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Items: 1 to 50 of 158

1.

Spliced XBP1 Rescues Renal Interstitial Inflammation Due to Loss of Sec63 in Collecting Ducts.

Ishikawa Y, Fedeles S, Marlier A, Zhang C, Gallagher AR, Lee AH, Somlo S.

J Am Soc Nephrol. 2019 Feb 11. pii: ASN.2018060614. doi: 10.1681/ASN.2018060614. [Epub ahead of print]

PMID:
30745418
2.

Mcp1 Promotes Macrophage-Dependent Cyst Expansion in Autosomal Dominant Polycystic Kidney Disease.

Cassini MF, Kakade VR, Kurtz E, Sulkowski P, Glazer P, Torres R, Somlo S, Cantley LG.

J Am Soc Nephrol. 2018 Oct;29(10):2471-2481. doi: 10.1681/ASN.2018050518. Epub 2018 Sep 12.

PMID:
30209078
3.

TREX1 Mutation Causing Autosomal Dominant Thrombotic Microangiopathy and CKD-A Novel Presentation.

Gulati A, Bale AE, Dykas DJ, Bia MJ, Danovitch GM, Moeckel GW, Somlo S, Dahl NK.

Am J Kidney Dis. 2018 Dec;72(6):895-899. doi: 10.1053/j.ajkd.2018.05.006. Epub 2018 Jun 22.

PMID:
29941221
4.

Monoallelic Mutations to DNAJB11 Cause Atypical Autosomal-Dominant Polycystic Kidney Disease.

Cornec-Le Gall E, Olson RJ, Besse W, Heyer CM, Gainullin VG, Smith JM, Audrézet MP, Hopp K, Porath B, Shi B, Baheti S, Senum SR, Arroyo J, Madsen CD, Férec C, Joly D, Jouret F, Fikri-Benbrahim O, Charasse C, Coulibaly JM, Yu AS, Khalili K, Pei Y, Somlo S, Le Meur Y, Torres VE; Genkyst Study Group; HALT Progression of Polycystic Kidney Disease Group; Consortium for Radiologic Imaging Studies of Polycystic Kidney Disease, Harris PC.

Am J Hum Genet. 2018 May 3;102(5):832-844. doi: 10.1016/j.ajhg.2018.03.013. Epub 2018 Apr 26.

5.

Genomic Analysis to Avoid Misdiagnosis of Adults With Bilateral Renal Cysts.

Gulati A, Bae KT, Somlo S, Watnick T.

Ann Intern Med. 2018 Jul 17;169(2):130-131. doi: 10.7326/L17-0644. Epub 2018 Mar 27. No abstract available.

PMID:
29582070
6.

Polycystin-2-dependent control of cardiomyocyte autophagy.

Criollo A, Altamirano F, Pedrozo Z, Schiattarella GG, Li DL, Rivera-Mejías P, Sotomayor-Flores C, Parra V, Villalobos E, Battiprolu PK, Jiang N, May HI, Morselli E, Somlo S, de Smedt H, Gillette TG, Lavandero S, Hill JA.

J Mol Cell Cardiol. 2018 May;118:110-121. doi: 10.1016/j.yjmcc.2018.03.002. Epub 2018 Mar 6.

PMID:
29518398
7.

Glutamine metabolism via glutaminase 1 in autosomal-dominant polycystic kidney disease.

Soomro I, Sun Y, Li Z, Diggs L, Hatzivassiliou G, Thomas AG, Rais R, Slusher BS, Somlo S, Skolnik EY.

Nephrol Dial Transplant. 2018 Aug 1;33(8):1343-1353. doi: 10.1093/ndt/gfx349.

PMID:
29420817
8.

Ganetespib limits ciliation and cystogenesis in autosomal-dominant polycystic kidney disease (ADPKD).

Nikonova AS, Deneka AY, Kiseleva AA, Korobeynikov V, Gaponova A, Serebriiskii IG, Kopp MC, Hensley HH, Seeger-Nukpezah TN, Somlo S, Proia DA, Golemis EA.

FASEB J. 2018 May;32(5):2735-2746. doi: 10.1096/fj.201700909R. Epub 2018 Jan 10.

9.

A noncoding variant in GANAB explains isolated polycystic liver disease (PCLD) in a large family.

Besse W, Choi J, Ahram D, Mane S, Sanna-Cherchi S, Torres V, Somlo S.

Hum Mutat. 2018 Mar;39(3):378-382. doi: 10.1002/humu.23383. Epub 2018 Jan 24.

10.

Adenylyl cyclase 5 deficiency reduces renal cyclic AMP and cyst growth in an orthologous mouse model of polycystic kidney disease.

Wang Q, Cobo-Stark P, Patel V, Somlo S, Han PL, Igarashi P.

Kidney Int. 2018 Feb;93(2):403-415. doi: 10.1016/j.kint.2017.08.005. Epub 2017 Oct 14.

11.

Isolated polycystic liver disease genes define effectors of polycystin-1 function.

Besse W, Dong K, Choi J, Punia S, Fedeles SV, Choi M, Gallagher AR, Huang EB, Gulati A, Knight J, Mane S, Tahvanainen E, Tahvanainen P, Sanna-Cherchi S, Lifton RP, Watnick T, Pei YP, Torres VE, Somlo S.

J Clin Invest. 2017 Sep 1;127(9):3558. doi: 10.1172/JCI96729. Epub 2017 Sep 1.

12.

Whole exome sequencing: a state-of-the-art approach for defining (and exploring!) genetic landscapes in pediatric nephrology.

Gulati A, Somlo S.

Pediatr Nephrol. 2018 May;33(5):745-761. doi: 10.1007/s00467-017-3698-0. Epub 2017 Jun 29. Review.

PMID:
28660367
13.

Isolated polycystic liver disease genes define effectors of polycystin-1 function.

Besse W, Dong K, Choi J, Punia S, Fedeles SV, Choi M, Gallagher AR, Huang EB, Gulati A, Knight J, Mane S, Tahvanainen E, Tahvanainen P, Sanna-Cherchi S, Lifton RP, Watnick T, Pei YP, Torres VE, Somlo S.

J Clin Invest. 2017 May 1;127(5):1772-1785. doi: 10.1172/JCI90129. Epub 2017 Apr 4. Erratum in: J Clin Invest. 2017 Sep 1;127(9):3558.

14.

Ciliary Mechanisms of Cyst Formation in Polycystic Kidney Disease.

Ma M, Gallagher AR, Somlo S.

Cold Spring Harb Perspect Biol. 2017 Nov 1;9(11). pii: a028209. doi: 10.1101/cshperspect.a028209. Review.

PMID:
28320755
15.

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.

16.

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.

17.

Deletion of ADP Ribosylation Factor-Like GTPase 13B Leads to Kidney Cysts.

Li Y, Tian X, Ma M, Jerman S, Kong S, Somlo S, Sun Z.

J Am Soc Nephrol. 2016 Dec;27(12):3628-3638. Epub 2016 May 6.

18.

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.

19.

Human polycystin-2 transgene dose-dependently rescues ADPKD phenotypes in Pkd2 mutant mice.

Li A, Tian X, Zhang X, Huang S, Ma Y, Wu D, Moeckel G, Somlo S, Wu G.

Am J Pathol. 2015 Oct;185(10):2843-60. doi: 10.1016/j.ajpath.2015.06.014.

20.

Essential Role of X-Box Binding Protein-1 during Endoplasmic Reticulum Stress in Podocytes.

Hassan H, Tian X, Inoue K, Chai N, Liu C, Soda K, Moeckel G, Tufro A, Lee AH, Somlo S, Fedeles S, Ishibe S.

J Am Soc Nephrol. 2016 Apr;27(4):1055-65. doi: 10.1681/ASN.2015020191. Epub 2015 Aug 24.

21.

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.

22.

The Future of Polycystic Kidney Disease Research--As Seen By the 12 Kaplan Awardees.

Antignac C, Calvet JP, Germino GG, Grantham JJ, Guay-Woodford LM, Harris PC, Hildebrandt F, Peters DJ, Somlo S, Torres VE, Walz G, Zhou J, Yu AS.

J Am Soc Nephrol. 2015 Sep;26(9):2081-95. doi: 10.1681/ASN.2014121192. Epub 2015 May 7.

23.

Polycystin-1 Is a Cardiomyocyte Mechanosensor That Governs L-Type Ca2+ Channel Protein Stability.

Pedrozo Z, Criollo A, Battiprolu PK, Morales CR, Contreras-Ferrat A, Fernández C, Jiang N, Luo X, Caplan MJ, Somlo S, Rothermel BA, Gillette TG, Lavandero S, Hill JA.

Circulation. 2015 Jun 16;131(24):2131-42. doi: 10.1161/CIRCULATIONAHA.114.013537. Epub 2015 Apr 17.

24.

Sec63 and Xbp1 regulate IRE1α activity and polycystic disease severity.

Fedeles SV, So JS, Shrikhande A, Lee SH, Gallagher AR, Barkauskas CE, Somlo S, Lee AH.

J Clin Invest. 2015 May;125(5):1955-67. doi: 10.1172/JCI78863. Epub 2015 Apr 6.

25.

Altered trafficking and stability of polycystins underlie polycystic kidney disease.

Cai Y, Fedeles SV, Dong K, Anyatonwu G, Onoe T, Mitobe M, Gao JD, Okuhara D, Tian X, Gallagher AR, Tang Z, Xie X, Lalioti MD, Lee AH, Ehrlich BE, Somlo S.

J Clin Invest. 2014 Dec;124(12):5129-44. doi: 10.1172/JCI67273. Epub 2014 Nov 3.

26.

The kidney research national dialogue: gearing up to move forward.

Bonventre JV, Boulware LE, Dember LM, Freedman BI, Furth SL, Holzman LB, Ketchum CJ, Little MH, Mehrotra R, Moe SM, Sands JM, Sedor JR, Somlo S, Star RA, Rys-Sikora KE; Kidney Research National Dialogue (KRND).

Clin J Am Soc Nephrol. 2014 Oct 7;9(10):1806-11. doi: 10.2215/CJN.07310714. Epub 2014 Sep 15. Review.

27.

The imperative to invest in science has never been greater.

Carethers JM, Coughlin S, Diamond B, Erzurum S, Fried LP, Jameson JL, Kaushansky K, Klotman ME, Lemon S, Mitchell B, Rothman P, Sawyers C, Seidman C, Somlo S.

J Clin Invest. 2014 Sep;124(9):3680-1. doi: 10.1172/JCI77894. Epub 2014 Sep 2. No abstract available.

28.

Filling the holes in cystic kidney disease research.

Guay-Woodford LM, Henske E, Igarashi P, Perrone RD, Reed-Gitomer B, Somlo S, Torres VE, Ketchum CJ, Star RA, Flessner MF, Rasooly RS.

Clin J Am Soc Nephrol. 2014 Oct 7;9(10):1799-801. doi: 10.2215/CJN.03410414. Epub 2014 Jun 5. Review.

29.

Cyst growth, polycystins, and primary cilia in autosomal dominant polycystic kidney disease.

Lee SH, Somlo S.

Kidney Res Clin Pract. 2014 Jun;33(2):73-8. doi: 10.1016/j.krcp.2014.05.002. Epub 2014 Jun 14. Review.

30.

N-glycosylation determines the abundance of the transient receptor potential channel TRPP2.

Hofherr A, Wagner C, Fedeles S, Somlo S, Köttgen M.

J Biol Chem. 2014 May 23;289(21):14854-67. doi: 10.1074/jbc.M114.562264. Epub 2014 Apr 9.

31.

Steviol retards renal cyst growth through reduction of CFTR expression and inhibition of epithelial cell proliferation in a mouse model of polycystic kidney disease.

Yuajit C, Muanprasat C, Gallagher AR, Fedeles SV, Kittayaruksakul S, Homvisasevongsa S, Somlo S, Chatsudthipong V.

Biochem Pharmacol. 2014 Apr 1;88(3):412-21. doi: 10.1016/j.bcp.2014.01.038. Epub 2014 Feb 8.

PMID:
24518257
32.

Polycystin-1: a master regulator of intersecting cystic pathways.

Fedeles SV, Gallagher AR, Somlo S.

Trends Mol Med. 2014 May;20(5):251-60. doi: 10.1016/j.molmed.2014.01.004. Epub 2014 Jan 31. Review.

33.

Loss of cilia suppresses cyst growth in genetic models of autosomal dominant polycystic kidney disease.

Ma M, Tian X, Igarashi P, Pazour GJ, Somlo S.

Nat Genet. 2013 Sep;45(9):1004-12. doi: 10.1038/ng.2715. Epub 2013 Jul 28.

34.

miR-17~92 miRNA cluster promotes kidney cyst growth in polycystic kidney disease.

Patel V, Williams D, Hajarnis S, Hunter R, Pontoglio M, Somlo S, Igarashi P.

Proc Natl Acad Sci U S A. 2013 Jun 25;110(26):10765-70. doi: 10.1073/pnas.1301693110. Epub 2013 Jun 12.

35.

Mechanoprotection by polycystins against apoptosis is mediated through the opening of stretch-activated K(2P) channels.

Peyronnet R, Sharif-Naeini R, Folgering JH, Arhatte M, Jodar M, El Boustany C, Gallian C, Tauc M, Duranton C, Rubera I, Lesage F, Pei Y, Peters DJ, Somlo S, Sachs F, Patel A, Honoré E, Duprat F.

Cell Rep. 2012 Mar 29;1(3):241-50. doi: 10.1016/j.celrep.2012.01.006. Epub 2012 Mar 8.

36.

Polycystin-1 regulates amphiregulin expression through CREB and AP1 signalling: implications in ADPKD cell proliferation.

Aguiari G, Bizzarri F, Bonon A, Mangolini A, Magri E, Pedriali M, Querzoli P, Somlo S, Harris PC, Catizone L, Del Senno L.

J Mol Med (Berl). 2012 Nov;90(11):1267-82. doi: 10.1007/s00109-012-0902-3. Epub 2012 May 9.

37.

Different effects of Sec61α, Sec62 and Sec63 depletion on transport of polypeptides into the endoplasmic reticulum of mammalian cells.

Lang S, Benedix J, Fedeles SV, Schorr S, Schirra C, Schäuble N, Jalal C, Greiner M, Hassdenteufel S, Tatzelt J, Kreutzer B, Edelmann L, Krause E, Rettig J, Somlo S, Zimmermann R, Dudek J.

J Cell Sci. 2012 Apr 15;125(Pt 8):1958-69. doi: 10.1242/jcs.096727. Epub 2012 Feb 28.

38.

Evaluation of urine biomarkers of kidney injury in polycystic kidney disease.

Parikh CR, Dahl NK, Chapman AB, Bost JE, Edelstein CL, Comer DM, Zeltner R, Tian X, Grantham JJ, Somlo S.

Kidney Int. 2012 Apr;81(8):784-90. doi: 10.1038/ki.2011.465. Epub 2012 Jan 18.

39.

The γ-secretase cleavage product of polycystin-1 regulates TCF and CHOP-mediated transcriptional activation through a p300-dependent mechanism.

Merrick D, Chapin H, Baggs JE, Yu Z, Somlo S, Sun Z, Hogenesch JB, Caplan MJ.

Dev Cell. 2012 Jan 17;22(1):197-210. doi: 10.1016/j.devcel.2011.10.028. Epub 2011 Dec 15.

40.

Macrophages promote cyst growth in polycystic kidney disease.

Karihaloo A, Koraishy F, Huen SC, Lee Y, Merrick D, Caplan MJ, Somlo S, Cantley LG.

J Am Soc Nephrol. 2011 Oct;22(10):1809-14. doi: 10.1681/ASN.2011010084. Epub 2011 Sep 15.

41.

A genetic interaction network of five genes for human polycystic kidney and liver diseases defines polycystin-1 as the central determinant of cyst formation.

Fedeles SV, Tian X, Gallagher AR, Mitobe M, Nishio S, Lee SH, Cai Y, Geng L, Crews CM, Somlo S.

Nat Genet. 2011 Jun 19;43(7):639-47. doi: 10.1038/ng.860.

42.

Polycystin-2 and phosphodiesterase 4C are components of a ciliary A-kinase anchoring protein complex that is disrupted in cystic kidney diseases.

Choi YH, Suzuki A, Hajarnis S, Ma Z, Chapin HC, Caplan MJ, Pontoglio M, Somlo S, Igarashi P.

Proc Natl Acad Sci U S A. 2011 Jun 28;108(26):10679-84. doi: 10.1073/pnas.1016214108. Epub 2011 Jun 13.

43.

Polycystic liver diseases: congenital disorders of cholangiocyte signaling.

Strazzabosco M, Somlo S.

Gastroenterology. 2011 Jun;140(7):1855-9, 1859.e1. doi: 10.1053/j.gastro.2011.04.030. Epub 2011 Apr 22. Review.

44.

T-cell factor/β-catenin activity is suppressed in two different models of autosomal dominant polycystic kidney disease.

Miller MM, Iglesias DM, Zhang Z, Corsini R, Chu L, Murawski I, Gupta I, Somlo S, Germino GG, Goodyer PR.

Kidney Int. 2011 Jul;80(2):146-53. doi: 10.1038/ki.2011.56. Epub 2011 Mar 9.

45.

Activating AMP-activated protein kinase (AMPK) slows renal cystogenesis.

Takiar V, Nishio S, Seo-Mayer P, King JD Jr, Li H, Zhang L, Karihaloo A, Hallows KR, Somlo S, Caplan MJ.

Proc Natl Acad Sci U S A. 2011 Feb 8;108(6):2462-7. doi: 10.1073/pnas.1011498108. Epub 2011 Jan 24.

46.

Polycystin-2 activation by inositol 1,4,5-trisphosphate-induced Ca2+ release requires its direct association with the inositol 1,4,5-trisphosphate receptor in a signaling microdomain.

Sammels E, Devogelaere B, Mekahli D, Bultynck G, Missiaen L, Parys JB, Cai Y, Somlo S, De Smedt H.

J Biol Chem. 2010 Jun 11;285(24):18794-805. doi: 10.1074/jbc.M109.090662. Epub 2010 Apr 7.

47.

Molecular advances in autosomal dominant polycystic kidney disease.

Gallagher AR, Germino GG, Somlo S.

Adv Chronic Kidney Dis. 2010 Mar;17(2):118-30. doi: 10.1053/j.ackd.2010.01.002. Review.

48.

Mammalian target of rapamycin regulates vascular endothelial growth factor-dependent liver cyst growth in polycystin-2-defective mice.

Spirli C, Okolicsanyi S, Fiorotto R, Fabris L, Cadamuro M, Lecchi S, Tian X, Somlo S, Strazzabosco M.

Hepatology. 2010 May;51(5):1778-88. doi: 10.1002/hep.23511.

49.

Secondary and tertiary structure modeling reveals effects of novel mutations in polycystic liver disease genes PRKCSH and SEC63.

Waanders E, Venselaar H, te Morsche RH, de Koning DB, Kamath PS, Torres VE, Somlo S, Drenth JP.

Clin Genet. 2010 Jul;78(1):47-56. doi: 10.1111/j.1399-0004.2009.01353.x. Epub 2010 Jan 20.

50.

Chemical modifier screen identifies HDAC inhibitors as suppressors of PKD models.

Cao Y, Semanchik N, Lee SH, Somlo S, Barbano PE, Coifman R, Sun Z.

Proc Natl Acad Sci U S A. 2009 Dec 22;106(51):21819-24. doi: 10.1073/pnas.0911987106. Epub 2009 Dec 4.

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