{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,3]],"date-time":"2026-09-03T10:33:20Z","timestamp":1788431600781,"version":"build-2803163510"},"reference-count":140,"publisher":"MDPI AG","issue":"17","license":[{"start":{"date-parts":[[2020,8,24]],"date-time":"2020-08-24T00:00:00Z","timestamp":1598227200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100002347","name":"Bundesministerium f\u00fcr Bildung und Forschung","doi-asserted-by":"publisher","award":["01GM1515 and 01GM1903"],"award-info":[{"award-number":["01GM1515 and 01GM1903"]}],"id":[{"id":"10.13039\/501100002347","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["IJMS"],"abstract":"<jats:p>Autosomal recessive and autosomal dominant polycystic kidney disease (ARPKD, ADPKD) are systemic disorders with pronounced hepatorenal phenotypes. While the main underlying genetic causes of both ARPKD and ADPKD have been well-known for years, the exact molecular mechanisms resulting in the observed clinical phenotypes in the different organs, remain incompletely understood. Recent research has identified cellular metabolic changes in PKD. These findings are of major relevance as there may be an immediate translation into clinical trials and potentially clinical practice. Here, we review important results in the field regarding metabolic changes in PKD and their modulation as a potential target of systemic treatment.<\/jats:p>","DOI":"10.3390\/ijms21176093","type":"journal-article","created":{"date-parts":[[2020,8,24]],"date-time":"2020-08-24T10:37:32Z","timestamp":1598265452000},"page":"6093","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":23,"title":["Metabolic Changes in Polycystic Kidney Disease as a Potential Target for Systemic Treatment"],"prefix":"10.3390","volume":"21","author":[{"given":"Sophie","family":"Haumann","sequence":"first","affiliation":[{"name":"Department of Pediatrics, University of Cologne, Faculty of Medicine and University Hospital Cologne, 50937 Cologne, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6910-0745","authenticated-orcid":false,"given":"Roman-Ulrich","family":"M\u00fcller","sequence":"additional","affiliation":[{"name":"Department II of Internal Medicine, University of Cologne, Faculty of Medicine and University Hospital Cologne, 50937 Cologne, Germany"},{"name":"CECAD, University of Cologne, Faculty of Medicine and University Hospital Cologne, 50931 Cologne, Germany"},{"name":"Systems Biology of Ageing Cologne, University of Cologne, 50931 Cologne, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0494-9080","authenticated-orcid":false,"given":"Max C.","family":"Liebau","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, University of Cologne, Faculty of Medicine and University Hospital Cologne, 50937 Cologne, Germany"},{"name":"Center for Molecular Medicine, University of Cologne, Faculty of Medicine and University Hospital Cologne, 50931 Cologne, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2020,8,24]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"332","DOI":"10.1056\/NEJM199307293290508","article-title":"Autosomal Dominant Polycystic Kidney Disease","volume":"329","author":"Gabow","year":"1993","journal-title":"N. Engl. J. Med."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"17","DOI":"10.1111\/j.1365-2796.2006.01743.x","article-title":"Polycystic kidney disease: Genes, proteins, animal models, disease mechanisms and therapeutic opportunities","volume":"261","author":"Torres","year":"2007","journal-title":"J. Intern. Med."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"297","DOI":"10.1053\/j.ackd.2015.04.001","article-title":"Cystic Kidney Disease: A Primer","volume":"22","author":"Cramer","year":"2015","journal-title":"Adv. Chronic Kidney Dis."},{"key":"ref_4","doi-asserted-by":"crossref","unstructured":"De Rechter, S., Breysem, L., and Mekahli, D. (2017). Is Autosomal Dominant Polycystic Kidney Disease Becoming a Pediatric Disorder?. Front. Pediatr., 5.","DOI":"10.3389\/fped.2017.00272"},{"key":"ref_5","doi-asserted-by":"crossref","unstructured":"Cordido, A., Besada-Cerecedo, L., and Garc\u00eda-Gonz\u00e1lez, M.A. (2017). The Genetic and Cellular Basis of Autosomal Dominant Polycystic Kidney Disease\u2014A Primer for Clinicians. Front. Pediatr., 5.","DOI":"10.3389\/fped.2017.00279"},{"key":"ref_6","doi-asserted-by":"crossref","unstructured":"Bergmann, C. (2018). Genetics of Autosomal Recessive Polycystic Kidney Disease and Its Differential Diagnoses. Front. Pediatr., 5.","DOI":"10.3389\/fped.2017.00221"},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"50","DOI":"10.1038\/s41572-018-0047-y","article-title":"Polycystic kidney disease","volume":"4","author":"Bergmann","year":"2018","journal-title":"Nat. Rev. Dis. Prim."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"1765","DOI":"10.2215\/CJN.03960318","article-title":"Recent Advances in the Management of Autosomal Dominant Polycystic Kidney Disease","volume":"13","author":"Chebib","year":"2018","journal-title":"Clin. J. Am. Soc. Nephrol."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"2407","DOI":"10.1056\/NEJMoa1205511","article-title":"Tolvaptan in Patients with Autosomal Dominant Polycystic Kidney Disease","volume":"367","author":"Torres","year":"2012","journal-title":"N. Engl. J. Med."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"1930","DOI":"10.1056\/NEJMoa1710030","article-title":"Tolvaptan in Later-Stage Autosomal Dominant Polycystic Kidney Disease","volume":"377","author":"Torres","year":"2017","journal-title":"N. Engl. J. Med."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"1140","DOI":"10.1681\/ASN.2013101037","article-title":"Vasopressin-2 Receptor Signaling and Autosomal Dominant Polycystic Kidney Disease: From Bench to Bedside and Back Again","volume":"25","author":"Rinschen","year":"2014","journal-title":"J. Am. Soc. Nephrol."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"69","DOI":"10.1259\/imaging\/21023865","article-title":"Cystic diseases of the kidney in children","volume":"17","author":"Chakraborty","year":"2005","journal-title":"Imaging"},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"186","DOI":"10.1097\/MOP.0000000000000196","article-title":"Clinical manifestations of autosomal recessive polycystic kidney disease","volume":"27","author":"Hoyer","year":"2015","journal-title":"Curr. Opin. Pediatr."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"820","DOI":"10.1111\/petr.12593","article-title":"Review of combined liver and kidney transplantation in children","volume":"19","author":"Ganschow","year":"2015","journal-title":"Pediatr. Transplant."},{"key":"ref_15","doi-asserted-by":"crossref","unstructured":"Ebner, K., Feldkoetter, M., Ariceta, G., Bergmann, C., Buettner, R., Doyon, A., Duzova, A., G\u00f6bel, H., Haffner, D., and Hero, B. (2015). Rationale, design and objectives of ARegPKD, a European ARPKD registry study. BMC Nephrol., 16.","DOI":"10.1186\/s12882-015-0002-z"},{"key":"ref_16","doi-asserted-by":"crossref","unstructured":"Ebner, K., Schaefer, F., Liebau, M.C., The ARegPKD Consortium, Eid, L.A., Ranguelov, N., Adams, B., Van Hoeck, K., Raes, A., and Mekahli, D. (2017). Recent Progress of the ARegPKD Registry Study on Autosomal Recessive Polycystic Kidney Disease. Front. Pediatr., 5.","DOI":"10.3389\/fped.2017.00018"},{"key":"ref_17","doi-asserted-by":"crossref","unstructured":"Alzarka, B., Morizono, H., Bollman, J.W., Kim, D., and Guay-Woodford, L.M. (2017). Design and Implementation of the Hepatorenal Fibrocystic Disease Core Center Clinical Database: A Centralized Resource for Characterizing Autosomal Recessive Polycystic Kidney Disease and Other Hepatorenal Fibrocystic Diseases. Front. Pediatr., 5.","DOI":"10.3389\/fped.2017.00080"},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"1025","DOI":"10.1038\/ng.3871","article-title":"Mutations in DZIP1L, which encodes a ciliary-transition-zone protein, cause autosomal recessive polycystic kidney disease","volume":"49","author":"Lu","year":"2017","journal-title":"Nat. Genet."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"21","DOI":"10.1083\/jcb.200910096","article-title":"The cytoplasmic tail of fibrocystin contains a ciliary targeting sequence","volume":"188","author":"Follit","year":"2010","journal-title":"J. Cell Boil."},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"942","DOI":"10.1093\/hmg\/ddm039","article-title":"Polyductin undergoes notch-like processing and regulated release from primary cilia","volume":"16","author":"Kaimori","year":"2007","journal-title":"Hum. Mol. Genet."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"34357","DOI":"10.1074\/jbc.M606740200","article-title":"Proteolytic Cleavage and Nuclear Translocation of Fibrocystin Is Regulated by Intracellular Ca2+ and Activation of Protein Kinase C","volume":"281","author":"Hiesberger","year":"2006","journal-title":"J. Boil. Chem."},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1038\/s41598-017-08284-4","article-title":"NEDD4-family E3 ligase dysfunction due to PKHD1\/Pkhd1 defects suggests a mechanistic model for ARPKD pathobiology","volume":"7","author":"Kaimori","year":"2017","journal-title":"Sci. Rep."},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"1130","DOI":"10.1016\/j.kint.2017.04.027","article-title":"A novel model of autosomal recessive polycystic kidney questions the role of the fibrocystin C-terminus in disease mechanism","volume":"92","author":"Outeda","year":"2017","journal-title":"Kidney Int."},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"1940","DOI":"10.1093\/hmg\/ddm141","article-title":"Genetic interaction studies link autosomal dominant and recessive polycystic kidney disease in a common pathway","volume":"16","author":"Menezes","year":"2007","journal-title":"Hum. Mol. Genet."},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"1113","DOI":"10.1002\/hep.20655","article-title":"A mouse model for cystic biliary dysgenesis in autosomal recessive polycystic kidney disease (ARPKD)","volume":"41","author":"Moser","year":"2005","journal-title":"Hepatology"},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"328","DOI":"10.1038\/sj.ki.5002294","article-title":"A mouse model of autosomal recessive polycystic kidney disease with biliary duct and proximal tubule dilatation","volume":"72","author":"Woollard","year":"2007","journal-title":"Kidney Int."},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"792","DOI":"10.1053\/j.ajkd.2015.07.037","article-title":"Autosomal Dominant Polycystic Kidney Disease: Core Curriculum 2016","volume":"67","author":"Chebib","year":"2015","journal-title":"Am. J. Kidney Dis."},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"471","DOI":"10.3949\/ccjm.84a.16107","article-title":"Autosomal dominant polycystic kidney disease and the heart and brain","volume":"84","author":"Krishnappa","year":"2017","journal-title":"Clevel. Clin. J. Med."},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"2047","DOI":"10.1681\/ASN.2010101080","article-title":"Mutations in multiple PKD genes may explain early and severe polycystic kidney disease","volume":"22","author":"Bergmann","year":"2011","journal-title":"J. Am. Soc. Nephrol."},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"197","DOI":"10.1038\/nrneph.2010.18","article-title":"Molecular diagnostics for autosomal dominant polycystic kidney disease","volume":"6","author":"Harris","year":"2010","journal-title":"Nat. Rev. Nephrol."},{"key":"ref_31","doi-asserted-by":"crossref","first-page":"868","DOI":"10.1681\/ASN.2013040417","article-title":"The Mutation, a Key Determinant of Phenotype in ADPKD","volume":"24","author":"Harris","year":"2013","journal-title":"J. Am. Soc. Nephrol."},{"key":"ref_32","doi-asserted-by":"crossref","first-page":"1006","DOI":"10.1681\/ASN.2012070650","article-title":"Type of PKD1 Mutation Influences Renal Outcome in ADPKD","volume":"24","author":"Gall","year":"2013","journal-title":"J. Am. Soc. Nephrol."},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"1193","DOI":"10.1016\/j.ajhg.2016.05.004","article-title":"Mutations in GANAB, Encoding the Glucosidase II\u03b1 Subunit, Cause Autosomal-Dominant Polycystic Kidney and Liver Disease","volume":"98","author":"Porath","year":"2016","journal-title":"Am. J. Hum. Genet."},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"13","DOI":"10.1681\/ASN.2017050483","article-title":"Genetic Complexity of Autosomal Dominant Polycystic Kidney and Liver Diseases","volume":"29","author":"Gall","year":"2017","journal-title":"J. Am. Soc. Nephrol."},{"key":"ref_35","doi-asserted-by":"crossref","first-page":"160","DOI":"10.1681\/ASN.2013101138","article-title":"Imaging Classification of Autosomal Dominant Polycystic Kidney Disease: A Simple Model for Selecting Patients for Clinical Trials","volume":"26","author":"Irazabal","year":"2014","journal-title":"J. Am. Soc. Nephrol."},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"942","DOI":"10.1681\/ASN.2015010016","article-title":"The PROPKD Score: A New Algorithm to Predict Renal Survival in Autosomal Dominant Polycystic Kidney Disease","volume":"27","author":"Gall","year":"2015","journal-title":"J. Am. Soc. Nephrol."},{"key":"ref_37","doi-asserted-by":"crossref","first-page":"1310","DOI":"10.1093\/ndt\/gfx262","article-title":"Targeting new cellular disease pathways in autosomal dominant polycystic kidney disease","volume":"33","author":"Chang","year":"2017","journal-title":"Nephrol. Dial. Transplant."},{"key":"ref_38","doi-asserted-by":"crossref","first-page":"3530","DOI":"10.1681\/ASN.2016040425","article-title":"Variable Cyst Development in Autosomal Dominant Polycystic Kidney Disease: The Biologic Context","volume":"27","author":"Leonhard","year":"2016","journal-title":"J. Am. Soc. Nephrol."},{"key":"ref_39","doi-asserted-by":"crossref","first-page":"699","DOI":"10.1038\/ki.2015.207","article-title":"A polycystin-centric view of cyst formation and disease: The polycystins revisited","volume":"88","author":"Ong","year":"2015","journal-title":"Kidney Int."},{"key":"ref_40","doi-asserted-by":"crossref","first-page":"2113","DOI":"10.1681\/ASN.2019020150","article-title":"Synergistic Genetic Interactions between Pkhd1 and Pkd1 Result in an ARPKD-Like Phenotype in Murine Models","volume":"30","author":"Olson","year":"2019","journal-title":"J. Am. Soc. Nephrol."},{"key":"ref_41","doi-asserted-by":"crossref","first-page":"31559","DOI":"10.1074\/jbc.M805452200","article-title":"Polycystin-2 Expression Is Regulated by a PC2-binding Domain in the Intracellular Portion of Fibrocystin","volume":"283","author":"Kim","year":"2008","journal-title":"J. Boil. Chem."},{"key":"ref_42","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1038\/s41598-020-58087-3","article-title":"Analysis of the polycystin complex (PCC) in human urinary exosome\u2013like vesicles (ELVs)","volume":"10","author":"Lea","year":"2020","journal-title":"Sci. Rep."},{"key":"ref_43","doi-asserted-by":"crossref","first-page":"979","DOI":"10.1016\/S0092-8674(00)81793-6","article-title":"The Molecular Basis of Focal Cyst Formation in Human Autosomal Dominant Polycystic Kidney Disease Type I","volume":"87","author":"Qian","year":"1996","journal-title":"Cell"},{"key":"ref_44","doi-asserted-by":"crossref","first-page":"247","DOI":"10.1016\/S1097-2765(00)80135-5","article-title":"Somatic mutation in individual liver cysts supports a two-hit model of cystogenesis in autosomal dominant polycystic kidney disease","volume":"2","author":"Watnick","year":"1998","journal-title":"Mol. Cell"},{"key":"ref_45","doi-asserted-by":"crossref","first-page":"1524","DOI":"10.1681\/ASN.V1071524","article-title":"Somatic PKD2 mutations in individual kidney and liver cysts support a \u201ctwo-hit\u201d model of cystogenesis in type 2 autosomal dominant polycystic kidney disease","volume":"10","author":"Pei","year":"1999","journal-title":"J. Am. Soc. Nephrol."},{"key":"ref_46","doi-asserted-by":"crossref","first-page":"793","DOI":"10.1681\/ASN.2011030284","article-title":"Third-hit signaling in renal cyst formation","volume":"22","author":"Weimbs","year":"2011","journal-title":"J. Am. Soc. Nephrol."},{"key":"ref_47","doi-asserted-by":"crossref","first-page":"1983","DOI":"10.1046\/j.1523-1755.2003.00023.x","article-title":"Cyclic AMP activates B-Raf and ERK in cyst epithelial cells from autosomal-dominant polycystic kidneys","volume":"63","author":"Yamaguchi","year":"2003","journal-title":"Kidney Int."},{"key":"ref_48","doi-asserted-by":"crossref","unstructured":"Zhou, J.X., Fan, L.X., Li, X., Calvet, J.P., and Li, X. (2015). TNF\u03b1 Signaling Regulates Cystic Epithelial Cell Proliferation through Akt\/mTOR and ERK\/MAPK\/Cdk2 Mediated Id2 Signaling. PLOS ONE, 10.","DOI":"10.1371\/journal.pone.0131043"},{"key":"ref_49","doi-asserted-by":"crossref","first-page":"515","DOI":"10.1038\/nrneph.2015.46","article-title":"The hallmarks of cancer: Relevance to the pathogenesis of polycystic kidney disease","volume":"11","author":"Geynisman","year":"2015","journal-title":"Nat. Rev. Nephrol."},{"key":"ref_50","doi-asserted-by":"crossref","first-page":"598","DOI":"10.1093\/ndt\/gfi181","article-title":"Inhibition of mTOR with sirolimus slows disease progression in Han:SPRD rats with autosomal dominant polycystic kidney disease (ADPKD)","volume":"21","author":"Wahl","year":"2005","journal-title":"Nephrol. Dial. Transplant."},{"key":"ref_51","doi-asserted-by":"crossref","first-page":"5466","DOI":"10.1073\/pnas.0509694103","article-title":"The mTOR pathway is regulated by polycystin-1, and its inhibition reverses renal cystogenesis in polycystic kidney disease","volume":"103","author":"Shillingford","year":"2006","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_52","doi-asserted-by":"crossref","first-page":"F1","DOI":"10.1152\/ajprenal.00014.2012","article-title":"Mammalian target of rapamycin and the kidney. I. The signaling pathway","volume":"303","author":"Lieberthal","year":"2012","journal-title":"Am. J. Physiol. Physiol."},{"key":"ref_53","doi-asserted-by":"crossref","first-page":"1167","DOI":"10.1007\/s00109-013-1057-6","article-title":"mTOR and regulation of energy homeostasis in humans","volume":"91","author":"Mannaa","year":"2013","journal-title":"J. Mol. Med."},{"key":"ref_54","doi-asserted-by":"crossref","first-page":"2493","DOI":"10.1681\/ASN.2008111186","article-title":"The Role of the Mammalian Target Of Rapamycin (mTOR) in Renal Disease","volume":"20","author":"Lieberthal","year":"2009","journal-title":"J. Am. Soc. Nephrol."},{"key":"ref_55","doi-asserted-by":"crossref","unstructured":"Rabanal-Ruiz, Y., and Korolchuk, V.I. (2018). mTORC1 and Nutrient Homeostasis: The Central Role of the Lysosome. Int. J. Mol. Sci., 19.","DOI":"10.3390\/ijms19030818"},{"key":"ref_56","doi-asserted-by":"crossref","first-page":"502","DOI":"10.1038\/ki.2010.457","article-title":"mTOR and rapamycin in the kidney: Signaling and therapeutic implications beyond immunosuppression","volume":"79","author":"Huber","year":"2011","journal-title":"Kidney Int."},{"key":"ref_57","doi-asserted-by":"crossref","unstructured":"Schmeisser, K., and Parker, J.A. (2019). Pleiotropic Effects of mTOR and Autophagy During Development and Aging. Front. Cell Dev. Boil., 7.","DOI":"10.3389\/fcell.2019.00192"},{"key":"ref_58","doi-asserted-by":"crossref","first-page":"830","DOI":"10.1056\/NEJMoa1003491","article-title":"Everolimus in Patients with Autosomal Dominant Polycystic Kidney Disease","volume":"363","author":"Walz","year":"2010","journal-title":"N. Engl. J. Med."},{"key":"ref_59","doi-asserted-by":"crossref","first-page":"2122","DOI":"10.1056\/NEJMoa054341","article-title":"Volume Progression in Polycystic Kidney Disease","volume":"354","author":"Grantham","year":"2006","journal-title":"N. Engl. J. Med."},{"key":"ref_60","doi-asserted-by":"crossref","first-page":"333","DOI":"10.1159\/000355375","article-title":"Novel approach to estimate kidney and cyst volumes using mid-slice magnetic resonance images in polycystic kidney disease","volume":"38","author":"Bae","year":"2013","journal-title":"Am. J. Nephrol."},{"key":"ref_61","doi-asserted-by":"crossref","first-page":"146","DOI":"10.1038\/ki.2015.71","article-title":"A comparison of ultrasound and magnetic resonance imaging shows that kidney length predicts chronic kidney disease in autosomal dominant polycystic kidney disease","volume":"88","author":"Bhutani","year":"2015","journal-title":"Kidney Int."},{"key":"ref_62","doi-asserted-by":"crossref","unstructured":"Tangri, N., Hougen, I., Alam, A., Perrone, R., McFarlane, P., and Pei, Y. (2017). Total Kidney Volume as a Biomarker of Disease Progression in Autosomal Dominant Polycystic Kidney Disease. Can. J. Kidney Heal. Dis., 4.","DOI":"10.1177\/2054358117693355"},{"key":"ref_63","doi-asserted-by":"crossref","unstructured":"Serra, A.L., Kistler, A.D., Poster, D., Struker, M., W\u00fcthrich, R.P., Weishaupt, D., and Tschirch, F. (2007). Clinical proof-of-concept trial to assess the therapeutic effect of sirolimus in patients with autosomal dominant polycystic kidney disease: SUISSE ADPKD study. BMC Nephrol., 8.","DOI":"10.1186\/1471-2369-8-13"},{"key":"ref_64","doi-asserted-by":"crossref","first-page":"820","DOI":"10.1056\/NEJMoa0907419","article-title":"Sirolimus and Kidney Growth in Autosomal Dominant Polycystic Kidney Disease","volume":"363","author":"Serra","year":"2010","journal-title":"N. Engl. J. Med."},{"key":"ref_65","doi-asserted-by":"crossref","first-page":"754","DOI":"10.1038\/ki.2010.250","article-title":"Sirolimus attenuates disease progression in an orthologous mouse model of human autosomal dominant polycystic kidney disease","volume":"78","author":"Zafar","year":"2010","journal-title":"Kidney Int."},{"key":"ref_66","doi-asserted-by":"crossref","first-page":"46","DOI":"10.1681\/ASN.2004080660","article-title":"Rapamycin Markedly Slows Disease Progression in a Rat Model of Polycystic Kidney Disease","volume":"16","author":"Tao","year":"2004","journal-title":"J. Am. Soc. Nephrol."},{"key":"ref_67","doi-asserted-by":"crossref","first-page":"477","DOI":"10.1538\/expanim.61.477","article-title":"Animal models for human polycystic kidney disease","volume":"61","author":"Nagao","year":"2012","journal-title":"Exp. Anim."},{"key":"ref_68","doi-asserted-by":"crossref","first-page":"842","DOI":"10.1681\/ASN.2011040340","article-title":"Dose-Dependent Effects of Sirolimus on mTOR Signaling and Polycystic Kidney Disease","volume":"23","author":"Novalic","year":"2012","journal-title":"J. Am. Soc. Nephrol."},{"key":"ref_69","first-page":"1701","article-title":"Therapeutic mTOR Inhibition in Autosomal Dominant Polycystic Kidney Disease: What Is the Appropriate Serum Level?","volume":"10","author":"Canaud","year":"2010","journal-title":"Arab. Archaeol. Epigr."},{"key":"ref_70","doi-asserted-by":"crossref","first-page":"242","DOI":"10.1093\/ndt\/gfs519","article-title":"mTOR inhibition in autosomal-dominant polycystic kidney disease (ADPKD): The question remains open","volume":"28","author":"Jardine","year":"2012","journal-title":"Nephrol. Dial. Transplant."},{"key":"ref_71","doi-asserted-by":"crossref","first-page":"1674","DOI":"10.1681\/ASN.2012040367","article-title":"Folate-Conjugated Rapamycin Slows Progression of Polycystic Kidney Disease","volume":"23","author":"Shillingford","year":"2012","journal-title":"J. Am. Soc. Nephrol."},{"key":"ref_72","doi-asserted-by":"crossref","first-page":"i9","DOI":"10.1093\/ndt\/gft491","article-title":"mTOR controls kidney epithelia in health and disease","volume":"29","author":"Grahammer","year":"2014","journal-title":"Nephrol. Dial. Transplant."},{"key":"ref_73","doi-asserted-by":"crossref","first-page":"2359","DOI":"10.1128\/MCB.01259-08","article-title":"Polycystin-1 Regulates Extracellular Signal-Regulated Kinase-Dependent Phosphorylation of Tuberin To Control Cell Size through mTOR and Its Downstream Effectors S6K and 4EBP1","volume":"29","author":"Distefano","year":"2009","journal-title":"Mol. Cell. Boil."},{"key":"ref_74","doi-asserted-by":"crossref","first-page":"10786","DOI":"10.1038\/ncomms10786","article-title":"mTORC1-mediated inhibition of polycystin-1 expression drives renal cyst formation in tuberous sclerosis complex","volume":"7","author":"Pema","year":"2016","journal-title":"Nat. Commun."},{"key":"ref_75","doi-asserted-by":"crossref","unstructured":"Wang, S., Wu, M., Yao, G., Zhang, J., and Zhou, J. (2014). The Cytoplasmic Tail of FPC Antagonizes the Full-Length Protein in the Regulation of mTOR Pathway. PLoS ONE, 9.","DOI":"10.1371\/journal.pone.0095630"},{"key":"ref_76","doi-asserted-by":"crossref","first-page":"1819","DOI":"10.1093\/ndt\/gfn744","article-title":"Activation of the AKT\/mTOR pathway in autosomal recessive polycystic kidney disease (ARPKD)","volume":"24","author":"Fischer","year":"2009","journal-title":"Nephrol. Dial. Transplant."},{"key":"ref_77","doi-asserted-by":"crossref","unstructured":"Ren, X.S., Sato, Y., Harada, K., Sasaki, M., Furubo, S., Song, J.Y., and Nakanuma, Y. (2014). Activation of the PI3K\/mTOR Pathway Is Involved in Cystic Proliferation of Cholangiocytes of the PCK Rat. PLoS ONE, 9.","DOI":"10.1371\/journal.pone.0087660"},{"key":"ref_78","first-page":"158","article-title":"Autophagy activators suppress cystogenesis in an autosomal dominant polycystic kidney disease model","volume":"26","author":"Zhu","year":"2016","journal-title":"Hum. Mol. Genet."},{"key":"ref_79","doi-asserted-by":"crossref","first-page":"1480","DOI":"10.1093\/ndt\/gft521","article-title":"Defective metabolism in polycystic kidney disease: Potential for therapy and open questions","volume":"29","author":"Rowe","year":"2014","journal-title":"Nephrol. Dial. Transplant."},{"key":"ref_80","doi-asserted-by":"crossref","first-page":"7","DOI":"10.1016\/j.bbcan.2017.01.002","article-title":"Cancer cell metabolism and mitochondria: Nutrient plasticity for TCA cycle fueling","volume":"1868","author":"Corbet","year":"2017","journal-title":"Biochim. et Biophys. Acta (BBA)-Rev. Cancer"},{"key":"ref_81","doi-asserted-by":"crossref","first-page":"309","DOI":"10.1126\/science.123.3191.309","article-title":"On the Origin of Cancer Cells","volume":"123","author":"Warburg","year":"1956","journal-title":"Science"},{"key":"ref_82","doi-asserted-by":"crossref","unstructured":"Burns, J.S., and Manda, G. (2017). Metabolic Pathways of the Warburg Effect in Health and Disease: Perspectives of Choice, Chain or Chance. Int. J. Mol. Sci., 18.","DOI":"10.3390\/ijms18122755"},{"key":"ref_83","doi-asserted-by":"crossref","first-page":"1499","DOI":"10.1042\/BST20160094","article-title":"The Warburg effect: 80 years on","volume":"44","author":"Potter","year":"2016","journal-title":"Biochem. Soc. Trans."},{"key":"ref_84","doi-asserted-by":"crossref","first-page":"1029","DOI":"10.1126\/science.1160809","article-title":"Understanding the Warburg Effect: The Metabolic Requirements of Cell Proliferation","volume":"324","author":"Heiden","year":"2009","journal-title":"Science"},{"key":"ref_85","doi-asserted-by":"crossref","first-page":"4633","DOI":"10.1038\/sj.onc.1209597","article-title":"Glycolysis inhibition for anticancer treatment","volume":"25","author":"Pelicano","year":"2006","journal-title":"Oncogene"},{"key":"ref_86","doi-asserted-by":"crossref","first-page":"646","DOI":"10.1016\/j.cell.2011.02.013","article-title":"Hallmarks of Cancer: The Next Generation","volume":"144","author":"Hanahan","year":"2011","journal-title":"Cell"},{"key":"ref_87","doi-asserted-by":"crossref","unstructured":"Scalise, M., Pochini, L., Galluccio, M., Console, L., and Indiveri, C. (2017). Glutamine Transport and Mitochondrial Metabolism in Cancer Cell Growth. Front. Oncol., 7.","DOI":"10.3389\/fonc.2017.00306"},{"key":"ref_88","doi-asserted-by":"crossref","first-page":"3430","DOI":"10.7150\/jca.21125","article-title":"The Glycolytic Switch in Tumors: How Many Players Are Involved?","volume":"8","author":"Yu","year":"2017","journal-title":"J. Cancer"},{"key":"ref_89","doi-asserted-by":"crossref","first-page":"488","DOI":"10.1038\/nm.3092","article-title":"Defective glucose metabolism in polycystic kidney disease identifies a new therapeutic strategy","volume":"19","author":"Rowe","year":"2013","journal-title":"Nat. Med."},{"key":"ref_90","doi-asserted-by":"crossref","first-page":"2328","DOI":"10.1093\/hmg\/ddp165","article-title":"Systems biology of autosomal dominant polycystic kidney disease (ADPKD): Computational identification of gene expression pathways and integrated regulatory networks","volume":"18","author":"Song","year":"2009","journal-title":"Hum. Mol. Genet."},{"key":"ref_91","doi-asserted-by":"crossref","first-page":"1958","DOI":"10.1681\/ASN.2015030231","article-title":"2-Deoxy-d-Glucose Ameliorates PKD Progression","volume":"27","author":"Chiaravalli","year":"2015","journal-title":"J. Am. Soc. Nephrol."},{"key":"ref_92","doi-asserted-by":"crossref","unstructured":"Riwanto, M., Kapoor, S., Rodriguez, D., Edenhofer, I., Segerer, S., and W\u00fcthrich, R.P. (2016). Inhibition of Aerobic Glycolysis Attenuates Disease Progression in Polycystic Kidney Disease. PLoS ONE, 11.","DOI":"10.1371\/journal.pone.0146654"},{"key":"ref_93","doi-asserted-by":"crossref","first-page":"374","DOI":"10.1016\/j.bbrc.2017.05.060","article-title":"The changes in glucose metabolism and cell proliferation in the kidneys of polycystic kidney disease mini-pig models","volume":"488","author":"Lian","year":"2017","journal-title":"Biochem. Biophys. Res. Commun."},{"key":"ref_94","doi-asserted-by":"crossref","first-page":"2743","DOI":"10.1038\/s41598-018-20856-6","article-title":"A cleavage product of Polycystin-1 is a mitochondrial matrix protein that affects mitochondria morphology and function when heterologously expressed","volume":"8","author":"Lin","year":"2018","journal-title":"Sci. Rep."},{"key":"ref_95","doi-asserted-by":"crossref","first-page":"e00337-17","DOI":"10.1128\/MCB.00337-17","article-title":"Mitochondrial Abnormality Facilitates Cyst Formation in Autosomal Dominant Polycystic Kidney Disease","volume":"37","author":"Ishimoto","year":"2017","journal-title":"Mol. Cell. Boil."},{"key":"ref_96","doi-asserted-by":"crossref","unstructured":"Podrini, C., Cassina, L., and Boletta, A. (2020). Metabolic reprogramming and the role of mitochondria in polycystic kidney disease. Cell. Signal., 67.","DOI":"10.1016\/j.cellsig.2019.109495"},{"key":"ref_97","doi-asserted-by":"crossref","first-page":"261","DOI":"10.1091\/mbc.e16-08-0597","article-title":"The polycystins are modulated by cellular oxygen-sensing pathways and regulate mitochondrial function","volume":"28","author":"Padovano","year":"2017","journal-title":"Mol. Boil. Cell"},{"key":"ref_98","doi-asserted-by":"crossref","first-page":"eaat7397","DOI":"10.1126\/scisignal.aat7397","article-title":"Polycystin 2 regulates mitochondrial Ca2+ signaling, bioenergetics, and dynamics through mitofusin 2","volume":"12","author":"Kuo","year":"2019","journal-title":"Sci. Signal."},{"key":"ref_99","doi-asserted-by":"crossref","first-page":"6493","DOI":"10.1096\/fj.201901739RR","article-title":"Increased mitochondrial fragmentation in polycystic kidney disease acts as a modifier of disease progression","volume":"34","author":"Cassina","year":"2020","journal-title":"FASEB J."},{"key":"ref_100","doi-asserted-by":"crossref","first-page":"19345","DOI":"10.1073\/pnas.0709747104","article-title":"Beyond aerobic glycolysis: Transformed cells can engage in glutamine metabolism that exceeds the requirement for protein and nucleotide synthesis","volume":"104","author":"DeBerardinis","year":"2007","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_101","doi-asserted-by":"crossref","first-page":"F492","DOI":"10.1152\/ajprenal.00238.2015","article-title":"The cpk model of recessive PKD shows glutamine dependence associated with the production of the oncometabolite 2-hydroxyglutarate","volume":"309","author":"Hwang","year":"2015","journal-title":"Am. J. Physiol. Physiol."},{"key":"ref_102","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1038\/s41467-018-03036-y","article-title":"Lkb1 deficiency confers glutamine dependency in polycystic kidney disease","volume":"9","author":"Flowers","year":"2018","journal-title":"Nat. Commun."},{"key":"ref_103","doi-asserted-by":"crossref","first-page":"194","DOI":"10.1038\/s42003-018-0200-x","article-title":"Dissection of metabolic reprogramming in polycystic kidney disease reveals coordinated rewiring of bioenergetic pathways","volume":"1","author":"Podrini","year":"2018","journal-title":"Commun. Boil."},{"key":"ref_104","doi-asserted-by":"crossref","first-page":"F1855","DOI":"10.1152\/ajprenal.00025.2018","article-title":"Arginine reprogramming in ADPKD results in arginine-dependent cystogenesis","volume":"315","author":"Trott","year":"2018","journal-title":"Am. J. Physiol. Physiol."},{"key":"ref_105","doi-asserted-by":"crossref","first-page":"821","DOI":"10.1146\/annurev.biochem.67.1.821","article-title":"THE AMP-ACTIVATED\/SNF1 PROTEIN KINASE SUBFAMILY: Metabolic Sensors of the Eukaryotic Cell?","volume":"67","author":"Hardie","year":"1998","journal-title":"Annu. Rev. Biochem."},{"key":"ref_106","doi-asserted-by":"crossref","first-page":"121","DOI":"10.1038\/nrm.2017.95","article-title":"AMPK: Guardian of metabolism and mitochondrial homeostasis","volume":"19","author":"Herzig","year":"2017","journal-title":"Nat. Rev. Mol. Cell Boil."},{"key":"ref_107","doi-asserted-by":"crossref","first-page":"147","DOI":"10.1016\/j.bcp.2018.01.049","article-title":"Promise and challenges for direct small molecule AMPK activators","volume":"153","author":"Olivier","year":"2018","journal-title":"Biochem. Pharmacol."},{"key":"ref_108","doi-asserted-by":"crossref","first-page":"e245","DOI":"10.1038\/emm.2016.81","article-title":"Regulation and function of AMPK in physiology and diseases","volume":"48","author":"Jeon","year":"2016","journal-title":"Exp. Mol. Med."},{"key":"ref_109","doi-asserted-by":"crossref","first-page":"321","DOI":"10.1515\/hsz-2017-0271","article-title":"Metformin-induced anticancer activities: Recent insights","volume":"399","author":"Safe","year":"2018","journal-title":"Boil. Chem."},{"key":"ref_110","doi-asserted-by":"crossref","first-page":"53","DOI":"10.1016\/j.lfs.2017.07.029","article-title":"Metformin: Insights into its anticancer potential with special reference to AMPK dependent and independent pathways","volume":"185","author":"Ikhlas","year":"2017","journal-title":"Life Sci."},{"key":"ref_111","doi-asserted-by":"crossref","first-page":"18","DOI":"10.7554\/eLife.02242","article-title":"Metformin inhibits mitochondrial complex I of cancer cells to reduce tumorigenesis","volume":"3","author":"Wheaton","year":"2014","journal-title":"eLife"},{"key":"ref_112","doi-asserted-by":"crossref","first-page":"171","DOI":"10.1111\/apha.12311","article-title":"Metformin induces lactate production in peripheral blood mononuclear cells and platelets through specific mitochondrial complex I inhibition","volume":"213","author":"Piel","year":"2014","journal-title":"Acta Physiol."},{"key":"ref_113","doi-asserted-by":"crossref","first-page":"2462","DOI":"10.1073\/pnas.1011498108","article-title":"Activating AMP-activated protein kinase (AMPK) slows renal cystogenesis","volume":"108","author":"Takiar","year":"2011","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_114","first-page":"1591","article-title":"Polycystin-1 but not polycystin-2 deficiency causes upregulation of the mTOR pathway and can be synergistically targeted with rapamycin and metformin","volume":"466","author":"Mekhali","year":"2013","journal-title":"Pfl\u00fcgers Arch. Eur. J. Physiol."},{"key":"ref_115","doi-asserted-by":"crossref","first-page":"436","DOI":"10.1016\/j.ebiom.2019.08.041","article-title":"Salsalate, but not metformin or canagliflozin, slows kidney cyst growth in an adult-onset mouse model of polycystic kidney disease","volume":"47","author":"Leonhard","year":"2019","journal-title":"EBioMedicine"},{"key":"ref_116","doi-asserted-by":"crossref","unstructured":"Menezes, L.F., Zhou, F., Patterson, A.D., Piontek, K.B., Krausz, K.W., Gonzalez, F.J., and Germino, G.G. (2012). Network Analysis of a Pkd1-Mouse Model of Autosomal Dominant Polycystic Kidney Disease Identifies HNF4\u03b1 as a Disease Modifier. PLoS Genet., 8.","DOI":"10.1371\/journal.pgen.1003053"},{"key":"ref_117","doi-asserted-by":"crossref","first-page":"27495","DOI":"10.1074\/jbc.M304112200","article-title":"AMP-activated Protein Kinase Regulates HNF4\u03b1 Transcriptional Activity by Inhibiting Dimer Formation and Decreasing Protein Stability","volume":"278","author":"Hong","year":"2003","journal-title":"J. Boil. Chem."},{"key":"ref_118","first-page":"1013","article-title":"cAMP-mediated regulation of HNF-4\u03b1 depends on the level of coactivator PGC-1\u03b1","volume":"1803","author":"Dankel","year":"2010","journal-title":"Biochim. et Biophys. Acta (BBA)-Bioenerg."},{"key":"ref_119","doi-asserted-by":"crossref","first-page":"285","DOI":"10.1042\/BJ20060659","article-title":"Hepatocyte nuclear factor-4\u03b1 contributes to carbohydrate-induced transcriptional activation of hepatic fatty acid synthase","volume":"399","author":"Adamson","year":"2006","journal-title":"Biochem. J."},{"key":"ref_120","doi-asserted-by":"crossref","first-page":"14683","DOI":"10.1074\/jbc.M512636200","article-title":"Partnership of PGC-1\u03b1 and HNF4\u03b1 in the Regulation of Lipoprotein Metabolism","volume":"281","author":"Rhee","year":"2006","journal-title":"J. Boil. Chem."},{"key":"ref_121","doi-asserted-by":"crossref","first-page":"960","DOI":"10.1016\/j.cell.2017.02.004","article-title":"mTOR Signaling in Growth, Metabolism, and Disease","volume":"168","author":"Saxton","year":"2017","journal-title":"Cell"},{"key":"ref_122","doi-asserted-by":"crossref","unstructured":"Simon, N., and Hertig, A. (2015). Alteration of Fatty Acid Oxidation in Tubular Epithelial Cells: From Acute Kidney Injury to Renal Fibrogenesis. Front. Med., 2.","DOI":"10.3389\/fmed.2015.00052"},{"key":"ref_123","doi-asserted-by":"crossref","first-page":"23","DOI":"10.1146\/annurev-physiol-021115-105045","article-title":"The Biochemistry and Physiology of Mitochondrial Fatty Acid \u03b2-Oxidation and Its Genetic Disorders","volume":"78","author":"Houten","year":"2016","journal-title":"Annu. Rev. Physiol."},{"key":"ref_124","doi-asserted-by":"crossref","first-page":"791","DOI":"10.1007\/s00467-016-3556-5","article-title":"Disorders of fatty acid oxidation and autosomal recessive polycystic kidney disease\u2014different clinical entities and comparable perinatal renal abnormalities","volume":"32","author":"Hackl","year":"2017","journal-title":"Pediatr. Nephrol."},{"key":"ref_125","doi-asserted-by":"crossref","first-page":"183","DOI":"10.1016\/j.ebiom.2016.01.027","article-title":"Fatty Acid Oxidation is Impaired in An Orthologous Mouse Model of Autosomal Dominant Polycystic Kidney Disease","volume":"5","author":"Menezes","year":"2016","journal-title":"EBioMedicine"},{"key":"ref_126","doi-asserted-by":"crossref","first-page":"F122","DOI":"10.1152\/ajprenal.00352.2017","article-title":"PPAR\u03b1 agonist fenofibrate enhances fatty acid \u03b2-oxidation and attenuates polycystic kidney and liver disease in mice","volume":"314","author":"Lakhia","year":"2018","journal-title":"Am. J. Physiol. Physiol."},{"key":"ref_127","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1155\/2012\/695898","article-title":"Global Gene Expression Profiling in PPAR-\u03b3 Agonist-Treated Kidneys in an Orthologous Rat Model of Human Autosomal Recessive Polycystic Kidney Disease","volume":"2012","author":"Yoshihara","year":"2012","journal-title":"PPAR Res."},{"key":"ref_128","doi-asserted-by":"crossref","first-page":"4445","DOI":"10.1038\/onc.2017.54","article-title":"P53\/Rb inhibition induces metastatic adrenocortical carcinomas in a preclinical transgenic model","volume":"36","author":"Tissier","year":"2017","journal-title":"Oncogene"},{"key":"ref_129","doi-asserted-by":"crossref","first-page":"3084","DOI":"10.1172\/JCI64401","article-title":"Sirtuin 1 inhibition delays cyst formation in autosomal-dominant polycystic kidney disease","volume":"123","author":"Zhou","year":"2013","journal-title":"J. Clin. Investig."},{"key":"ref_130","doi-asserted-by":"crossref","first-page":"51","DOI":"10.1093\/abbs\/gms108","article-title":"SIRT1 and energy metabolism","volume":"45","author":"Li","year":"2012","journal-title":"Acta Biochim. et Biophys. Sin."},{"key":"ref_131","doi-asserted-by":"crossref","first-page":"443","DOI":"10.1016\/j.cmet.2011.07.014","article-title":"Metabolic Flux and the Regulation of Mammalian Cell Growth","volume":"14","author":"Locasale","year":"2011","journal-title":"Cell Metab."},{"key":"ref_132","doi-asserted-by":"crossref","first-page":"1533","DOI":"10.1056\/NEJMra1010172","article-title":"Ciliopathies","volume":"364","author":"Hildebrandt","year":"2011","journal-title":"N. Engl. J. Med."},{"key":"ref_133","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1186\/s40348-015-0019-1","article-title":"Ciliopathies - from rare inherited cystic kidney diseases to basic cellular function","volume":"2","author":"Habbig","year":"2015","journal-title":"Mol. Cell. Pediatr."},{"key":"ref_134","first-page":"1115","article-title":"Primary cilia regulate mTORC1 activity and cell size through Lkb1","volume":"12","author":"Boehlke","year":"2010","journal-title":"Nature"},{"key":"ref_135","doi-asserted-by":"crossref","first-page":"1437","DOI":"10.1681\/ASN.2015020132","article-title":"Food Restriction Ameliorates the Development of Polycystic Kidney Disease","volume":"27","author":"Warner","year":"2015","journal-title":"J. Am. Soc. Nephrol."},{"key":"ref_136","doi-asserted-by":"crossref","first-page":"F726","DOI":"10.1152\/ajprenal.00551.2015","article-title":"A mild reduction of food intake slows disease progression in an orthologous mouse model of polycystic kidney disease","volume":"310","author":"Kipp","year":"2016","journal-title":"Am. J. Physiol. Physiol."},{"key":"ref_137","doi-asserted-by":"crossref","first-page":"298","DOI":"10.1038\/nrm1616","article-title":"Calorie restriction, SIRT1 and metabolism: Understanding longevity","volume":"6","author":"Bordone","year":"2005","journal-title":"Nat. Rev. Mol. Cell Boil."},{"key":"ref_138","doi-asserted-by":"crossref","first-page":"727","DOI":"10.1146\/annurev.biochem.77.061206.171059","article-title":"Aging and Survival: The Genetics of Life Span Extension by Dietary Restriction","volume":"77","author":"Mair","year":"2008","journal-title":"Annu. Rev. Biochem."},{"key":"ref_139","doi-asserted-by":"crossref","first-page":"571","DOI":"10.1681\/ASN.2017070819","article-title":"Overweight and Obesity Are Predictors of Progression in Early Autosomal Dominant Polycystic Kidney Disease","volume":"29","author":"Nowak","year":"2017","journal-title":"J. Am. Soc. Nephrol."},{"key":"ref_140","doi-asserted-by":"crossref","first-page":"1007","DOI":"10.1016\/j.cmet.2019.09.012","article-title":"Ketosis Ameliorates Renal Cyst Growth in Polycystic Kidney Disease","volume":"30","author":"Torres","year":"2019","journal-title":"Cell Metab."}],"container-title":["International Journal of Molecular Sciences"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/1422-0067\/21\/17\/6093\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,11]],"date-time":"2025-10-11T10:05:49Z","timestamp":1760177149000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/1422-0067\/21\/17\/6093"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2020,8,24]]},"references-count":140,"journal-issue":{"issue":"17","published-online":{"date-parts":[[2020,9]]}},"alternative-id":["ijms21176093"],"URL":"https:\/\/doi.org\/10.3390\/ijms21176093","relation":{},"ISSN":["1422-0067"],"issn-type":[{"value":"1422-0067","type":"electronic"}],"subject":[],"published":{"date-parts":[[2020,8,24]]}}}