{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,30]],"date-time":"2026-09-30T23:38:07Z","timestamp":1790811487735,"version":"4.1.0"},"reference-count":50,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"6","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2014,6]]},"DOI":"10.1681\/asn.2013060598","type":"journal-article","created":{"date-parts":[[2014,2,13]],"date-time":"2014-02-13T23:31:04Z","timestamp":1392334264000},"page":"1256-1269","source":"Crossref","is-referenced-by-count":81,"title":["Time Course of Pathogenic and Adaptation Mechanisms in Cystinotic Mouse Kidneys"],"prefix":"10.1681","volume":"25","author":[{"given":"H\u00e9lo\u00efse P.","family":"Gaide Chevronnay","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Virginie","family":"Janssens","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Patrick","family":"Van Der Smissen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Francisca","family":"N\u2019Kuli","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nathalie","family":"Nevo","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yves","family":"Guiot","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elena","family":"Levtchenko","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Etienne","family":"Marbaix","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christophe E.","family":"Pierreux","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"St\u00e9phanie","family":"Cherqui","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Corinne","family":"Antignac","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pierre J.","family":"Courtoy","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"276","reference":[{"key":"R1-16-20230208","doi-asserted-by":"crossref","first-page":"111","DOI":"10.1056\/NEJMra020552","article-title":"Cystinosis.","volume":"347","author":"Gahl","year":"2002","journal-title":"N Engl J Med"},{"key":"R2-16-20230208","doi-asserted-by":"crossref","first-page":"51","DOI":"10.1007\/s00467-012-2242-5","article-title":"Cystinosis: The evolution of a treatable disease.","volume":"28","author":"Nesterova","year":"2013","journal-title":"Pediatr Nephrol"},{"key":"R3-16-20230208","doi-asserted-by":"crossref","first-page":"5940","DOI":"10.1093\/emboj\/20.21.5940","article-title":"Cystinosin, the protein defective in cystinosis, is a H(+)-driven lysosomal cystine transporter.","volume":"20","author":"Kalatzis","year":"2001","journal-title":"EMBO J"},{"key":"R4-16-20230208","doi-asserted-by":"crossref","first-page":"1361","DOI":"10.1093\/hmg\/ddh152","article-title":"Molecular pathogenesis of cystinosis: Effect of CTNS mutations on the transport activity and subcellular localization of cystinosin.","volume":"13","author":"Kalatzis","year":"2004","journal-title":"Hum Mol Genet"},{"key":"R5-16-20230208","doi-asserted-by":"crossref","first-page":"1059","DOI":"10.1093\/ndt\/gfp553","article-title":"Renal phenotype of the cystinosis mouse model is dependent upon genetic background.","volume":"25","author":"Nevo","year":"2010","journal-title":"Nephrol Dial Transplant"},{"key":"R6-16-20230208","doi-asserted-by":"crossref","first-page":"1152","DOI":"10.1126\/science.166.3909.1152","article-title":"Cystine: Compartmentalization within lysosomes in cystinotic leukocytes.","volume":"166","author":"Schulman","year":"1969","journal-title":"Science"},{"key":"R7-16-20230208","doi-asserted-by":"crossref","first-page":"859","DOI":"10.1126\/science.168.3933.859","article-title":"Human cystinosis: Intracellular deposition of cystine.","volume":"168","author":"Hummeler","year":"1970","journal-title":"Science"},{"key":"R8-16-20230208","doi-asserted-by":"crossref","first-page":"154","DOI":"10.1016\/0006-291X(83)90394-7","article-title":"The intralysosomal pH in cultured human skin fibroblasts in relation to cystine accumulation in patients with cystinosis.","volume":"116","author":"Oude Elferink","year":"1983","journal-title":"Biochem Biophys Res Commun"},{"key":"R9-16-20230208","doi-asserted-by":"crossref","first-page":"4505","DOI":"10.1073\/pnas.74.10.4505","article-title":"Cystinotic fibroblasts accumulate cystine from intracellular protein degradation.","volume":"74","author":"Thoene","year":"1977","journal-title":"Proc Natl Acad Sci U S A"},{"key":"R10-16-20230208","doi-asserted-by":"crossref","first-page":"785","DOI":"10.1203\/00006450-198006000-00001","article-title":"Modulation of the intracellular cystine content of cystinotic fibroblasts by extracellular albumin.","volume":"14","author":"Thoene","year":"1980","journal-title":"Pediatr Res"},{"key":"R11-16-20230208","doi-asserted-by":"crossref","first-page":"823","DOI":"10.1042\/bj2080823","article-title":"Cystine accumulation in cystinotic fibroblasts from free and protein-linked cystine but not cysteine.","volume":"208","author":"Thoene","year":"1982","journal-title":"Biochem J"},{"key":"R12-16-20230208","doi-asserted-by":"crossref","first-page":"327","DOI":"10.1083\/jcb.110.2.327","article-title":"A cysteine-specific lysosomal transport system provides a major route for the delivery of thiol to human fibroblast lysosomes: Possible role in supporting lysosomal proteolysis.","volume":"110","author":"Pisoni","year":"1990","journal-title":"J Cell Biol"},{"key":"R13-16-20230208","first-page":"43","article-title":"Rapid membrane recycling in renal proximal tubule cells.","volume":"29","author":"Christensen","year":"1982","journal-title":"Eur J Cell Biol"},{"key":"R14-16-20230208","doi-asserted-by":"crossref","first-page":"933","DOI":"10.1111\/tra.12079","article-title":"Class III phosphoinositide 3-kinase\/VPS34 and dynamin are critical for apical endocytic recycling.","volume":"14","author":"Carpentier","year":"2013","journal-title":"Traffic"},{"key":"R15-16-20230208","doi-asserted-by":"crossref","first-page":"421","DOI":"10.1016\/j.devcel.2011.07.016","article-title":"Transcriptional activation of lysosomal exocytosis promotes cellular clearance.","volume":"21","author":"Medina","year":"2011","journal-title":"Dev Cell"},{"key":"R16-16-20230208","doi-asserted-by":"crossref","first-page":"805","DOI":"10.1002\/cphy.c100073","article-title":"Uriniferous tubule: Structural and functional organization.","volume":"2","author":"Christensen","year":"2012","journal-title":"Compr Physiol"},{"key":"R17-16-20230208","doi-asserted-by":"crossref","first-page":"50","DOI":"10.1007\/PL00013448","article-title":"Early development of the renal lesions in infantile cystinosis.","volume":"15","author":"Mahoney","year":"2000","journal-title":"Pediatr Nephrol"},{"key":"R18-16-20230208","doi-asserted-by":"crossref","first-page":"478","DOI":"10.1681\/ASN.2009070698","article-title":"ZONAB promotes proliferation and represses differentiation of proximal tubule epithelial cells.","volume":"21","author":"Lima","year":"2010","journal-title":"J Am Soc Nephrol"},{"key":"R19-16-20230208","doi-asserted-by":"crossref","first-page":"1699","DOI":"10.1089\/ars.2012.5033","article-title":"Functions and cellular compartmentation of the thioredoxin and glutathione pathways in yeast.","volume":"18","author":"Toledano","year":"2013","journal-title":"Antioxid Redox Signal"},{"key":"R20-16-20230208","doi-asserted-by":"crossref","first-page":"993","DOI":"10.1007\/s00467-002-0956-5","article-title":"Megalin and cubilin, role in proximal tubule function and during development.","volume":"17","author":"Christensen","year":"2002","journal-title":"Pediatr Nephrol"},{"key":"R21-16-20230208","doi-asserted-by":"crossref","first-page":"608","DOI":"10.1083\/jcb.64.3.608","article-title":"A morphometric study of cellular autophagy including diurnal variations in kidney tubules of normal rats.","volume":"64","author":"Pfeifer","year":"1975","journal-title":"J Cell Biol"},{"key":"R22-16-20230208","doi-asserted-by":"crossref","first-page":"256","DOI":"10.1038\/nrm778","article-title":"Megalin and cubilin: Multifunctional endocytic receptors.","volume":"3","author":"Christensen","year":"2002","journal-title":"Nat Rev Mol Cell Biol"},{"key":"R23-16-20230208","doi-asserted-by":"crossref","first-page":"1361","DOI":"10.1016\/S0002-9440(10)65238-8","article-title":"Megalin knockout mice as an animal model of low molecular weight proteinuria.","volume":"155","author":"Leheste","year":"1999","journal-title":"Am J Pathol"},{"key":"R24-16-20230208","doi-asserted-by":"crossref","first-page":"3446","DOI":"10.1093\/ndt\/gfr525","article-title":"Mouse model of proximal tubule endocytic dysfunction.","volume":"26","author":"Weyer","year":"2011","journal-title":"Nephrol Dial Transplant"},{"key":"R25-16-20230208","doi-asserted-by":"crossref","first-page":"591","DOI":"10.1681\/ASN.2011101034","article-title":"Generation of urinary albumin fragments does not require proximal tubular uptake.","volume":"23","author":"Weyer","year":"2012","journal-title":"J Am Soc Nephrol"},{"key":"R26-16-20230208","doi-asserted-by":"crossref","first-page":"1859","DOI":"10.1681\/ASN.2010050492","article-title":"Cubilin is essential for albumin reabsorption in the renal proximal tubule.","volume":"21","author":"Amsellem","year":"2010","journal-title":"J Am Soc Nephrol"},{"key":"R27-16-20230208","doi-asserted-by":"crossref","first-page":"8472","DOI":"10.1073\/pnas.1432873100","article-title":"Loss of chloride channel ClC-5 impairs endocytosis by defective trafficking of megalin and cubilin in kidney proximal tubules.","volume":"100","author":"Christensen","year":"2003","journal-title":"Proc Natl Acad Sci U S A"},{"key":"R28-16-20230208","doi-asserted-by":"crossref","first-page":"5407","DOI":"10.1073\/pnas.0700330104","article-title":"Endocytosis provides a major alternative pathway for lysosomal biogenesis in kidney proximal tubular cells.","volume":"104","author":"Nielsen","year":"2007","journal-title":"Proc Natl Acad Sci U S A"},{"key":"R29-16-20230208","doi-asserted-by":"crossref","first-page":"893","DOI":"10.1053\/j.ajkd.2008.03.010","article-title":"Urinary protein excretion pattern and renal expression of megalin and cubilin in nephropathic cystinosis.","volume":"51","author":"Wilmer","year":"2008","journal-title":"Am J Kidney Dis"},{"key":"R30-16-20230208","doi-asserted-by":"crossref","first-page":"611","DOI":"10.1097\/01.ASN.0000051725.00406.0C","article-title":"Digital three-dimensional reconstruction and ultrastructure of the mouse proximal tubule.","volume":"14","author":"Zhai","year":"2003","journal-title":"J Am Soc Nephrol"},{"key":"R31-16-20230208","doi-asserted-by":"crossref","first-page":"497","DOI":"10.1083\/jcb.20.3.497","article-title":"Cytochemical observations on the relationship between lysosomes and phagosomes in kidney and liver by combined staining for acid phosphatase and intravenously injected horseradish peroxidase.","volume":"20","author":"Straus","year":"1964","journal-title":"J Cell Biol"},{"key":"R32-16-20230208","doi-asserted-by":"crossref","first-page":"F454","DOI":"10.1152\/ajprenal.00413.2009","article-title":"Single photon emission-computed tomography (SPECT) for functional investigation of the proximal tubule in conscious mice.","volume":"298","author":"Jouret","year":"2010","journal-title":"Am J Physiol Renal Physiol"},{"key":"R33-16-20230208","doi-asserted-by":"crossref","first-page":"2878","DOI":"10.1097\/01.ASN.0000036867.49866.59","article-title":"Lysosomal cystine storage augments apoptosis in cultured human fibroblasts and renal tubular epithelial cells.","volume":"13","author":"Park","year":"2002","journal-title":"J Am Soc Nephrol"},{"key":"R34-16-20230208","doi-asserted-by":"crossref","first-page":"F905","DOI":"10.1152\/ajprenal.00318.2010","article-title":"The pathogenesis of cystinosis: Mechanisms beyond cystine accumulation.","volume":"299","author":"Wilmer","year":"2010","journal-title":"Am J Physiol Renal Physiol"},{"key":"R35-16-20230208","doi-asserted-by":"crossref","first-page":"417","DOI":"10.1016\/j.ymgme.2010.08.015","article-title":"The incidence of atubular glomeruli in nephropathic cystinosis renal biopsies.","volume":"101","author":"Larsen","year":"2010","journal-title":"Mol Genet Metab"},{"key":"R36-16-20230208","doi-asserted-by":"crossref","first-page":"197","DOI":"10.1681\/ASN.2007080862","article-title":"Generation and evolution of atubular glomeruli in the progression of renal disorders.","volume":"19","author":"Chevalier","year":"2008","journal-title":"J Am Soc Nephrol"},{"key":"R37-16-20230208","doi-asserted-by":"crossref","first-page":"473","DOI":"10.1126\/science.1174447","article-title":"A gene network regulating lysosomal biogenesis and function.","volume":"325","author":"Sardiello","year":"2009","journal-title":"Science"},{"key":"R38-16-20230208","doi-asserted-by":"crossref","first-page":"2950","DOI":"10.1128\/MCB.00417-13","article-title":"Upregulation of the Rab27a-dependent trafficking and secretory mechanisms improves lysosomal transport, alleviates endoplasmic reticulum stress, and reduces lysosome overload in cystinosis.","volume":"33","author":"Johnson","year":"2013","journal-title":"Mol Cell Biol"},{"key":"R39-16-20230208","doi-asserted-by":"crossref","first-page":"435","DOI":"10.1146\/annurev.ph.28.030166.002251","article-title":"Functions of lysosomes.","volume":"28","author":"De Duve","year":"1966","journal-title":"Annu Rev Physiol"},{"key":"R40-16-20230208","doi-asserted-by":"crossref","first-page":"197","DOI":"10.1016\/S0022-5320(66)80059-X","article-title":"Observations on the ultrastructure and acid phosphatase activity of the cytoplasmic bodies in rat kidney proximal tubule cells. With a comment on their classification.","volume":"16","author":"Maunsbach","year":"1966","journal-title":"J Ultrastruct Res"},{"key":"R41-16-20230208","first-page":"2212","article-title":"Quantitative in vivo and ex vivo confocal microscopy analysis of corneal cystine crystals in the Ctns knockout mouse.","volume":"17","author":"Simpson","year":"2011","journal-title":"Mol Vis"},{"key":"R42-16-20230208","doi-asserted-by":"crossref","first-page":"105","DOI":"10.1016\/0002-9343(86)90487-0","article-title":"Experimental studies on nephrotoxicity of aminoglycosides at low doses. Mechanisms and perspectives.","volume":"80","author":"Tulkens","year":"1986","journal-title":"Am J Med"},{"key":"R43-16-20230208","doi-asserted-by":"crossref","first-page":"375","DOI":"10.1016\/S0021-9258(18)84756-1","article-title":"The colorimetric determination of phosphorus.","volume":"66","author":"Fiske","year":"1925","journal-title":"J Biol Chem"},{"key":"R44-16-20230208","doi-asserted-by":"crossref","first-page":"242","DOI":"10.1016\/S0022-5320(66)80109-0","article-title":"The influence of different fixatives and fixation methods on the ultrastructure of rat kidney proximal tubule cells. I. Comparison of different perfusion fixation methods and of glutaraldehyde, formaldehyde and osmium tetroxide fixatives.","volume":"15","author":"Maunsbach","year":"1966","journal-title":"J Ultrastruct Res"},{"key":"R45-16-20230208","doi-asserted-by":"crossref","first-page":"1015","DOI":"10.1210\/en.2007-0849","article-title":"Opposite regulation of transforming growth factors-beta2 and -beta3 expression in the human endometrium.","volume":"149","author":"Gaide Chevronnay","year":"2008","journal-title":"Endocrinology"},{"key":"R46-16-20230208","doi-asserted-by":"crossref","first-page":"351","DOI":"10.1111\/j.1582-4934.2007.00192.x","article-title":"Intravital multi-photon microscopy reveals several levels of heterogeneity in endocytic uptake by mouse renal proximal tubules.","volume":"12","author":"Caplanusi","year":"2008","journal-title":"J Cell Mol Med"},{"key":"R47-16-20230208","doi-asserted-by":"crossref","first-page":"216","DOI":"10.1016\/j.ydbio.2010.08.024","article-title":"Epithelial: Endothelial cross-talk regulates exocrine differentiation in developing pancreas.","volume":"347","author":"Pierreux","year":"2010","journal-title":"Dev Biol"},{"key":"R48-16-20230208","doi-asserted-by":"crossref","first-page":"66","DOI":"10.1186\/1471-213X-9-66","article-title":"Mechanism of primitive duct formation in the pancreas and submandibular glands: A role for SDF-1.","volume":"9","author":"Hick","year":"2009","journal-title":"BMC Dev Biol"},{"key":"R49-16-20230208","doi-asserted-by":"crossref","first-page":"2795","DOI":"10.1093\/ndt\/gfm898","article-title":"Lysosomal enzymuria is a feature of hereditary Fanconi syndrome and is related to elevated CI-mannose-6-P-receptor excretion.","volume":"23","author":"Norden","year":"2008","journal-title":"Nephrol Dial Transplant"},{"key":"R50-16-20230208","doi-asserted-by":"crossref","first-page":"622","DOI":"10.1038\/nrm2217","article-title":"Lysosomes: Fusion and function.","volume":"8","author":"Luzio","year":"2007","journal-title":"Nat Rev Mol Cell Biol"}],"container-title":["Journal of the American Society of Nephrology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.ovid.com\/00001751-201406000-00016","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,8,7]],"date-time":"2026-08-07T19:23:50Z","timestamp":1786130630000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.ovid.com\/00001751-201406000-00016"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2014,6]]},"references-count":50,"journal-issue":{"issue":"6","published-print":{"date-parts":[[2014]]}},"URL":"https:\/\/doi.org\/10.1681\/asn.2013060598","relation":{},"ISSN":["1046-6673"],"issn-type":[{"value":"1046-6673","type":"print"}],"subject":[],"published":{"date-parts":[[2014,6]]}}}