Literature DB >> 19321761

Long-term rapamycin therapy in the Han:SPRD rat model of polycystic kidney disease (PKD).

Iram Zafar1, Franck A Belibi, Zhibin He, Charles L Edelstein.   

Abstract

BACKGROUND: Short-term studies have demonstrated that rapamycin or everolimus treatment decreases cyst formation and improves renal function in animal models of polycystic kidney disease (PKD). Autosomal dominant polycystic kidney disease (ADPKD) patients would likely require life-long treatment with rapamycin.
METHODS: Male Han:SPRD rats with PKD (Cy/+) were treated with rapamycin (0.2 mg/kg/day IP) or vehicle from 1 to 12 months of age. Mean trough levels of rapamycin (ng/mL) were 6.6 +/- 0.1 at 8 weeks of age. Twelve-month-old littermates (+/+) were used as normal controls.
RESULTS: Twelve-month-old male Cy/+ rats treated with the vehicle had a more than doubling of kidney volume, severe chronic renal failure, severe hypertension and increased heart weight compared to normal littermate controls (+/+). After rapamycin treatment, 12-month-old Cy/+ rats had markedly improved kidney volume, renal function, blood pressure and heart weight not statistically different from controls. Rapamycin reduced the cyst volume density (CVD) by 72%. Mammalian target of rapamycin (mTOR) activation in the heart, as evidenced by a marked increase in the phospho-S6 protein that was inhibited by rapamycin, was demonstrated in 12-month-old Cy/+ rats.
CONCLUSION: In conclusion, long-term rapamycin treatment in Cy/+ rats results in a normalization of kidney volume, renal function, blood pressure and heart weight. The novel finding that rapamycin decreases hypertension, heart enlargement and mTOR signalling in the heart in PKD rats is reported. The only side effect of rapamycin treatment was an 11% decrease in body weight.

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Year:  2009        PMID: 19321761      PMCID: PMC2727300          DOI: 10.1093/ndt/gfp129

Source DB:  PubMed          Journal:  Nephrol Dial Transplant        ISSN: 0931-0509            Impact factor:   5.992


  24 in total

Review 1.  Hypertension in autosomal-dominant polycystic kidney disease: early occurrence and unique aspects.

Authors:  Tevfik Ecder; Robert W Schrier
Journal:  J Am Soc Nephrol       Date:  2001-01       Impact factor: 10.121

2.  Hypertension and renal injury in experimental polycystic kidney disease.

Authors:  T M Kennefick; M A Al-Nimri; T T Oyama; M M Thompson; F J Kelly; J G Chapman; S Anderson
Journal:  Kidney Int       Date:  1999-12       Impact factor: 10.612

3.  Gender and the effect of gonadal hormones on the progression of inherited polycystic kidney disease in rats.

Authors:  B D Cowley; J C Rupp; M J Muessel; V H Gattone
Journal:  Am J Kidney Dis       Date:  1997-02       Impact factor: 8.860

4.  The mTOR pathway is regulated by polycystin-1, and its inhibition reverses renal cystogenesis in polycystic kidney disease.

Authors:  Jonathan M Shillingford; Noel S Murcia; Claire H Larson; Seng Hui Low; Ryan Hedgepeth; Nicole Brown; Chris A Flask; Andrew C Novick; David A Goldfarb; Albrecht Kramer-Zucker; Gerd Walz; Klaus B Piontek; Gregory G Germino; Thomas Weimbs
Journal:  Proc Natl Acad Sci U S A       Date:  2006-03-27       Impact factor: 11.205

5.  Reversal of left ventricular hypertrophy with angiotensin converting enzyme inhibition in hypertensive patients with autosomal dominant polycystic kidney disease.

Authors:  T Ecder; C L Edelstein; A B Chapman; A M Johnson; L Tison; E A Gill; G M Brosnahan; R W Schrier
Journal:  Nephrol Dial Transplant       Date:  1999-05       Impact factor: 5.992

6.  Downregulation of the calpain inhibitor protein calpastatin by caspases during renal ischemia-reperfusion.

Authors:  Y Shi; V Y Melnikov; R W Schrier; C L Edelstein
Journal:  Am J Physiol Renal Physiol       Date:  2000-09

7.  Inhibition of mTOR with sirolimus slows disease progression in Han:SPRD rats with autosomal dominant polycystic kidney disease (ADPKD).

Authors:  Patricia R Wahl; Andreas L Serra; Michel Le Hir; Klaus D Molle; Michael N Hall; Rudolf P Wüthrich
Journal:  Nephrol Dial Transplant       Date:  2005-10-12       Impact factor: 5.992

8.  Effect of sirolimus on left ventricular hypertrophy in kidney transplant recipients: a 1-year nonrandomized controlled trial.

Authors:  Ernesto Paoletti; Marco Amidone; Paolo Cassottana; Maurizio Gherzi; Luigina Marsano; Giuseppe Cannella
Journal:  Am J Kidney Dis       Date:  2008-06-30       Impact factor: 8.860

9.  Everolimus retards cyst growth and preserves kidney function in a rodent model for polycystic kidney disease.

Authors:  Ming Wu; Patricia R Wahl; Michel Le Hir; Ying Wackerle-Men; Rudolf P Wuthrich; Andreas L Serra
Journal:  Kidney Blood Press Res       Date:  2007-06-27       Impact factor: 2.687

10.  Effect of statin and angiotensin-converting enzyme inhibition on structural and hemodynamic alterations in autosomal dominant polycystic kidney disease model.

Authors:  Iram Zafar; Yunxia Tao; Sandor Falk; Kimberly McFann; Robert W Schrier; Charles L Edelstein
Journal:  Am J Physiol Renal Physiol       Date:  2007-06-20
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  35 in total

1.  Hypoxia-inducible factor-1α (HIF-1α) and autophagy in polycystic kidney disease (PKD).

Authors:  Franck Belibi; Iram Zafar; Kameswaran Ravichandran; Anamarija Bauer Segvic; Alkesh Jani; Danica Galesic Ljubanovic; Charles L Edelstein
Journal:  Am J Physiol Renal Physiol       Date:  2011-01-26

2.  Effect of Sirolimus on Disease Progression in Patients with Autosomal Dominant Polycystic Kidney Disease and CKD Stages 3b-4.

Authors:  Piero Ruggenenti; Giorgio Gentile; Norberto Perico; Annalisa Perna; Luca Barcella; Matias Trillini; Monica Cortinovis; Claudia Patricia Ferrer Siles; Jorge Arturo Reyes Loaeza; Maria Carolina Aparicio; Giorgio Fasolini; Flavio Gaspari; Davide Martinetti; Fabiola Carrara; Nadia Rubis; Silvia Prandini; Anna Caroli; Kanishka Sharma; Luca Antiga; Andrea Remuzzi; Giuseppe Remuzzi
Journal:  Clin J Am Soc Nephrol       Date:  2016-02-22       Impact factor: 8.237

3.  An mTOR anti-sense oligonucleotide decreases polycystic kidney disease in mice with a targeted mutation in Pkd2.

Authors:  Kameswaran Ravichandran; Iram Zafar; Zhibin He; R Brian Doctor; Radu Moldovan; Adam E Mullick; Charles L Edelstein
Journal:  Hum Mol Genet       Date:  2014-05-08       Impact factor: 6.150

4.  Autophagy activators suppress cystogenesis in an autosomal dominant polycystic kidney disease model.

Authors:  Ping Zhu; Cynthia J Sieben; Xiaolei Xu; Peter C Harris; Xueying Lin
Journal:  Hum Mol Genet       Date:  2017-01-01       Impact factor: 6.150

5.  Dose-dependent effects of sirolimus on mTOR signaling and polycystic kidney disease.

Authors:  Zlata Novalic; Annemieke M van der Wal; Wouter N Leonhard; Gudrun Koehl; Martijn H Breuning; Edward K Geissler; Emile de Heer; Dorien J M Peters
Journal:  J Am Soc Nephrol       Date:  2012-02-16       Impact factor: 10.121

Review 6.  Drug discovery for polycystic kidney disease.

Authors:  Ying Sun; Hong Zhou; Bao-xue Yang
Journal:  Acta Pharmacol Sin       Date:  2011-06       Impact factor: 6.150

Review 7.  Looking at the (w)hole: magnet resonance imaging in polycystic kidney disease.

Authors:  Max Christoph Liebau; Andreas Lucas Serra
Journal:  Pediatr Nephrol       Date:  2012-12-14       Impact factor: 3.714

8.  Polycystin-1 and Gα12 regulate the cleavage of E-cadherin in kidney epithelial cells.

Authors:  Jen X Xu; Tzong-Shi Lu; Suyan Li; Yong Wu; Lai Ding; Bradley M Denker; Joseph V Bonventre; Tianqing Kong
Journal:  Physiol Genomics       Date:  2014-12-09       Impact factor: 3.107

9.  Rapamycin ameliorates PKD resulting from conditional inactivation of Pkd1.

Authors:  Jonathan M Shillingford; Klaus B Piontek; Gregory G Germino; Thomas Weimbs
Journal:  J Am Soc Nephrol       Date:  2010-01-14       Impact factor: 10.121

10.  Polycystin-1 but not polycystin-2 deficiency causes upregulation of the mTOR pathway and can be synergistically targeted with rapamycin and metformin.

Authors:  Djalila Mekahli; Jean-Paul Decuypere; Eva Sammels; Kirsten Welkenhuyzen; Joost Schoeber; Marie-Pierre Audrezet; Anniek Corvelyn; Georges Dechênes; Albert C M Ong; Martijn J Wilmer; Lambertus van den Heuvel; Geert Bultynck; Jan B Parys; Ludwig Missiaen; Elena Levtchenko; Humbert De Smedt
Journal:  Pflugers Arch       Date:  2013-11-06       Impact factor: 3.657

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