{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,27]],"date-time":"2026-09-27T03:11:17Z","timestamp":1790478677617,"version":"4.1.0"},"reference-count":104,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2019,1,1]],"date-time":"2019-01-01T00:00:00Z","timestamp":1546300800000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"},{"start":{"date-parts":[[2019,1,23]],"date-time":"2019-01-23T00:00:00Z","timestamp":1548201600000},"content-version":"vor","delay-in-days":22,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["W81XWH-14-1-0343"],"award-info":[{"award-number":["W81XWH-14-1-0343"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["DK107535"],"award-info":[{"award-number":["DK107535"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["HL48459"],"award-info":[{"award-number":["HL48459"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["R21 12121"],"award-info":[{"award-number":["R21 12121"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["P30CA016056"],"award-info":[{"award-number":["P30CA016056"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Le Bonheur","award":["650700"],"award-info":[{"award-number":["650700"]}]},{"name":"Memphis Research Consortium"},{"name":"Veterans Administration","award":["I01 BX001000-06"],"award-info":[{"award-number":["I01 BX001000-06"]}]},{"name":"Center for Genetics of Transport and Epithelial Biology"},{"DOI":"10.13039\/100001320","name":"Roswell Park Cancer Institute","doi-asserted-by":"crossref","id":[{"id":"10.13039\/100001320","id-type":"DOI","asserted-by":"crossref"}]},{"name":"Agliata family in memory of Jacob"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Physiol Rep"],"published-print":{"date-parts":[[2019,1]]},"DOI":"10.14814\/phy2.13983","type":"journal-article","created":{"date-parts":[[2019,1,24]],"date-time":"2019-01-24T19:27:36Z","timestamp":1548358056000},"page":"e13983","source":"Crossref","is-referenced-by-count":32,"title":["Tuberous sclerosis complex exhibits a new renal cystogenic mechanism"],"prefix":"10.14814","volume":"7","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-9685-9183","authenticated-orcid":false,"given":"John J.","family":"Bissler","sequence":"first","affiliation":[{"name":"Department of Pediatrics; University of Tennessee Health Science Center and Le Bonheur Children's Hospital; Memphis Tennessee"},{"name":"St. Jude Children's Research Hospital; Memphis Tennessee"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fahad","family":"Zadjali","sequence":"additional","affiliation":[{"name":"Department of Clinical Biochemistry; College of Medicine & Health Sciences; Sultan Qaboos University; Muscat Oman"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dave","family":"Bridges","sequence":"additional","affiliation":[{"name":"Department of Nutritional Sciences; University of Michigan School of Public Health; Ann Arbor Michigan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Aristotelis","family":"Astrinidis","sequence":"additional","affiliation":[{"name":"Department of Pediatrics; University of Tennessee Health Science Center and Le Bonheur Children's Hospital; Memphis Tennessee"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sharon","family":"Barone","sequence":"additional","affiliation":[{"name":"Departments of Medicine; University of Cincinnati College of Medicine; Cincinnati Ohio"},{"name":"Center on Genetics of Transport; University of Cincinnati College of Medicine; Cincinnati Ohio"},{"name":"Research Services; Veterans Affairs Medical Center; Cincinnati Ohio"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ying","family":"Yao","sequence":"additional","affiliation":[{"name":"Department of Pediatrics; University of Tennessee Health Science Center and Le Bonheur Children's Hospital; Memphis Tennessee"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"JeAnna R.","family":"Redd","sequence":"additional","affiliation":[{"name":"Department of Nutritional Sciences; University of Michigan School of Public Health; Ann Arbor Michigan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Brian J.","family":"Siroky","sequence":"additional","affiliation":[{"name":"Department of Pediatrics; University of Cincinnati College of Medicine; Cincinnati Ohio"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yanqing","family":"Wang","sequence":"additional","affiliation":[{"name":"Department of Molecular and Cellular Biology; Roswell Park Cancer Institute; Buffalo New York"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Joel T.","family":"Finley","sequence":"additional","affiliation":[{"name":"Department of Pediatrics; University of Tennessee Health Science Center and Le Bonheur Children's Hospital; Memphis Tennessee"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael E.","family":"Rusiniak","sequence":"additional","affiliation":[{"name":"Department of Molecular and Cellular Biology; Roswell Park Cancer Institute; Buffalo New York"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Heinz","family":"Baumann","sequence":"additional","affiliation":[{"name":"Department of Molecular and Cellular Biology; Roswell Park Cancer Institute; Buffalo New York"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kamyar","family":"Zahedi","sequence":"additional","affiliation":[{"name":"Departments of Medicine; University of Cincinnati College of Medicine; Cincinnati Ohio"},{"name":"Center on Genetics of Transport; University of Cincinnati College of Medicine; Cincinnati Ohio"},{"name":"Research Services; Veterans Affairs Medical Center; Cincinnati Ohio"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kenneth W.","family":"Gross","sequence":"additional","affiliation":[{"name":"Department of Molecular and Cellular Biology; Roswell Park Cancer Institute; Buffalo New York"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Manoocher","family":"Soleimani","sequence":"additional","affiliation":[{"name":"Departments of Medicine; University of Cincinnati College of Medicine; Cincinnati Ohio"},{"name":"Center on Genetics of Transport; University of Cincinnati College of Medicine; Cincinnati Ohio"},{"name":"Research Services; Veterans Affairs Medical Center; Cincinnati Ohio"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2019,1,23]]},"reference":[{"key":"10.14814\/phy2.13983-BIB0001|phy213983-cit-0001","doi-asserted-by":"crossref","first-page":"F584","DOI":"10.1152\/ajprenal.00141.2012","article-title":"Cystogenesis and elongated primary cilia in Tsc1-deficient distal convoluted tubules","volume":"303","author":"Armour","year":"2012","journal-title":"Am. J. Physiol. Renal Physiol."},{"key":"10.14814\/phy2.13983-BIB0002|phy213983-cit-0002","doi-asserted-by":"crossref","first-page":"781","DOI":"10.1007\/s00234-009-0563-2","article-title":"Magnetic resonance imaging and diffusion-weighted imaging of normal-appearing white matter in children and young adults with tuberous sclerosis complex","volume":"51","author":"Arulrajah","year":"2009","journal-title":"Neuroradiology"},{"key":"10.14814\/phy2.13983-BIB0003|phy213983-cit-0003","doi-asserted-by":"crossref","first-page":"487","DOI":"10.1038\/sj.ejhg.5200484","article-title":"Loss of heterozygosity in renal and hepatic epithelial cystic cells from ADPKD1 patients","volume":"8","author":"Badenas","year":"2000","journal-title":"Eur. J. Hum. Genet."},{"key":"10.14814\/phy2.13983-BIB0004|phy213983-cit-0004","doi-asserted-by":"crossref","first-page":"849","DOI":"10.3389\/fphys.2018.00849","article-title":"Probenecid pre-treatment downregulates the kidney Cl(-)\/HCO3(-) exchanger (pendrin) and potentiates hydrochlorothiazide-induced diuresis","volume":"9","author":"Barone","year":"2018","journal-title":"Front. Physiol."},{"key":"10.14814\/phy2.13983-BIB0005|phy213983-cit-0005","doi-asserted-by":"crossref","first-page":"6306","DOI":"10.1038\/sj.onc.1204009","article-title":"The tuberous sclerosis-1 (TSC1) gene product hamartin suppresses cell growth and augments the expression of the TSC2 product tuberin by inhibiting its ubiquitination","volume":"19","author":"Benvenuto","year":"2000","journal-title":"Oncogene"},{"key":"10.14814\/phy2.13983-BIB0006|phy213983-cit-0006","doi-asserted-by":"crossref","first-page":"51","DOI":"10.1007\/978-1-4939-7784-0_3","volume-title":"Polycystic Kidney Disease: translating Mechanisms into Therapy","author":"Bissler","year":"2018"},{"key":"10.14814\/phy2.13983-BIB0007|phy213983-cit-0007","doi-asserted-by":"crossref","first-page":"279","DOI":"10.1177\/1756287216641353","article-title":"Optimal treatment of tuberous sclerosis complex associated renal angiomyolipomata: a systematic review","volume":"8","author":"Bissler","year":"2016","journal-title":"Ther. Adv. Urol."},{"key":"10.14814\/phy2.13983-BIB0008|phy213983-cit-0008","doi-asserted-by":"crossref","first-page":"338","DOI":"10.1002\/ajmg.c.31654","article-title":"Renal manifestation of tuberous sclerosis complex","volume":"178","author":"Bissler","year":"2018","journal-title":"Am. J. Med. Genet."},{"key":"10.14814\/phy2.13983-BIB0009|phy213983-cit-0009","doi-asserted-by":"crossref","first-page":"140","DOI":"10.1056\/NEJMoa063564","article-title":"Sirolimus for angiomyolipoma in tuberous sclerosis complex or lymphangioleiomyomatosis","volume":"358","author":"Bissler","year":"2008","journal-title":"N. Engl. J. Med."},{"key":"10.14814\/phy2.13983-BIB0010|phy213983-cit-0010","doi-asserted-by":"crossref","first-page":"817","DOI":"10.1016\/S0140-6736(12)61767-X","article-title":"Everolimus for angiomyolipoma associated with tuberous sclerosis complex or sporadic lymphangioleiomyomatosis (EXIST-2): a multicentre, randomised, double-blind, placebo-controlled trial","volume":"381","author":"Bissler","year":"2013","journal-title":"Lancet"},{"key":"10.14814\/phy2.13983-BIB0011|phy213983-cit-0011","doi-asserted-by":"crossref","first-page":"433","DOI":"10.1097\/MNH.0b013e32832e9c58","article-title":"Cellular physiology of the renal H+ATPase","volume":"18","author":"Blake-Palmer","year":"2009","journal-title":"Curr. Opin. Nephrol. Hypertens."},{"key":"10.14814\/phy2.13983-BIB0012|phy213983-cit-0012","doi-asserted-by":"crossref","first-page":"95516","DOI":"10.18632\/oncotarget.20764","article-title":"Subependymal giant cell astrocytomas in tuberous sclerosis complex have consistent TSC1\/TSC2 biallelic inactivation, and no BRAF mutations","volume":"8","author":"Bongaarts","year":"2017","journal-title":"Oncotarget"},{"key":"10.14814\/phy2.13983-BIB0013|phy213983-cit-0013","doi-asserted-by":"crossref","first-page":"972","DOI":"10.1016\/j.prp.2016.04.005","article-title":"Tuberous sclerosis complex: hamartin and tuberin expression in renal cysts and its discordant expression in renal neoplasms","volume":"212","author":"Bonsib","year":"2016","journal-title":"Pathol. Res. Pract."},{"key":"10.14814\/phy2.13983-BIB0014|phy213983-cit-0014","doi-asserted-by":"crossref","first-page":"194","DOI":"10.1172\/JCI119147","article-title":"Loss of the polycystic kidney disease (PKD1) region of chromosome 16p13 in renal cyst cells supports a loss-of-function model for cyst pathogenesis","volume":"99","author":"Brasier","year":"1997","journal-title":"J. Clin. Invest."},{"key":"10.14814\/phy2.13983-BIB0015|phy213983-cit-0015","first-page":"318","article-title":"Regulation of luminal acidification by the V-ATPase","volume":"28","author":"Breton","year":"2013","journal-title":"Physiology (Bethesda)"},{"key":"10.14814\/phy2.13983-BIB0016|phy213983-cit-0016","doi-asserted-by":"crossref","first-page":"328","DOI":"10.1038\/ng1294-328","article-title":"Deletion of the TSC2 and PKD1 genes associated with severe infantile polycystic kidney disease-a contiguous gene syndrome","volume":"8","author":"Brook-Carter","year":"1994","journal-title":"Nat. Genet."},{"key":"10.14814\/phy2.13983-BIB0017|phy213983-cit-0017","doi-asserted-by":"crossref","first-page":"1762","DOI":"10.1242\/jeb.028803","article-title":"Regulation of the V-ATPase in kidney epithelial cells: dual role in acid-base homeostasis and vesicle trafficking","volume":"212","author":"Brown","year":"2009","journal-title":"J. Exp. Biol."},{"key":"10.14814\/phy2.13983-BIB0018|phy213983-cit-0018","doi-asserted-by":"crossref","first-page":"457","DOI":"10.1016\/S0002-9440(10)63840-0","article-title":"Polycystic kidney disease as a result of loss of the tuberous sclerosis 2 tumor suppressor gene during development","volume":"162","author":"Cai","year":"2003","journal-title":"Am. J. Pathol."},{"key":"10.14814\/phy2.13983-BIB0019|phy213983-cit-0019","doi-asserted-by":"crossref","first-page":"R104","DOI":"10.1152\/ajpregu.00209.2013","article-title":"The (pro)renin receptor and body fluid homeostasis","volume":"305","author":"Cao","year":"2013","journal-title":"Am. J. Physiol. Regul. Integr. Comp. Physiol."},{"key":"10.14814\/phy2.13983-BIB0020|phy213983-cit-0020","unstructured":"Cell_Signaling_Technology 2018 https:\/\/www.cellsignal.com\/products\/primary-antibodies\/tuberin-tsc2-antibody\/3612. 2018"},{"key":"10.14814\/phy2.13983-BIB0021|phy213983-cit-0021","doi-asserted-by":"crossref","first-page":"534","DOI":"10.1681\/ASN.2013030225","article-title":"Activation of mTORC1 in collecting ducts causes hyperkalemia","volume":"25","author":"Chen","year":"2014","journal-title":"J. Am. Soc. Nephrol."},{"key":"10.14814\/phy2.13983-BIB0022|phy213983-cit-0022","doi-asserted-by":"crossref","first-page":"F693","DOI":"10.1152\/ajprenal.00605.2013","article-title":"H-K-ATPase type 2: relevance for renal physiology and beyond","volume":"306","author":"Crambert","year":"2014","journal-title":"Am. J. Physiol. Renal Physiol."},{"key":"10.14814\/phy2.13983-BIB0023|phy213983-cit-0023","doi-asserted-by":"crossref","first-page":"1716","DOI":"10.1212\/WNL.0b013e3181e04325","article-title":"Biallelic TSC gene inactivation in tuberous sclerosis complex","volume":"74","author":"Crino","year":"2010","journal-title":"Neurology"},{"key":"10.14814\/phy2.13983-BIB0024|phy213983-cit-0024","doi-asserted-by":"crossref","first-page":"64","DOI":"10.1086\/316951","article-title":"Mutational analysis in a cohort of 224 tuberous sclerosis patients indicates increased severity of TSC2, compared with TSC1, disease in multiple organs","volume":"68","author":"Dabora","year":"2001","journal-title":"Am. J. Hum. Genet."},{"key":"10.14814\/phy2.13983-BIB0025|phy213983-cit-0025","doi-asserted-by":"crossref","first-page":"1187","DOI":"10.1093\/ajh\/hpv045","article-title":"The role of the (Pro)renin receptor in hypertensive disease","volume":"28","author":"Danser","year":"2015","journal-title":"Am. J. Hypertens."},{"key":"10.14814\/phy2.13983-BIB0026|phy213983-cit-0026","doi-asserted-by":"crossref","first-page":"2239","DOI":"10.1038\/onc.2014.164","article-title":"mTORC1 drives HIF-1alpha and VEGF-A signalling via multiple mechanisms involving 4E-BP1, S6K1 and STAT3","volume":"34","author":"Dodd","year":"2015","journal-title":"Oncogene"},{"key":"10.14814\/phy2.13983-BIB0027|phy213983-cit-0027","doi-asserted-by":"crossref","first-page":"1262","DOI":"10.1038\/nbt.3026","article-title":"Rational design of highly active sgRNAs for CRISPR-Cas9-mediated gene inactivation","volume":"32","author":"Doench","year":"2014","journal-title":"Nat. Biotechnol."},{"key":"10.14814\/phy2.13983-BIB0028|phy213983-cit-0028","doi-asserted-by":"crossref","first-page":"184","DOI":"10.1038\/nbt.3437","article-title":"Optimized sgRNA design to maximize activity and minimize off-target effects of CRISPR-Cas9","volume":"34","author":"Doench","year":"2016","journal-title":"Nat. Biotechnol."},{"key":"10.14814\/phy2.13983-BIB0029|phy213983-cit-0029","doi-asserted-by":"crossref","first-page":"57","DOI":"10.1016\/j.semcdb.2014.08.003","article-title":"Kidney stem cells in development, regeneration and cancer","volume":"36","author":"Dziedzic","year":"2014","journal-title":"Semin. Cell Dev. Biol."},{"key":"10.14814\/phy2.13983-BIB0030|phy213983-cit-0030","doi-asserted-by":"crossref","first-page":"681","DOI":"10.1016\/j.molcel.2012.09.017","article-title":"HIF2alpha acts as an mTORC1 activator through the amino acid carrier SLC7A5","volume":"48","author":"Elorza","year":"2012","journal-title":"Mol. Cell"},{"key":"10.14814\/phy2.13983-BIB0031|phy213983-cit-0031","doi-asserted-by":"crossref","first-page":"231","DOI":"10.1016\/j.neurad.2009.12.006","article-title":"Cerebellar abnormality in children and young adults with tuberous sclerosis complex: MR and diffusion weighted imaging findings","volume":"37","author":"Ertan","year":"2010","journal-title":"J. Neuroradiol."},{"key":"10.14814\/phy2.13983-BIB0032|phy213983-cit-0032","doi-asserted-by":"crossref","first-page":"141","DOI":"10.1016\/S0022-5347(01)63072-6","article-title":"Renal lesion growth in children with tuberous sclerosis complex","volume":"160","author":"Ewalt","year":"1998","journal-title":"J. Urol."},{"key":"10.14814\/phy2.13983-BIB0033|phy213983-cit-0033","doi-asserted-by":"crossref","first-page":"193","DOI":"10.1007\/s002329900228","article-title":"Role of Cl- in electrogenic H+ secretion by cortical distal tubule","volume":"157","author":"Fernandez","year":"1997","journal-title":"J. Membr. Biol."},{"key":"10.14814\/phy2.13983-BIB0034|phy213983-cit-0034","first-page":"211","article-title":"Everolimus in the treatment of subependymal giant cell astrocytomas, angiomyolipomas, and pulmonary and skin lesions associated with tuberous sclerosis complex","volume":"7","author":"Franz","year":"2013","journal-title":"Biologics"},{"key":"10.14814\/phy2.13983-BIB0035|phy213983-cit-0035","doi-asserted-by":"crossref","first-page":"1796","DOI":"10.1111\/jdv.14964","article-title":"Effect of everolimus on skin lesions in patients treated for subependymal giant cell astrocytoma and renal angiomyolipoma: final 4-year results from the randomised EXIST-1 and EXIST-2 studies","volume":"10","author":"Franz","year":"2018","journal-title":"J. Eur. Acad. Dermatol. Venereol."},{"key":"10.14814\/phy2.13983-BIB0036|phy213983-cit-0036","doi-asserted-by":"crossref","first-page":"626","DOI":"10.1053\/j.ajkd.2011.06.004","article-title":"Pathophysiology of metabolic alkalosis: a new classification based on the centrality of stimulated collecting duct ion transport","volume":"58","author":"Gennari","year":"2011","journal-title":"Am. J. Kidney Dis."},{"key":"10.14814\/phy2.13983-BIB0037|phy213983-cit-0037","doi-asserted-by":"crossref","first-page":"243","DOI":"10.1152\/physiolgenomics.00017.2008","article-title":"In vivo analysis of key elements within the renin regulatory region","volume":"35","author":"Glenn","year":"2008","journal-title":"Physiol. Genomics"},{"key":"10.14814\/phy2.13983-BIB0038|phy213983-cit-0038","doi-asserted-by":"crossref","first-page":"1053","DOI":"10.1007\/s00467-014-2882-8","article-title":"Rationale for early treatment of polycystic kidney disease","volume":"30","author":"Grantham","year":"2015","journal-title":"Pediatr. Nephrol."},{"key":"10.14814\/phy2.13983-BIB0039|phy213983-cit-0039","doi-asserted-by":"crossref","first-page":"1145","DOI":"10.1038\/ki.1987.121","article-title":"Cyst formation and growth in autosomal dominant polycystic kidney disease","volume":"31","author":"Grantham","year":"1987","journal-title":"Kidney Int."},{"key":"10.14814\/phy2.13983-BIB0040|phy213983-cit-0040","doi-asserted-by":"crossref","first-page":"309","DOI":"10.1097\/00041552-200205000-00007","article-title":"Molecular basis of polycystic kidney disease: PKD1, PKD2 and PKHD1","volume":"11","author":"Harris","year":"2002","journal-title":"Curr. Opin. Nephrol. Hypertens."},{"key":"10.14814\/phy2.13983-BIB0041|phy213983-cit-0041","doi-asserted-by":"crossref","first-page":"18722","DOI":"10.1073\/pnas.0908853106","article-title":"Homozygous loss of BHD causes early embryonic lethality and kidney tumor development with activation of mTORC1 and mTORC2","volume":"106","author":"Hasumi","year":"2009","journal-title":"Proc. Natl. Acad. Sci. U S A"},{"key":"10.14814\/phy2.13983-BIB0042|phy213983-cit-0042","doi-asserted-by":"crossref","first-page":"295","DOI":"10.1002\/gcc.2870130411","article-title":"Loss of heterozygosity in the tuberous sclerosis (TSC2) region of chromosome band 16p13 occurs in sporadic as well as TSC-associated renal angiomyolipomas","volume":"13","author":"Henske","year":"1995","journal-title":"Genes Chromosom. Cancer"},{"key":"10.14814\/phy2.13983-BIB0043|phy213983-cit-0043","first-page":"400","article-title":"Allelic loss is frequent in tuberous sclerosis kidney lesions but rare in brain lesions","volume":"59","author":"Henske","year":"1996","journal-title":"Am. J. Hum. Genet."},{"key":"10.14814\/phy2.13983-BIB0044|phy213983-cit-0044","doi-asserted-by":"crossref","first-page":"278","DOI":"10.1681\/ASN.2008060564","article-title":"Characterization of PKD protein-positive exosome-like vesicles","volume":"20","author":"Hogan","year":"2009","journal-title":"J. Am. Soc. Nephrol."},{"key":"10.14814\/phy2.13983-BIB0045|phy213983-cit-0045","doi-asserted-by":"crossref","first-page":"827","DOI":"10.1038\/nbt.2647","article-title":"DNA targeting specificity of RNA-guided Cas9 nucleases","volume":"31","author":"Hsu","year":"2013","journal-title":"Nat. Biotechnol."},{"key":"10.14814\/phy2.13983-BIB0046|phy213983-cit-0046","doi-asserted-by":"crossref","first-page":"258","DOI":"10.1016\/j.ydbio.2007.04.044","article-title":"Foxi3 transcription factors and Notch signaling control the formation of skin ionocytes from epidermal precursors of the zebrafish embryo","volume":"307","author":"Janicke","year":"2007","journal-title":"Dev. Biol."},{"key":"10.14814\/phy2.13983-BIB0047|phy213983-cit-0047","doi-asserted-by":"crossref","first-page":"2155","DOI":"10.1093\/hmg\/6.12.2155","article-title":"Molecular genetic and phenotypic analysis reveals differences between TSC1 and TSC2 associated familial and sporadic tuberous sclerosis","volume":"6","author":"Jones","year":"1997","journal-title":"Hum. Mol. Genet."},{"key":"10.14814\/phy2.13983-BIB0048|phy213983-cit-0048","doi-asserted-by":"crossref","first-page":"170","DOI":"10.18632\/oncoscience.375","article-title":"Reprogramming patient-derived tumor cells generates model cell lines for tuberous sclerosis-associated lymphangioleiomyomatosis","volume":"4","author":"Julian","year":"2017","journal-title":"Oncoscience"},{"key":"10.14814\/phy2.13983-BIB0049|phy213983-cit-0049","doi-asserted-by":"crossref","first-page":"622","DOI":"10.1177\/197140090702000603","article-title":"Apparent diffusion coefficient is increased in children with tuberous sclerosis complex personal experience and review of the literature","volume":"20","author":"Jurkiewicz","year":"2007","journal-title":"Neuroradiol. J."},{"key":"10.14814\/phy2.13983-BIB0050|phy213983-cit-0050","doi-asserted-by":"crossref","first-page":"166","DOI":"10.1016\/j.bbrc.2016.10.019","article-title":"Multivesicular body formation enhancement and exosome release during endoplasmic reticulum stress","volume":"480","author":"Kanemoto","year":"2016","journal-title":"Biochem. Biophys. Res. Commun."},{"key":"10.14814\/phy2.13983-BIB0051|phy213983-cit-0051","doi-asserted-by":"crossref","first-page":"641","DOI":"10.1038\/sj.ki.5001627","article-title":"Increased expression of cyclooxygenase-2 in the renal cortex of human prorenin receptor gene-transgenic rats","volume":"70","author":"Kaneshiro","year":"2006","journal-title":"Kidney Int."},{"key":"10.14814\/phy2.13983-BIB0052|phy213983-cit-0052","doi-asserted-by":"crossref","first-page":"915","DOI":"10.1007\/s00726-016-2171-x","article-title":"Rag GTPase in amino acid signaling","volume":"48","author":"Kim","year":"2016","journal-title":"Amino Acids"},{"key":"10.14814\/phy2.13983-BIB0053|phy213983-cit-0053","doi-asserted-by":"crossref","first-page":"5482","DOI":"10.1038\/ncomms6482","article-title":"Ciliary membrane proteins traffic through the Golgi via a Rabep1\/GGA1\/Arl3-dependent mechanism","volume":"5","author":"Kim","year":"2014","journal-title":"Nat. Commun."},{"key":"10.14814\/phy2.13983-BIB0054|phy213983-cit-0054","doi-asserted-by":"crossref","first-page":"421","DOI":"10.1186\/1471-2407-12-421","article-title":"Hypoxic enhancement of exosome release by breast cancer cells","volume":"12","author":"King","year":"2012","journal-title":"BMC Cancer"},{"key":"10.14814\/phy2.13983-BIB0055|phy213983-cit-0055","doi-asserted-by":"crossref","first-page":"525","DOI":"10.1093\/hmg\/11.5.525","article-title":"A mouse model of TSC1 reveals sex-dependent lethality from liver hemangiomas, and up-regulation of p70S6 kinase activity in Tsc1 null cells","volume":"11","author":"Kwiatkowski","year":"2002","journal-title":"Hum. Mol. Genet."},{"key":"10.14814\/phy2.13983-BIB0056|phy213983-cit-0056","doi-asserted-by":"crossref","first-page":"219","DOI":"10.1002\/path.4827","article-title":"New developments in the genetics and pathogenesis of tumours in tuberous sclerosis complex","volume":"241","author":"Lam","year":"2017","journal-title":"J. Pathol."},{"key":"10.14814\/phy2.13983-BIB0057|phy213983-cit-0057","doi-asserted-by":"crossref","first-page":"107","DOI":"10.2174\/138920312800493188","article-title":"Regulation of V-ATPase expression in mammalian cells","volume":"13","author":"Lee","year":"2012","journal-title":"Curr. Protein Pept. Sci."},{"key":"10.14814\/phy2.13983-BIB0058|phy213983-cit-0058","doi-asserted-by":"crossref","first-page":"28","DOI":"10.1159\/000438606","article-title":"Regulation of CFTR expression and arginine vasopressin activity are dependent on polycystin-1 in kidney-derived cells","volume":"38","author":"Lemos Barbosa","year":"2016","journal-title":"Cell. Physiol. Biochem."},{"key":"10.14814\/phy2.13983-BIB0059|phy213983-cit-0059","doi-asserted-by":"crossref","first-page":"172","DOI":"10.1016\/j.tcb.2016.11.003","article-title":"Extracellular vesicles: unique intercellular delivery vehicles","volume":"27","author":"Maas","year":"2017","journal-title":"Trends Cell Biol."},{"key":"10.14814\/phy2.13983-BIB0060|phy213983-cit-0060","doi-asserted-by":"crossref","first-page":"1595","DOI":"10.1056\/NEJMoa1100391","article-title":"Efficacy and safety of sirolimus in lymphangioleiomyomatosis","volume":"364","author":"McCormack","year":"2011","journal-title":"N. Engl. J. Med."},{"key":"10.14814\/phy2.13983-BIB0500|phy213983-cit-0500","doi-asserted-by":"crossref","first-page":"218","DOI":"10.1023\/B:DDAS.0000017441.06479.c8","article-title":"Analysis of promoter region of X-linked pgk-1 gene polymorphisms: evidence for polyclonality of adult mouse gastric glands","volume":"49","author":"Nomura","year":"2004","journal-title":"Dig Dis Sci."},{"key":"10.14814\/phy2.13983-BIB0061|phy213983-cit-0061","doi-asserted-by":"crossref","first-page":"687","DOI":"10.1172\/JCI7319","article-title":"Tsc2(+\/-) mice develop tumors in multiple sites that express gelsolin and are influenced by genetic background","volume":"104","author":"Onda","year":"1999","journal-title":"J. Clin. Invest."},{"key":"10.14814\/phy2.13983-BIB0062|phy213983-cit-0062","doi-asserted-by":"crossref","first-page":"34211","DOI":"10.1074\/jbc.M109.041152","article-title":"Microenvironmental pH is a key factor for exosome traffic in tumor cells","volume":"284","author":"Parolini","year":"2009","journal-title":"J. Biol. Chem."},{"key":"10.14814\/phy2.13983-BIB0063|phy213983-cit-0063","doi-asserted-by":"crossref","first-page":"350","DOI":"10.1007\/s004390000390","article-title":"Molecular analysis of the TSC1 and TSC2 tumour suppressor genes in sporadic\u00a0glial and glioneuronal tumours","volume":"107","author":"Parry","year":"2000","journal-title":"Hum. Genet."},{"key":"10.14814\/phy2.13983-BIB0064|phy213983-cit-0064","doi-asserted-by":"crossref","first-page":"3027","DOI":"10.1038\/onc.2015.358","article-title":"Exosomes mediate the acquisition of the disease phenotypes by cells with normal genome in tuberous sclerosis complex","volume":"35","author":"Patel","year":"2016","journal-title":"Oncogene"},{"key":"10.14814\/phy2.13983-BIB0065|phy213983-cit-0065","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":"10.14814\/phy2.13983-BIB0066|phy213983-cit-0066","doi-asserted-by":"crossref","first-page":"3987","DOI":"10.4161\/cc.10.23.18251","article-title":"TFEB, a novel mTORC1 effector implicated in lysosome biogenesis, endocytosis and autophagy","volume":"10","author":"Pena-Llopis","year":"2011","journal-title":"Cell Cycle"},{"key":"10.14814\/phy2.13983-BIB0067|phy213983-cit-0067","doi-asserted-by":"crossref","first-page":"3242","DOI":"10.1038\/emboj.2011.257","article-title":"Regulation of TFEB and V-ATPases by mTORC1","volume":"30","author":"Pena-Llopis","year":"2011","journal-title":"EMBO J."},{"key":"10.14814\/phy2.13983-BIB0068|phy213983-cit-0068","doi-asserted-by":"crossref","first-page":"1245","DOI":"10.1007\/s00424-017-2005-z","article-title":"The (pro)renin receptor and its interaction partners","volume":"469","author":"Peters","year":"2017","journal-title":"Pflugers Arch."},{"key":"10.14814\/phy2.13983-BIB0069|phy213983-cit-0069","doi-asserted-by":"crossref","first-page":"37","DOI":"10.3389\/fmolb.2017.00037","article-title":"Extracellular vesicles in renal pathophysiology","volume":"4","author":"Pomatto","year":"2017","journal-title":"Front. Mol. Biosci."},{"key":"10.14814\/phy2.13983-BIB0070|phy213983-cit-0070","unstructured":"ProteinTech 2018 Tuberin-Specific Antibody Catalog number: 20004-1-AP https:\/\/www.ptglab.com\/products\/TSC2-Specific-Antibody-20004-1-AP.htm#validation. 2018"},{"key":"10.14814\/phy2.13983-BIB0071|phy213983-cit-0071","doi-asserted-by":"crossref","first-page":"153","DOI":"10.1186\/s13287-015-0150-x","article-title":"Role of extracellular RNA-carrying vesicles in cell differentiation and reprogramming","volume":"6","author":"Quesenberry","year":"2015","journal-title":"Stem Cell Res. Ther."},{"key":"10.14814\/phy2.13983-BIB0072|phy213983-cit-0072","doi-asserted-by":"crossref","first-page":"C263","DOI":"10.1152\/ajpcell.00381.2012","article-title":"Prostaglandin E2 induces chloride secretion through crosstalk between cAMP and calcium signaling in mouse inner medullary collecting duct cells","volume":"306","author":"Rajagopal","year":"2014","journal-title":"Am. J. Physiol. Cell Physiol."},{"key":"10.14814\/phy2.13983-BIB0073|phy213983-cit-0073","doi-asserted-by":"crossref","first-page":"1777","DOI":"10.1038\/sj.ki.5001853","article-title":"Renal manifestations of tuberous sclerosis complex: incidence, prognosis, and predictive factors","volume":"70","author":"Rakowski","year":"2006","journal-title":"Kidney Int."},{"key":"10.14814\/phy2.13983-BIB0074|phy213983-cit-0074","doi-asserted-by":"crossref","first-page":"F1145","DOI":"10.1152\/ajprenal.00476.2016","article-title":"The nephron (pro)renin receptor: function and significance","volume":"311","author":"Ramkumar","year":"2016a","journal-title":"Am. J. Physiol. Renal Physiol."},{"key":"10.14814\/phy2.13983-BIB0075|phy213983-cit-0075","doi-asserted-by":"crossref","first-page":"29","DOI":"10.1007\/s11906-016-0638-5","article-title":"Role of the collecting duct renin angiotensin system in regulation of blood pressure and renal function","volume":"18","author":"Ramkumar","year":"2016b","journal-title":"Curr. Hypertens. Rep."},{"key":"10.14814\/phy2.13983-BIB0076|phy213983-cit-0076","doi-asserted-by":"crossref","first-page":"310","DOI":"10.1159\/000484096","article-title":"The von hippel-lindau gene is required to maintain renal proximal tubule and glomerulus integrity in zebrafish larvae","volume":"138","author":"Rooijen","year":"2018","journal-title":"Nephron"},{"issue":"2017","key":"10.14814\/phy2.13983-BIB0077|phy213983-cit-0077","doi-asserted-by":"crossref","first-page":"102","DOI":"10.5414\/NP301008","article-title":"Astrocytic tumor with large cells and worrisome features in two patients with tuberous sclerosis: drastically different diagnoses and prognoses","volume":"36","author":"Roux","year":"2017","journal-title":"Clin. Neuropathol."},{"key":"10.14814\/phy2.13983-BIB0078|phy213983-cit-0078","doi-asserted-by":"crossref","first-page":"305","DOI":"10.2215\/CJN.08880914","article-title":"Collecting duct intercalated cell function and regulation","volume":"10","author":"Roy","year":"2015","journal-title":"Clin. J. Am. Soc. Nephrol."},{"key":"10.14814\/phy2.13983-BIB0079|phy213983-cit-0079","doi-asserted-by":"crossref","first-page":"e12405","DOI":"10.14814\/phy2.12405","article-title":"Activation of the intrarenal renin-angiotensin-system in murine polycystic kidney disease","volume":"3","author":"Saigusa","year":"2015","journal-title":"Physiol. Rep."},{"key":"10.14814\/phy2.13983-BIB0080|phy213983-cit-0080","doi-asserted-by":"crossref","first-page":"290","DOI":"10.1016\/j.cell.2010.02.024","article-title":"Ragulator-Rag complex targets mTORC1 to the lysosomal surface and is necessary for its activation by amino acids","volume":"141","author":"Sancak","year":"2010","journal-title":"Cell"},{"key":"10.14814\/phy2.13983-BIB0081|phy213983-cit-0081","doi-asserted-by":"crossref","first-page":"2743","DOI":"10.1093\/hmg\/9.18.2743","article-title":"Polycystin-1, the product of the polycystic kidney disease 1 gene, co-localizes with desmosomes in MDCK cells","volume":"9","author":"Scheffers","year":"2000","journal-title":"Hum. Mol. Genet."},{"key":"10.14814\/phy2.13983-BIB0082|phy213983-cit-0082","doi-asserted-by":"crossref","first-page":"26","DOI":"10.1038\/nrneph.2012.249","article-title":"Key developments in renin-angiotensin-aldosterone system inhibition","volume":"9","author":"Seva Pessoa","year":"2013","journal-title":"Nat. Rev. Nephrol."},{"key":"10.14814\/phy2.13983-BIB0083|phy213983-cit-0083","doi-asserted-by":"crossref","first-page":"500","DOI":"10.2741\/E631","article-title":"(Pro)renin receptor: subcellular localizations and functions","volume":"5","author":"Sihn","year":"2013","journal-title":"Front. Biosci. (Elite Ed)"},{"key":"10.14814\/phy2.13983-BIB0084|phy213983-cit-0084","first-page":"143","article-title":"Renal involvement in tuberous sclerosis complex and von hippel-lindau disease: shared disease mechanisms?","volume":"5","author":"Siroky","year":"2009","journal-title":"Nat. Clin. Pract. Nephrol."},{"key":"10.14814\/phy2.13983-BIB0085|phy213983-cit-0085","doi-asserted-by":"crossref","first-page":"F831","DOI":"10.1152\/ajprenal.00441.2011","article-title":"Human TSC-associated renal angiomyolipoma cells are hypersensitive to ER stress","volume":"303","author":"Siroky","year":"2012","journal-title":"Am. J. Physiol. Renal Physiol."},{"key":"10.14814\/phy2.13983-BIB0086|phy213983-cit-0086","doi-asserted-by":"crossref","first-page":"F560","DOI":"10.1152\/ajprenal.00569.2013","article-title":"Evidence for pericyte origin of TSC-associated renal angiomyolipomas and implications for angiotensin receptor inhibition therapy","volume":"307","author":"Siroky","year":"2014","journal-title":"Am. J. Physiol. Renal Physiol."},{"key":"10.14814\/phy2.13983-BIB0087|phy213983-cit-0087","doi-asserted-by":"crossref","first-page":"F791","DOI":"10.1152\/ajprenal.00465.2015","article-title":"Primary cilia regulate the osmotic stress response of renal epithelial cells through TRPM3","volume":"312","author":"Siroky","year":"2017a","journal-title":"Am. J. Physiol. Renal Physiol."},{"key":"10.14814\/phy2.13983-BIB0088|phy213983-cit-0088","doi-asserted-by":"crossref","first-page":"318","DOI":"10.1016\/j.jpeds.2017.05.015","article-title":"Improvement in renal cystic disease of tuberous sclerosis complex after treatment with mammalian target of rapamycin inhibitor","volume":"187","author":"Siroky","year":"2017b","journal-title":"J. Pediatr."},{"key":"10.14814\/phy2.13983-BIB0089|phy213983-cit-0089","doi-asserted-by":"crossref","first-page":"134","DOI":"10.1016\/j.cell.2010.09.016","article-title":"Intestinal crypt homeostasis results from neutral competition between symmetrically dividing Lgr5 stem cells","volume":"143","author":"Snippert","year":"2010","journal-title":"Cell"},{"key":"10.14814\/phy2.13983-BIB0090|phy213983-cit-0090","doi-asserted-by":"crossref","first-page":"477","DOI":"10.1016\/S0022-5347(17)43234-4","article-title":"Renal lesions in tuberous sclerosis","volume":"138","author":"Stillwell","year":"1987","journal-title":"J. Urol."},{"key":"10.14814\/phy2.13983-BIB0091|phy213983-cit-0091","doi-asserted-by":"crossref","first-page":"1168","DOI":"10.1038\/ki.1991.148","article-title":"Characterization of a mouse cortical collecting duct cell line","volume":"39","author":"Stoos","year":"1991","journal-title":"Kidney Int."},{"key":"10.14814\/phy2.13983-BIB0092|phy213983-cit-0092","doi-asserted-by":"crossref","first-page":"1165","DOI":"10.1152\/physrev.1998.78.4.1165","article-title":"Epithelial transport in polycystic kidney disease","volume":"78","author":"Sullivan","year":"1998","journal-title":"Physiol. Rev."},{"key":"10.14814\/phy2.13983-BIB0093|phy213983-cit-0093","unstructured":"Team RC R: A language and environment for statistical computing R Foundation for Statistical Computing 2018 http:\/\/www.R-project.org\/"},{"key":"10.14814\/phy2.13983-BIB0094|phy213983-cit-0094","unstructured":"Therneau , T 2018 A Package for Survival Analysis in S. version 2.38 https:\/\/CRAN.R-project.org\/package=survival"},{"key":"10.14814\/phy2.13983-BIB0095|phy213983-cit-0095","doi-asserted-by":"crossref","first-page":"350","DOI":"10.1007\/978-1-4757-3294-8","volume-title":"Modeling Survival Data: extending the Cox Model","author":"Therneau","year":"2000"},{"key":"10.14814\/phy2.13983-BIB0096|phy213983-cit-0096","doi-asserted-by":"crossref","first-page":"979","DOI":"10.1038\/nm.1865","article-title":"An efficient and versatile system for acute and chronic modulation of renal tubular function in transgenic mice","volume":"14","author":"Traykova-Brauch","year":"2008","journal-title":"Nat. Med."},{"key":"10.14814\/phy2.13983-BIB0097|phy213983-cit-0097","doi-asserted-by":"crossref","first-page":"1551","DOI":"10.1159\/000487691","article-title":"Downregulation of the Cl-\/HCO3-exchanger pendrin in kidneys of mice with cystic fibrosis: role in the pathogenesis of metabolic alkalosis","volume":"45","author":"Varasteh Kia","year":"2018","journal-title":"Cell. Physiol. Biochem."},{"key":"10.14814\/phy2.13983-BIB0098|phy213983-cit-0098","doi-asserted-by":"crossref","first-page":"7934","DOI":"10.1158\/0008-5472.CAN-06-1740","article-title":"Tsc1 haploinsufficiency without mammalian target of rapamycin activation is sufficient for renal cyst formation in Tsc1\u00a0+\u00a0\/- mice","volume":"66","author":"Wilson","year":"2006","journal-title":"Cancer Res."},{"key":"10.14814\/phy2.13983-BIB0099|phy213983-cit-0099","doi-asserted-by":"crossref","first-page":"926","DOI":"10.1038\/ki.2011.196","article-title":"Slc26a11, a chloride transporter, localizes with the vacuolar H(+)-ATPase of A-intercalated cells of the kidney","volume":"80","author":"Xu","year":"2011","journal-title":"Kidney Int."},{"key":"10.14814\/phy2.13983-BIB0100|phy213983-cit-0100","doi-asserted-by":"crossref","first-page":"e79327","DOI":"10.1371\/journal.pone.0079327","article-title":"Potentiation of the effect of thiazide derivatives by carbonic anhydrase inhibitors: molecular mechanisms and potential clinical implications","volume":"8","author":"Zahedi","year":"2013","journal-title":"PLoS ONE"},{"key":"10.14814\/phy2.13983-BIB0101|phy213983-cit-0101","doi-asserted-by":"crossref","first-page":"4428","DOI":"10.1093\/hmg\/ddp398","article-title":"Loss of Tsc1, but not Pten, in renal tubular cells causes polycystic kidney disease by activating mTORC1","volume":"18","author":"Zhou","year":"2009","journal-title":"Hum. Mol. Genet."},{"key":"10.14814\/phy2.13983-BIB0102|phy213983-cit-0102","doi-asserted-by":"crossref","first-page":"1046","DOI":"10.1016\/j.cell.2015.04.042","article-title":"In Vivo imaging reveals extracellular vesicle-mediated phenocopying of metastatic behavior","volume":"161","author":"Zomer","year":"2015","journal-title":"Cell"},{"key":"10.14814\/phy2.13983-BIB0103|phy213983-cit-0103","doi-asserted-by":"crossref","first-page":"678","DOI":"10.1126\/science.1207056","article-title":"mTORC1 senses lysosomal amino acids through an inside-out mechanism that requires the vacuolar H(+)-ATPase","volume":"334","author":"Zoncu","year":"2011","journal-title":"Science"}],"container-title":["Physiological Reports"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.14814%2Fphy2.13983","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/onlinelibrary.wiley.com\/wol1\/doi\/10.14814\/phy2.13983\/fullpdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2020,11,23]],"date-time":"2020-11-23T16:03:36Z","timestamp":1606147416000},"score":1,"resource":{"primary":{"URL":"http:\/\/doi.wiley.com\/10.14814\/phy2.13983"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2019,1]]},"references-count":104,"journal-issue":{"issue":"2","published-print":{"date-parts":[[2019,1]]}},"URL":"https:\/\/doi.org\/10.14814\/phy2.13983","archive":["Portico"],"relation":{},"ISSN":["2051-817X"],"issn-type":[{"value":"2051-817X","type":"print"}],"subject":[],"published":{"date-parts":[[2019,1]]}}}