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The Science

Establishing a Core Outcome Set for Autosomal Dominant Polycystic Kidney Disease: Report of the Standardized Outcomes in Nephrology-Polycystic Kidney Disease (SONG-PKD) Consensus Workshop
Cho Y, Tong A, Craig JC, Mustafa RA, Chapman A, Perrone RD, Ahn C, Fowler K, Torres V, Gansevoort RT, Ong ACM, Coolican H, Kao JTze-Wah, Harris T, Gutman T, Shen JI, Viecelli AK, Johnson DW, Au E, El-Damanawi R, Logeman C, Ju A, Manera KE, Chonchol M, Odland D, Baron D, Pei Y, Sautenet B, Rastogi A, Sharma A, et al.
 Am J Kidney Dis. (2020) Available online 6 August 2020, In Press. PubMed

Scientific publications from members of our team related to kidney health

STAT signaling in polycystic kidney disease

Strubl S, Torres JA, Spindt AK, Pellegrini H, Liebau MC, Weimbs T. Cellular Signalling (2020) 72:109639. PubMed

Ketosis Ameliorates Renal Cyst Growth in Polycystic Kidney Disease
Jacob A Torres, Samantha Kruger, Caroline Broderick, Tselmeg Amarlkhagva, Shagun Agrawal, John R Dodam, Michal Mrug, Leslie A Lyons, Thomas Weimbs. Cell Metabolism. (2019) 30:1007–1023. PubMed

Crystal deposition triggers tubule dilation that accelerates cystogenesis in polycystic kidney disease
Torres JA, Rezaei M, Broderick C, Lin L, Wang X, Hoppe B, Cowley BD, Savica V, Torres VE, Khan S, Holmes RP, Mrug M, Weimbs T. Journal of Clinical Investigation. (2019) 130:4506-4522. PubMed

  • News Release: “Strike Three: Researchers uncover a previously unrecognized mechanism…”
  • Highlighted in “ASN In the Loop” (American Society of Nephrology)
  • Research Highlight in Nature Reviews Nephrology, “Crystal deposition aids cystogenesis”
  • Recommendation in F1000Prime, 12 Sep 2019

Emerging targeted strategies for the treatment of autosomal dominant polycystic kidney disease
Weimbs T, Shillingford JM, Torres J, Kruger SL, Bourgeois BC. Clinical Kidney Journal. (2018) 11(suppl_1):i27–i38. PubMed

Find additional publications here at the National Library of Medicine

References.

  1. Allison SJ. Crystal deposition aids cystogenesis. Nat Rev Nephrol 15: 730–730, 2019. doi: 10.1038/s41581-019-0215-7.
  2. Blijdorp CJ, Severs D, Musterd-Bhaggoe UM, Gansevoort RT, Zietse R, Hoorn EJ, DIPAK Consortium. Serum bicarbonate is associated with kidney outcomes in autosomal dominant polycystic kidney disease. Nephrol Dial Transplant , 2020. doi: 10.1093/ndt/gfaa283.
  3. Carney EF. Ketosis slows the progression of PKD. Nat Rev Nephrol 16: 1–1, 2020. doi: 10.1038/s41581-019-0226-4.
  4. Cassina L, Chiaravalli M, Boletta A. Increased mitochondrial fragmentation in polycystic kidney disease acts as a modifier of disease progression. FASEB J 34: 6493–6507, 2020. doi: 10.1096/fj.201901739RR.
  5. Chebib FT, Torres VE. Recent Advances in the Management of Autosomal Dominant Polycystic Kidney Disease. Clin J Am Soc Nephrol 13(11): 1765–1776, 2018. doi: 10.2215/CJN.03960318.
  6. 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. 2-Deoxy-d-Glucose Ameliorates PKD Progression. J Am Soc Nephrol 27: 1958–69, 2016. doi: 10.1681/ASN.2015030231.
  7. Cogswell ME, Zhang Z, Carriquiry AL, Gunn JP, Kuklina EV, Saydah SH, Yang Q, Moshfegh AJ. Sodium and potassium intakes among US adults: NHANES 2003-2008. Am J Clin Nutr 96: 647–57, 2012. doi: 10.3945/ajcn.112.034413.
  8. Errasti P, Manrique J, Lavilla J, Rossich E, Hernandez A, Pujante D, Ndarabu A, Garcia N, Purroy A. Autosomal-dominant polycystic kidney disease: high prevalence of graft loss for death-related malignancies and cardiovascular risk factors. Transpl Proc 35: 1717–9, 2003. doi: 10.1016/s0041-1345(03)00619-5.
  9. Espinosa-Ortiz EJ, Eisner BH, Lange D, Gerlach R. Current insights into the mechanisms and management of infection stones. Nat Rev Urol 16: 35–53, 2019. doi: 10.1038/s41585-018-0120-z. 10. Grampsas SA, Chandhoke PS, Fan J, Glass MA, Townsend R, Johnson AM, Gabow P. Anatomic and metabolic risk factors for nephrolithiasis in patients with autosomal dominant polycystic kidney disease. Am J Kidney Dis 36: 53–7, 2000. doi: 10.1053/ajkd.2000.8266.
  10. Holmes RP, Knight J, Assimos DG. Lowering urinary oxalate excretion to decrease calcium oxalate stone disease. Urolithiasis 44: 27–32, 2016. doi: 10.1007/s00240-015-0839-4.
  11. Hutchison AJ, Wilkie M. Use of magnesium as a drug in chronic kidney disease. Clin Kidney J 5: i62–i70, 2012. doi: 10.1093/ndtplus/sfr168.
  12. Kanbara A, Hakoda M, Seyama I. Urine alkalization facilitates uric acid excretion. Nutr J 9: 45, 2010. doi: 10.1186/1475-2891-9-45.
  13. Kocyigit I, Yilmaz MI, Orscelik O, Sipahioglu MH, Unal A, Eroglu E, Kalay N, Tokgoz B, Axelsson J, Oymak O. Serum uric acid levels and endothelial dysfunction in patients with autosomal dominant polycystic kidney disease. Nephron Clin Pr 123: 157–64, 2013. doi: 10.1159/000353730.
  14. Kramers BJ, Koorevaar IW, Drenth JPH, Fijter JW de, Neto AG, Peters DJM, Vart P, Wetzels JF, Zietse R, Gansevoort RT, Meijer E. Salt, but not protein intake, is associated with accelerated disease progression in autosomal dominant polycystic kidney disease. Kidney Int 98: 989–998, 2020. doi: 10.1016/j.kint.2020.04.053.
  15. Lemann J, Pleuss JA, Worcester EM, Hornick L, Schrab D, Hoffmann RG. Urinary oxalate excretion increases with body size and decreases with increasing dietary calcium intake among healthy adults. Kidney Int 49: 200–8, 1996. doi: 10.1038/ki.1996.27.
  16. Levine E, Grantham JJ. Calcified renal stones and cyst calcifications in autosomal dominant polycystic kidney disease: clinical and CT study in 84 patients. AJR Am J Roentgenol 159: 77–81, 1992. doi: 10.2214/ajr.159.1.1609726.
  17. Lucaya J, Enriquez G, Nieto J, Callis L, Garcia Peña P, Dominguez C. Renal calcifications in patients with autosomal recessive polycystic kidney disease: prevalence and cause. Am J Roentgenol 160: 359–362, 1993. doi: 10.2214/ajr.160.2.8424350.
  18. Lumlertgul N, Siribamrungwong M, Jaber BL, Susantitaphong P. Secondary Oxalate Nephropathy: A Systematic Review. Kidney Int Rep 3: 1363–1372, 2018. doi: 10.1016/j.ekir.2018.07.020.
  19. Magistroni R, Boletta A. Defective glycolysis and the use of 2-deoxy-D-glucose in polycystic kidney disease: from animal models to humans. J Nephrol 30: 511–519, 2017. doi: 10.1007/s40620-017-0395-9.
  20. Markowitz GS, Perazella MA. Acute phosphate nephropathy. Kidney Int 76: 1027–1034, 2009. doi: 10.1038/ki.2009.308.
  21. Mejias E, Navas J, Lluberes R, Martinez-Maldonado M. Hyperuricemia, gout, and autosomal dominant polycystic kidney disease. Am J Med Sci 297: 145–8, 1989.
  22. Mulay SR, Evan A, Anders H-J. Molecular mechanisms of crystal-related kidney inflammation and injury. Implications for cholesterol embolism, crystalline nephropathies and kidney stone disease. Nephrol Dial Transplant Off Publ Eur Dial Transpl Assoc – Eur Ren Assoc 29: 507–514, 2014. doi: 10.1093/ndt/gft248.
  23. Nishiura JL, Neves RF, Eloi SR, Cintra SM, Ajzen SA, Heilberg IP. Evaluation of nephrolithiasis in autosomal dominant polycystic kidney disease patients. Clin J Am Soc Nephrol 4: 838–44, 2009. doi: 10.2215/CJN.03100608.
  24. Nowak KL, Hopp K. Metabolic Reprogramming in Autosomal Dominant Polycystic Kidney Disease: Evidence and Therapeutic Potential. Clin J Am Soc Nephrol 15(4): 577–584, 2020. doi: 10.2215/CJN.13291019.
  25. Nowak KL, Hopp K. Metabolic Reprogramming in Autosomal Dominant Polycystic Kidney Disease: Evidence and Therapeutic Potential. Clin J Am Soc Nephrol 15: 577–584, 2020. doi: 10.2215/CJN.13291019.
  26. Padovano V, Kuo IY, Stavola LK, Aerni HR, Flaherty BJ, Chapin HC, Ma M, Somlo S, Boletta A, Ehrlich BE, Rinehart J, Caplan MJ. The polycystins are modulated by cellular oxygen-sensing pathways and regulate mitochondrial function. Mol Biol Cell 28: 261–269, 2017. doi: 10.1091/mbc.E16-08-0597.
  27. Padovano V, Podrini C, Boletta A, Caplan MJ. Metabolism and mitochondria in polycystic kidney disease research and therapy. Nat Rev Nephrol 14(11): 678–687, 2018. doi: 10.1038/s41581-018-0051-1.
  28. Pak CYC, Adams BV. Potassium Citrate Therapy of Nephrolithiasis. In: Renal Stone Disease: Pathogenesis, Prevention, and Treatment, edited by Pak CYC. Springer US, p. 201–224.
  29. Panizo N, Goicoechea M, Garcia de Vinuesa S, Arroyo D, Yuste C, Rincon A, Verdalles U, Ruiz-Caro C, Quiroga B, Luno J. Chronic kidney disease progression in patients with autosomal dominant polycystic kidney disease. Nefrologia 32: 197–205, 2012. doi: 10.3265/Nefrologia.pre2011.Dec.11177.
  30. Pearle MS, Goldfarb DS, Assimos DG, Curhan G, Denu-Ciocca CJ, Matlaga BR, Monga M, Penniston KL, Preminger GM, Turk TM, White JR, American Urological A. Medical management of kidney stones: AUA guideline. J Urol 192: 316–24, 2014. doi: 10.1016/j.juro.2014.05.006.
  31. Pérez FP. Uric Acid Renal Lithiasis: New Concepts. Uric Acid Chronic Kidney Dis 192: 116–124, 2018. doi: 10.1159/000484286.
  32. 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. Dissection of metabolic reprogramming in polycystic kidney disease reveals coordinated rewiring of bioenergetic pathways. Commun Biol 1: 194, 2018. doi: 10.1038/s42003-018-0200-x.
  33. Riwanto M, Kapoor S, Rodriguez D, Edenhofer I, Segerer S, Wuthrich RP. Inhibition of Aerobic Glycolysis Attenuates Disease Progression in Polycystic Kidney Disease. PLoS One 11: e0146654, 2016. doi: 10.1371/journal.pone.0146654.
  34. Rowe I, Boletta A. Defective metabolism in polycystic kidney disease: potential for therapy and open questions. Nephrol Dial Transpl 29: 1480–6, 2014. doi: 10.1093/ndt/gft521.
  35. 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. Nat Med 19: 488–93, 2013. doi: 10.1038/nm.3092.
  36. Sas KM, Yin H, Fitzgibbon WR, Baicu CF, Zile MR, Steele SL, Amria M, Saigusa T, Funk J, Bunni MA, Siegal GP, Siroky BJ, Bissler JJ, Bell PD. Hyperglycemia in the absence of cilia accelerates cystogenesis and induces renal damage. Am J Physiol Ren Physiol 309: F79-87, 2015. doi: 10.1152/ajprenal.00652.2014.
  37. Tanner GA. Potassium citrate/citric acid intake improves renal function in rats with polycystic kidney disease. J Am Soc Nephrol 9: 1242–8, 1998.
  38. Tanner GA, Tanner JA. Citrate therapy for polycystic kidney disease in rats. Kidney Int 58: 1859–69., 2000.
  39. Tanner GA, Tanner JA. Dietary citrate treatment of polycystic kidney disease in rats. Nephron Physiol 93: P14-20, 2003.
  40. Tanner JA, Tanner GA. Dietary potassium citrate does not harm the pcy mouse. Exp Biol Med Maywood 230: 57–60, 2005.
  41. Toblli JE, DeRosa G, Lago N, Angerosa M, Nyberg C, Pagano P. Potassium citrate administration ameliorates tubulointerstitial lesions in rats with uric acid nephropathy. Clin Nephrol 55: 59–68, 2001.
  42. Torres JA, Kruger SL, Broderick C, Amarlkhagva T, Agrawal S, Dodam JR, Mrug M, Lyons LA, Weimbs T. Ketosis Ameliorates Renal Cyst Growth in Polycystic Kidney Disease. Cell Metab 30: 1007-1023.e5, 2019. doi: 10.1016/j.cmet.2019.09.012.
  43. Torres JA, Kruger SL, Broderick C, Amarlkhagva T, Agrawal S, Dodam JR, Mrug M, Lyons LA, Weimbs T. Ketosis Ameliorates Renal Cyst Growth in Polycystic Kidney Disease.
  44. Torres JA, Rezaei M, Broderick C, Lin L, Wang X, Hoppe B, Cowley BD, Savica V, Torres VE, Khan S, Holmes RP, Mrug M, Weimbs T. Crystal deposition triggers tubule dilation that accelerates cystogenesis in polycystic kidney disease. J Clin Invest 129: 4506–4522, 2019. doi: 10.1172/JCI128503.
  45. Torres VE, Erickson SB, Smith LH, Wilson DM, Hattery RR, Segura JW. The association of nephrolithiasis and autosomal dominant polycystic kidney disease. Am J Kidney Dis Off J Natl Kidney Found 11: 318–325, 1988.
  46. Torres VE, Wilson DM, Hattery RR, Segura JW. Renal stone disease in autosomal dominant polycystic kidney disease. Am J Kidney Dis 22: 513–9, 1993. doi: 10.1016/S0272-6386(12)80922-X. 48. von Unruh GE, Voss S, Sauerbruch T, Hesse A. Dependence of oxalate absorption on the daily calcium intake. J Am Soc Nephrol 15: 1567–73, 2004. doi: 10.1097/01.asn.0000127864.26968.7f. 49. Wei K-Y, Gritter M, Vogt L, de Borst MH, Rotmans JI, Hoorn EJ. Dietary potassium and the kidney: lifesaving physiology. Clin Kidney J 13: 952–968, 2020. doi: 10.1093/ckj/sfaa157.
  47. Yurista SR, Chong C-R, Badimon JJ, Kelly DP, de Boer RA, Westenbrink BD. Therapeutic Potential of Ketone Bodies for Patients With Cardiovascular Disease: JACC Focus Seminar.
  48. Zuckerman JM, Assimos DG. Hypocitraturia: Pathophysiology and Medical Management. Rev Urol 11: 134–144, 2009. doi: 10.3909/riu0424].