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Items: 1 to 50 of 142

1.

Polycystic Kidney Disease, Autosomal Recessive.

Sweeney WE, Avner ED.

In: Adam MP, Ardinger HH, Pagon RA, Wallace SE, Bean LJH, Stephens K, Amemiya A, editors. GeneReviews® [Internet]. Seattle (WA): University of Washington, Seattle; 1993-2019.
2001 Jul 19 [updated 2019 Feb 14].

2.

Identification of molecular signatures of cystic fibrosis disease status with plasma-based functional genomics.

Levy H, Jia S, Pan A, Zhang X, Kaldunski M, Nugent ML, Reske M, Feliciano RA, Quintero D, Renda MM, Woods KJ, Murkowski K, Johnson K, Verbsky J, Dasu T, Ideozu JE, McColley S, Quasney MW, Dahmer MK, Avner E, Farrell PM, Cannon CL, Jacob H, Simpson PM, Hessner MJ.

Physiol Genomics. 2019 Jan 1;51(1):27-41. doi: 10.1152/physiolgenomics.00109.2018. Epub 2018 Dec 12.

PMID:
30540547
3.

Unique interstitial miRNA signature drives fibrosis in a murine model of autosomal dominant polycystic kidney disease.

Patil A, Sweeney WE Jr, Pan CG, Avner ED.

World J Nephrol. 2018 Sep 7;7(5):108-116. doi: 10.5527/wjn.v7.i5.108. Review.

4.

Tesevatinib ameliorates progression of polycystic kidney disease in rodent models of autosomal recessive polycystic kidney disease.

Sweeney WE, Frost P, Avner ED.

World J Nephrol. 2017 Jul 6;6(4):188-200. doi: 10.5527/wjn.v6.i4.188.

5.

Emerging Therapies for Childhood Polycystic Kidney Disease.

Sweeney WE Jr, Avner ED.

Front Pediatr. 2017 Apr 19;5:77. doi: 10.3389/fped.2017.00077. eCollection 2017. Review.

6.

Childhood Polycystic Kidney Disease.

Patil A, Sweeney WE Jr., Avner ED, Pan C.

In: Li X, editor. Polycystic Kidney Disease [Internet]. Brisbane (AU): Codon Publications; 2015 Nov. Chapter 2.

7.

Pathophysiology of childhood polycystic kidney diseases: new insights into disease-specific therapy.

Sweeney WE Jr, Avner ED.

Pediatr Res. 2014 Jan;75(1-2):148-57. doi: 10.1038/pr.2013.191. Epub 2013 Oct 31. Review.

8.

Smac-mimetic-induced epithelial cell death reduces the growth of renal cysts.

Fan LX, Zhou X, Sweeney WE Jr, Wallace DP, Avner ED, Grantham JJ, Li X.

J Am Soc Nephrol. 2013 Dec;24(12):2010-22. doi: 10.1681/ASN.2013020176. Epub 2013 Aug 29.

9.

Sirtuin 1 inhibition delays cyst formation in autosomal-dominant polycystic kidney disease.

Zhou X, Fan LX, Sweeney WE Jr, Denu JM, Avner ED, Li X.

J Clin Invest. 2013 Jul;123(7):3084-98. doi: 10.1172/JCI64401. Epub 2013 Jun 17.

10.

New approaches to the autosomal recessive polycystic kidney disease patient with dual kidney-liver complications.

Telega G, Cronin D, Avner ED.

Pediatr Transplant. 2013 Jun;17(4):328-35. doi: 10.1111/petr.12076. Epub 2013 Apr 17. Review.

11.

G-protein signaling modulator 1 deficiency accelerates cystic disease in an orthologous mouse model of autosomal dominant polycystic kidney disease.

Kwon M, Pavlov TS, Nozu K, Rasmussen SA, Ilatovskaya DV, Lerch-Gaggl A, North LM, Kim H, Qian F, Sweeney WE Jr, Avner ED, Blumer JB, Staruschenko A, Park F.

Proc Natl Acad Sci U S A. 2012 Dec 26;109(52):21462-7. doi: 10.1073/pnas.1216830110. Epub 2012 Dec 10.

12.

HDAC6 regulates epidermal growth factor receptor (EGFR) endocytic trafficking and degradation in renal epithelial cells.

Liu W, Fan LX, Zhou X, Sweeney WE Jr, Avner ED, Li X.

PLoS One. 2012;7(11):e49418. doi: 10.1371/journal.pone.0049418. Epub 2012 Nov 13.

13.

Role of genetic modifiers in an orthologous rat model of ARPKD.

O'Meara CC, Hoffman M, Sweeney WE Jr, Tsaih SW, Xiao B, Jacob HJ, Avner ED, Moreno C.

Physiol Genomics. 2012 Aug 1;44(15):741-53. doi: 10.1152/physiolgenomics.00187.2011. Epub 2012 Jun 5.

14.

Transport, cilia, and PKD: must we in (cyst) on interrelationships? Focus on "Increased Na+/H+ exchanger activity on the apical surface of a cilium-deficient cortical collecting duct principal cell model of polycystic kidney disease".

Avner ED, McDonough AA, Sweeney WE Jr.

Am J Physiol Cell Physiol. 2012 May 15;302(10):C1434-5. doi: 10.1152/ajpcell.00070.2012. Epub 2012 Mar 7. No abstract available.

15.

Loss of activator of G-protein signaling 3 impairs renal tubular regeneration following acute kidney injury in rodents.

Regner KR, Nozu K, Lanier SM, Blumer JB, Avner ED, Sweeney WE Jr, Park F.

FASEB J. 2011 Jun;25(6):1844-55. doi: 10.1096/fj.10-169797. Epub 2011 Feb 22.

16.

Diagnosis and management of childhood polycystic kidney disease.

Sweeney WE Jr, Avner ED.

Pediatr Nephrol. 2011 May;26(5):675-92. doi: 10.1007/s00467-010-1656-1. Epub 2010 Oct 29. Review.

PMID:
21046169
17.

Birth of a healthy infant following preimplantation PKHD1 haplotyping for autosomal recessive polycystic kidney disease using multiple displacement amplification.

Lau EC, Janson MM, Roesler MR, Avner ED, Strawn EY, Bick DP.

J Assist Reprod Genet. 2010 Jul;27(7):397-407. doi: 10.1007/s10815-010-9432-5. Epub 2010 May 20.

18.

20-HETE activates the Raf/MEK/ERK pathway in renal epithelial cells through an EGFR- and c-Src-dependent mechanism.

Akbulut T, Regner KR, Roman RJ, Avner ED, Falck JR, Park F.

Am J Physiol Renal Physiol. 2009 Sep;297(3):F662-70. doi: 10.1152/ajprenal.00146.2009. Epub 2009 Jul 1.

19.

Chronic blockade of 20-HETE synthesis reduces polycystic kidney disease in an orthologous rat model of ARPKD.

Park F, Sweeney WE Jr, Jia G, Akbulut T, Mueller B, Falck JR, Birudaraju S, Roman RJ, Avner ED.

Am J Physiol Renal Physiol. 2009 Mar;296(3):F575-82. doi: 10.1152/ajprenal.90705.2008. Epub 2009 Jan 7. Erratum in: Am J Physiol Renal Physiol. 2009 Oct;297(4):F1135.

20.

20-HETE mediates proliferation of renal epithelial cells in polycystic kidney disease.

Park F, Sweeney WE, Jia G, Roman RJ, Avner ED.

J Am Soc Nephrol. 2008 Oct;19(10):1929-39. doi: 10.1681/ASN.2007070771. Epub 2008 Jul 2.

21.

Src inhibition ameliorates polycystic kidney disease.

Sweeney WE Jr, von Vigier RO, Frost P, Avner ED.

J Am Soc Nephrol. 2008 Jul;19(7):1331-41. doi: 10.1681/ASN.2007060665. Epub 2008 Apr 2.

22.

Renal cystic disease: new insights for the clinician.

Avner ED, Sweeney WE Jr.

Pediatr Clin North Am. 2006 Oct;53(5):889-909, ix. Review.

PMID:
17027616
23.

Autosomal recessive polycystic kidney disease and congenital hepatic fibrosis: summary statement of a first National Institutes of Health/Office of Rare Diseases conference.

Gunay-Aygun M, Avner ED, Bacallao RL, Choyke PL, Flynn JT, Germino GG, Guay-Woodford L, Harris P, Heller T, Ingelfinger J, Kaskel F, Kleta R, LaRusso NF, Mohan P, Pazour GJ, Shneider BL, Torres VE, Wilson P, Zak C, Zhou J, Gahl WA.

J Pediatr. 2006 Aug;149(2):159-64. No abstract available.

24.

Molecular and cellular pathophysiology of autosomal recessive polycystic kidney disease (ARPKD).

Sweeney WE Jr, Avner ED.

Cell Tissue Res. 2006 Dec;326(3):671-85. Epub 2006 Jun 10. Review.

PMID:
16767405
25.

Haplotype analysis improves molecular diagnostics of autosomal recessive polycystic kidney disease.

Consugar MB, Anderson SA, Rossetti S, Pankratz VS, Ward CJ, Torra R, Coto E, El-Youssef M, Kantarci S, Utsch B, Hildebrandt F, Sweeney WE, Avner ED, Torres VE, Cunningham JM, Harris PC.

Am J Kidney Dis. 2005 Jan;45(1):77-87.

PMID:
15696446
26.

Epidermal growth factor receptor tyrosine kinase inhibition is not protective in PCK rats.

Torres VE, Sweeney WE Jr, Wang X, Qian Q, Harris PC, Frost P, Avner ED.

Kidney Int. 2004 Nov;66(5):1766-73.

27.

EGF-related growth factors in the pathogenesis of murine ARPKD.

MacRae Dell K, Nemo R, Sweeney WE Jr, Avner ED.

Kidney Int. 2004 Jun;65(6):2018-29.

28.
29.

EGF receptor tyrosine kinase inhibition attenuates the development of PKD in Han:SPRD rats.

Torres VE, Sweeney WE Jr, Wang X, Qian Q, Harris PC, Frost P, Avner ED.

Kidney Int. 2003 Nov;64(5):1573-9.

30.

Combination treatment of PKD utilizing dual inhibition of EGF-receptor activity and ligand bioavailability.

Sweeney WE Jr, Hamahira K, Sweeney J, Garcia-Gatrell M, Frost P, Avner ED.

Kidney Int. 2003 Oct;64(4):1310-9.

31.

Effect of the peritoneal dialysis prescription on pentosidine in children.

Bayazit AK, Vogt BA, Dell KM, Davis ID, Noyan A, Anarat A, Avner ED, Erhard P, Weiss MF.

Pediatr Nephrol. 2003 Oct;18(10):1049-54. Epub 2003 Jul 29.

PMID:
12898376
32.

Milder presentation of recessive polycystic kidney disease requires presence of amino acid substitution mutations.

Furu L, Onuchic LF, Gharavi A, Hou X, Esquivel EL, Nagasawa Y, Bergmann C, Senderek J, Avner E, Zerres K, Germino GG, Guay-Woodford LM, Somlo S.

J Am Soc Nephrol. 2003 Aug;14(8):2004-14.

33.

Polycystin-1 distribution is modulated by polycystin-2 expression in mammalian cells.

Grimm DH, Cai Y, Chauvet V, Rajendran V, Zeltner R, Geng L, Avner ED, Sweeney W, Somlo S, Caplan MJ.

J Biol Chem. 2003 Sep 19;278(38):36786-93. Epub 2003 Jul 2.

34.

Complications of peritoneal dialysis in children with Eagle-Barrett syndrome.

Wisanuyotin S, Dell KM, Vogt BA, O'Riordan MA, Avner ED, Davis ID.

Pediatr Nephrol. 2003 Feb;18(2):159-63. Epub 2002 Dec 19.

PMID:
12579407
35.

Critical choices in mentoring the next generation of academic pediatricians: nine circles of hell or salvation?

Drotar D, Avner ED.

J Pediatr. 2003 Jan;142(1):1-2. No abstract available.

PMID:
12520243
36.

Identification and characterization of Pkhd1, the mouse orthologue of the human ARPKD gene.

Nagasawa Y, Matthiesen S, Onuchic LF, Hou X, Bergmann C, Esquivel E, Senderek J, Ren Z, Zeltner R, Furu L, Avner E, Moser M, Somlo S, Guay-Woodford L, Büttner R, Zerres K, Germino GG.

J Am Soc Nephrol. 2002 Sep;13(9):2246-58.

37.

Refinement of the autosomal recessive polycystic kidney disease (PKHD1) interval and exclusion of an EF hand-containing gene as a PKHD1 candidate gene.

Onuchic LF, Mrug M, Hou X, Eggermann T, Bergmann C, Zerres K, Avner ED, Furu L, Somlo S, Nagasawa Y, Germino GG, Guay-Woodford LM.

Am J Med Genet. 2002 Jul 15;110(4):346-52.

PMID:
12116208
38.

PKHD1, the polycystic kidney and hepatic disease 1 gene, encodes a novel large protein containing multiple immunoglobulin-like plexin-transcription-factor domains and parallel beta-helix 1 repeats.

Onuchic LF, Furu L, Nagasawa Y, Hou X, Eggermann T, Ren Z, Bergmann C, Senderek J, Esquivel E, Zeltner R, Rudnik-Schöneborn S, Mrug M, Sweeney W, Avner ED, Zerres K, Guay-Woodford LM, Somlo S, Germino GG.

Am J Hum Genet. 2002 May;70(5):1305-17. Epub 2002 Mar 15.

39.

Phenotypic analysis of conditionally immortalized cells isolated from the BPK model of ARPKD.

Sweeney WE Jr, Kusner L, Carlin CR, Chang S, Futey L, Cotton CU, Dell KM, Avner ED.

Am J Physiol Cell Physiol. 2001 Nov;281(5):C1695-705.

40.

A novel inhibitor of tumor necrosis factor-alpha converting enzyme ameliorates polycystic kidney disease.

Dell KM, Nemo R, Sweeney WE Jr, Levin JI, Frost P, Avner ED.

Kidney Int. 2001 Oct;60(4):1240-8.

41.

Can progression of autosomal dominant or autosomal recessive polycystic kidney disease be prevented?

Davis ID, MacRae Dell K, Sweeney WE, Avner ED.

Semin Nephrol. 2001 Sep;21(5):430-40. Review.

PMID:
11559884
42.

Expression of the orpk disease gene during kidney development and maturation.

Nakanishi K, Sweeney WE Jr, Avner ED, Murcia NS.

Pediatr Nephrol. 2001 Mar;16(3):219-26.

PMID:
11322368
43.

Childhood ADPKD: answers and more questions.

Avner ED.

Kidney Int. 2001 May;59(5):1979-80. No abstract available.

44.

Role of CFTR in autosomal recessive polycystic kidney disease.

Nakanishi K, Sweeney WE Jr, Macrae Dell K, Cotton CU, Avner ED.

J Am Soc Nephrol. 2001 Apr;12(4):719-25.

45.

Renal dysfunction but not cystic change is ameliorated by neonatal epidermal growth factor in bpk mice.

Nakanishi K, Gattone VH 2nd, Sweeney WE, Avner ED.

Pediatr Nephrol. 2001 Jan;16(1):45-50.

PMID:
11198603
46.

Segment-specific c-ErbB2 expression in human autosomal recessive polycystic kidney disease.

Nakanishi K, Sweeney W Jr, Avner ED.

J Am Soc Nephrol. 2001 Feb;12(2):379-84.

47.

Generation and phenotype of cell lines derived from CF and non-CF mice that carry the H-2K(b)-tsA58 transgene.

Takacs-Jarrett M, Sweeney WE, Avner ED, Cotton CU.

Am J Physiol Cell Physiol. 2001 Jan;280(1):C228-36.

48.

Genomic structure of the gene for the human P1 protein (MCM3) and its exclusion as a candidate for autosomal recessive polycystic kidney disease.

Hofmann Y, Becker J, Wright F, Avner ED, Mrug M, Guay-Woodford LM, Somlo S, Zerres K, Germino GG, Onuchic LF.

Eur J Hum Genet. 2000 Mar;8(3):163-6.

49.

Proximal tubular cysts in fetal human autosomal recessive polycystic kidney disease.

Nakanishi K, Sweeney WE Jr, Zerres K, Guay-Woodford LM, Avner ED.

J Am Soc Nephrol. 2000 Apr;11(4):760-3.

50.

Treatment of polycystic kidney disease with a novel tyrosine kinase inhibitor.

Sweeney WE, Chen Y, Nakanishi K, Frost P, Avner ED.

Kidney Int. 2000 Jan;57(1):33-40.

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