{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,7]],"date-time":"2026-08-07T19:57:23Z","timestamp":1786132643982,"version":"build-2736575974"},"reference-count":58,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"11","funder":[{"DOI":"10.13039\/100005674","name":"CRF","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100005674","id-type":"DOI","asserted-by":"publisher"}]},{"name":"National Lottery"},{"name":"R\u00e9gion bruxelloise"},{"name":"R\u00e9gion wallonne"},{"DOI":"10.13039\/100007370","name":"Universit\u00e9 Catholique de Louvain","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100007370","id-type":"DOI","asserted-by":"publisher"}]},{"name":"de Duve Institute"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JASN"],"published-print":{"date-parts":[[2019,11]]},"abstract":"<jats:sec>\n                    <jats:title>Significance Statement<\/jats:title>\n                    <jats:p>\n                      Nephropathic cystinosis is the result of deletion or inactivating mutations of the gene encoding the lysosomal cystine transporter cystinosin, but the extent to which disease progression depends on cystine accumulation or transport-independent effects of cystinosin is unknown. Cysteamine, the current treatment to prevent cystine accumulation, delays progression to renal failure but does not correct the Fanconi syndrome nor does it provide a cure. The authors demonstrate that suppression of endocytosis in kidney proximal tubular cells of cystinosin-deficient mice by genetic excision of\n                      <jats:italic toggle=\"yes\">megalin\/Lrp2<\/jats:italic>\n                      largely prevents cystine accumulation and can help preserve kidney structure and proximal tubular cell differentiation. These observations stress the importance of cystine accumulation in disease progression and provide proof of concept for exploring novel strategies aiming at blocking the megalin pathway.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>\n                      Deletions or inactivating mutations of the cystinosin gene\n                      <jats:italic toggle=\"yes\">CTNS<\/jats:italic>\n                      lead to cystine accumulation and crystals at acidic pH in patients with nephropathic cystinosis, a rare lysosomal storage disease and the main cause of hereditary renal Fanconi syndrome. Early use of oral cysteamine to prevent cystine accumulation slows progression of nephropathic cystinosis but it is a demanding treatment and not a cure. The source of cystine accumulating in kidney proximal tubular cells and cystine\u2019s role in disease progression are unknown.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>\n                      To investigate whether receptor-mediated endocytosis by the megalin\/LRP2 pathway of ultrafiltrated, disulfide-rich plasma proteins could be a source of cystine in proximal tubular cells, we used a mouse model of cystinosis in which conditional excision of floxed\n                      <jats:italic toggle=\"yes\">megalin\/LRP2<\/jats:italic>\n                      alleles in proximal tubular cells of cystinotic mice was achieved by a Cre-LoxP strategy using\n                      <jats:italic toggle=\"yes\">Wnt4-CRE<\/jats:italic>\n                      . We evaluated mice aged 6\u20139 months for kidney cystine levels and crystals; histopathology, with emphasis on swan-neck lesions and proximal-tubular-cell apoptosis and proliferation (turnover); and proximal-tubular-cell expression of the major apical transporters sodium-phosphate cotransporter 2A (NaPi-IIa) and sodium-glucose cotransporter-2 (SGLT-2).\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      <jats:italic toggle=\"yes\">Wnt4-CRE<\/jats:italic>\n                      \u2013driven\n                      <jats:italic toggle=\"yes\">megalin\/LRP2<\/jats:italic>\n                      ablation in cystinotic mice efficiently blocked kidney cystine accumulation, thereby preventing lysosomal deformations and crystal deposition in proximal tubular cells. Swan-neck lesions were largely prevented and proximal-tubular-cell turnover was normalized. Apical expression of the two cotransporters was also preserved.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>These observations support a key role of the megalin\/LRP2 pathway in the progression of nephropathic cystinosis and provide a proof of concept for the pathway as a therapeutic target.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1681\/asn.2019040371","type":"journal-article","created":{"date-parts":[[2019,9,23]],"date-time":"2019-09-23T16:44:06Z","timestamp":1569257046000},"page":"2177-2190","source":"Crossref","is-referenced-by-count":17,"title":["Protection of Cystinotic Mice by Kidney-Specific Megalin Ablation Supports an Endocytosis-Based Mechanism for Nephropathic Cystinosis Progression"],"prefix":"10.1681","volume":"30","author":[{"given":"Virginie","family":"Janssens","sequence":"first","affiliation":[{"name":"Cell Biology Unit, 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