOR signalling may potentiate the reduction of cell growth in ADPKD cells.

de Stephanis L, Bonon A, Varani K, Lanza G, Gafà R, Pinton P, Pema M, Somlo S, Boletta A, Aguiari G.

Clin Exp Nephrol. 2017 Apr;21(2):203-211. doi: 10.1007/s10157-016-1289-1. Epub 2016 Jun 9.

14.

mTORC1-mediated inhibition of polycystin-1 expression drives renal cyst formation in tuberous sclerosis complex.

Pema M, Drusian L, Chiaravalli M, Castelli M, Yao Q, Ricciardi S, Somlo S, Qian F, Biffo S, Boletta A.

Nat Commun. 2016 Mar 2;7:10786. doi: 10.1038/ncomms10786.

15.

Slowing Polycystic Kidney Disease by Fasting.

Boletta A.

J Am Soc Nephrol. 2016 May;27(5):1268-70. doi: 10.1681/ASN.2015101113. Epub 2015 Nov 4. No abstract available.

16.

2-Deoxy-d-Glucose Ameliorates PKD Progression.

Chiaravalli M, Rowe I, Mannella V, Quilici G, Canu T, Bianchi V, Gurgone A, Antunes S, D'Adamo P, Esposito A, Musco G, Boletta A.

J Am Soc Nephrol. 2016 Jul;27(7):1958-69. doi: 10.1681/ASN.2015030231. Epub 2015 Nov 3.

17.

Role of the Polycystins in Cell Migration, Polarity, and Tissue Morphogenesis.

Nigro EA, Castelli M, Boletta A.

Cells. 2015 Oct 30;4(4):687-705. doi: 10.3390/cells4040687. Review.

18.

[The ciliopathies--Polycystic Kidney Disease].

Gritti AP, Boletta A.

G Ital Nefrol. 2015;32 Suppl 64. pii: gin/32.S64.15. Italian.

PMID:
26479061
19.

Phosphoinositide 3-Kinase-C2α Regulates Polycystin-2 Ciliary Entry and Protects against Kidney Cyst Formation.

Franco I, Margaria JP, De Santis MC, Ranghino A, Monteyne D, Chiaravalli M, Pema M, Campa CC, Ratto E, Gulluni F, Perez-Morga D, Somlo S, Merlo GR, Boletta A, Hirsch E.

J Am Soc Nephrol. 2016 Apr;27(4):1135-44. doi: 10.1681/ASN.2014100967. Epub 2015 Aug 13.

20.

Mechanosensory Genes Pkd1 and Pkd2 Contribute to the Planar Polarization of Brain Ventricular Epithelium.

Ohata S, Herranz-Pérez V, Nakatani J, Boletta A, García-Verdugo JM, Álvarez-Buylla A.

J Neurosci. 2015 Aug 5;35(31):11153-68. doi: 10.1523/JNEUROSCI.0686-15.2015.

21.

Regulation of the microtubular cytoskeleton by Polycystin-1 favors focal adhesions turnover to modulate cell adhesion and migration.

Castelli M, De Pascalis C, Distefano G, Ducano N, Oldani A, Lanzetti L, Boletta A.

BMC Cell Biol. 2015 May 7;16:15. doi: 10.1186/s12860-015-0059-3.

22.

Ciliary membrane proteins traffic through the Golgi via a Rabep1/GGA1/Arl3-dependent mechanism.

Kim H, Xu H, Yao Q, Li W, Huang Q, Outeda P, Cebotaru V, Chiaravalli M, Boletta A, Piontek K, Germino GG, Weinman EJ, Watnick T, Qian F.

Nat Commun. 2014 Nov 18;5:5482. doi: 10.1038/ncomms6482.

23.

Novel functional complexity of polycystin-1 by GPS cleavage in vivo: role in polycystic kidney disease.

Kurbegovic A, Kim H, Xu H, Yu S, Cruanès J, Maser RL, Boletta A, Trudel M, Qian F.

Mol Cell Biol. 2014 Sep;34(17):3341-53. doi: 10.1128/MCB.00687-14. Epub 2014 Jun 23.

24.

Renal-retinal ciliopathy gene Sdccag8 regulates DNA damage response signaling.

Airik R, Slaats GG, Guo Z, Weiss AC, Khan N, Ghosh A, Hurd TW, Bekker-Jensen S, Schrøder JM, Elledge SJ, Andersen JS, Kispert A, Castelli M, Boletta A, Giles RH, Hildebrandt F.

J Am Soc Nephrol. 2014 Nov;25(11):2573-83. doi: 10.1681/ASN.2013050565. Epub 2014 Apr 10.

25.

PI3K class II α controls spatially restricted endosomal PtdIns3P and Rab11 activation to promote primary cilium function.

Franco I, Gulluni F, Campa CC, Costa C, Margaria JP, Ciraolo E, Martini M, Monteyne D, De Luca E, Germena G, Posor Y, Maffucci T, Marengo S, Haucke V, Falasca M, Perez-Morga D, Boletta A, Merlo GR, Hirsch E.

Dev Cell. 2014 Mar 31;28(6):647-58. doi: 10.1016/j.devcel.2014.01.022.

26.

Impaired glomerulogenesis and endothelial cell migration in Pkd1-deficient renal organ cultures.

Rowe I, Chiaravalli M, Piontek KB, Germino GG, Boletta A.

Biochem Biophys Res Commun. 2014 Feb 21;444(4):473-9. doi: 10.1016/j.bbrc.2014.01.068. Epub 2014 Jan 25.

27.

Polycystin-1 negatively regulates Polycystin-2 expression via the aggresome/autophagosome pathway.

Cebotaru V, Cebotaru L, Kim H, Chiaravalli M, Boletta A, Qian F, Guggino WB.

J Biol Chem. 2014 Mar 7;289(10):6404-14. doi: 10.1074/jbc.M113.501205. Epub 2014 Jan 23.

28.

Defective metabolism in polycystic kidney disease: potential for therapy and open questions.

Rowe I, Boletta A.

Nephrol Dial Transplant. 2014 Aug;29(8):1480-6. doi: 10.1093/ndt/gft521. Epub 2014 Jan 23. Review.

PMID:
24459136
29.

Polycystin-1 binds Par3/aPKC and controls convergent extension during renal tubular morphogenesis.

Castelli M, Boca M, Chiaravalli M, Ramalingam H, Rowe I, Distefano G, Carroll T, Boletta A.

Nat Commun. 2013;4:2658. doi: 10.1038/ncomms3658.

30.

Defective glucose metabolism in polycystic kidney disease identifies a new therapeutic strategy.

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.

Nat Med. 2013 Apr;19(4):488-93. doi: 10.1038/nm.3092. Epub 2013 Mar 24.

31.

Polycystin-1 regulates the stability and ubiquitination of transcription factor Jade-1.

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.

Hum Mol Genet. 2012 Dec 15;21(26):5456-71. doi: 10.1093/hmg/dds391. Epub 2012 Sep 21.

32.

Polycystin-1 is required for stereocilia structure but not for mechanotransduction in inner ear hair cells.

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.

J Neurosci. 2011 Aug 24;31(34):12241-50. doi: 10.1523/JNEUROSCI.6531-10.2011.

33.

Nephrocystin-1 forms a complex with polycystin-1 via a polyproline motif/SH3 domain interaction and regulates the apoptotic response in mammals.

Wodarczyk C, Distefano G, Rowe I, Gaetani M, Bricoli B, Muorah M, Spitaleri A, Mannella V, Ricchiuto P, Pema M, Castelli M, Casanova AE, Mollica L, Banzi M, Boca M, Antignac C, Saunier S, Musco G, Boletta A.

PLoS One. 2010 Sep 14;5(9):e12719. doi: 10.1371/journal.pone.0012719.

34.

Identification of a polycystin-1 cleavage product, P100, that regulates store operated Ca entry through interactions with STIM1.

Woodward OM, Li Y, Yu S, Greenwell P, Wodarczyk C, Boletta A, Guggino WB, Qian F.

PLoS One. 2010 Aug 23;5(8):e12305. doi: 10.1371/journal.pone.0012305.

35.

Prospects for mTOR inhibitor use in patients with polycystic kidney disease and hamartomatous diseases.

Torres VE, Boletta A, Chapman A, Gattone V, Pei Y, Qian Q, Wallace DP, Weimbs T, Wüthrich RP.

Clin J Am Soc Nephrol. 2010 Jul;5(7):1312-29. doi: 10.2215/CJN.01360210. Epub 2010 May 24. Review.

36.

Emerging evidence of a link between the polycystins and the mTOR pathways.

Boletta A.

Pathogenetics. 2009 Oct 28;2(1):6. doi: 10.1186/1755-8417-2-6.

37.

A novel mouse model reveals that polycystin-1 deficiency in ependyma and choroid plexus results in dysfunctional cilia and hydrocephalus.

Wodarczyk C, Rowe I, Chiaravalli M, Pema M, Qian F, Boletta A.

PLoS One. 2009 Sep 23;4(9):e7137. doi: 10.1371/journal.pone.0007137.

38.

Polycystin-1 regulates extracellular signal-regulated kinase-dependent phosphorylation of tuberin to control cell size through mTOR and its downstream effectors S6K and 4EBP1.

Distefano G, Boca M, Rowe I, Wodarczyk C, Ma L, Piontek KB, Germino GG, Pandolfi PP, Boletta A.

Mol Cell Biol. 2009 May;29(9):2359-71. doi: 10.1128/MCB.01259-08. Epub 2009 Mar 2.

39.

Polycystin-1 induces cell migration by regulating phosphatidylinositol 3-kinase-dependent cytoskeletal rearrangements and GSK3beta-dependent cell cell mechanical adhesion.

Boca M, D'Amato L, Distefano G, Polishchuk RS, Germino GG, Boletta A.

Mol Biol Cell. 2007 Oct;18(10):4050-61. Epub 2007 Aug 1.

40.

A regulatory role of polycystin-1 on cystic fibrosis transmembrane conductance regulator plasma membrane expression.

Ikeda M, Fong P, Cheng J, Boletta A, Qian F, Zhang XM, Cai H, Germino GG, Guggino WB.

Cell Physiol Biochem. 2006;18(1-3):9-20. Epub 2006 Aug 14.

41.

Polycystin-1 induces resistance to apoptosis through the phosphatidylinositol 3-kinase/Akt signaling pathway.

Boca M, Distefano G, Qian F, Bhunia AK, Germino GG, Boletta A.

J Am Soc Nephrol. 2006 Mar;17(3):637-47. Epub 2006 Feb 1.

42.

Expression of polycystin-1 enhances endoplasmic reticulum calcium uptake and decreases capacitative calcium entry in ATP-stimulated MDCK cells.

Hooper KM, Boletta A, Germino GG, Hu Q, Ziegelstein RC, Sutters M.

Am J Physiol Renal Physiol. 2005 Sep;289(3):F521-30. Epub 2005 May 3.

43.

Role of polycystins in renal tubulogenesis.

Boletta A, Germino GG.

Trends Cell Biol. 2003 Sep;13(9):484-92. Review.

PMID:
12946628
44.

Cleavage of polycystin-1 requires the receptor for egg jelly domain and is disrupted by human autosomal-dominant polycystic kidney disease 1-associated mutations.

Qian F, Boletta A, Bhunia AK, Xu H, Liu L, Ahrabi AK, Watnick TJ, Zhou F, Germino GG.

Proc Natl Acad Sci U S A. 2002 Dec 24;99(26):16981-6. Epub 2002 Dec 13.

45.

PKD1 induces p21(waf1) and regulation of the cell cycle via direct activation of the JAK-STAT signaling pathway in a process requiring PKD2.

Bhunia AK, Piontek K, Boletta A, Liu L, Qian F, Xu PN, Germino FJ, Germino GG.

Cell. 2002 Apr 19;109(2):157-68.

46.

Biochemical characterization of bona fide polycystin-1 in vitro and in vivo.

Boletta A, Qian F, Onuchic LF, Bragonzi A, Cortese M, Deen PM, Courtoy PJ, Soria MR, Devuyst O, Monaco L, Germino GG.

Am J Kidney Dis. 2001 Dec;38(6):1421-9.

PMID:
11728985
47.

Co-assembly of polycystin-1 and -2 produces unique cation-permeable currents.

Hanaoka K, Qian F, Boletta A, Bhunia AK, Piontek K, Tsiokas L, Sukhatme VP, Guggino WB, Germino GG.

Nature. 2000 Dec 21-28;408(6815):990-4.

PMID:
11140688
48.

Polycystin-1, the gene product of PKD1, induces resistance to apoptosis and spontaneous tubulogenesis in MDCK cells.

Boletta A, Qian F, Onuchic LF, Bhunia AK, Phakdeekitcharoen B, Hanaoka K, Guggino W, Monaco L, Germino GG.

Mol Cell. 2000 Nov;6(5):1267-73.

49.

Glomerular filtration is required for transfection of proximal tubular cells in the rat kidney following injection of DNA complexes into the renal artery.

Foglieni C, Bragonzi A, Cortese M, Cantù L, Boletta A, Chiossone I, Soria MR, Monaco L.

Gene Ther. 2000 Feb;7(4):279-85.

50.

Comparison between cationic polymers and lipids in mediating systemic gene delivery to the lungs.

Bragonzi A, Boletta A, Biffi A, Muggia A, Sersale G, Cheng SH, Bordignon C, Assael BM, Conese M.

Gene Ther. 1999 Dec;6(12):1995-2004.

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