{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,29]],"date-time":"2026-09-29T17:22:27Z","timestamp":1790702547353,"version":"4.1.0"},"reference-count":16,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2021,11,11]],"date-time":"2021-11-11T00:00:00Z","timestamp":1636588800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["J Intern Med"],"published-print":{"date-parts":[[2022,3]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Kidney failure is the major cause of morbidity and mortality in familial lecithin:cholesterol acyltransferase deficiency (FLD), a rare inherited lipid disorder with no cure. Lipoprotein X (LpX), an abnormal lipoprotein, is primarily accountable for nephrotoxicity.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>CER\u2010001 was tested in an FLD patient with dramatic kidney disease for 12 weeks.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Infusions of CER\u2010001 normalized the lipoprotein profile, with a disappearance of the abnormal LpX in favour of normal\u2010sized LDL. The worsening of kidney function was slowed by the treatment, and kidney biopsy showed a slight reduction of lipid deposits and a stabilization of the disease. In vitro experiments demonstrate that CER\u2010001 progressively reverts lipid accumulation in podocytes by a dual effect: remodelling plasma lipoproteins and removing LpX\u2010induced lipid deposit.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>This study demonstrates that CER\u2010001 may represent a therapeutic option in FLD patients. It also has the potential to be beneficial in other renal diseases characterized by kidney lipid deposits.<\/jats:p><\/jats:sec>","DOI":"10.1111\/joim.13404","type":"journal-article","created":{"date-parts":[[2021,11,11]],"date-time":"2021-11-11T12:14:44Z","timestamp":1636632884000},"page":"364-370","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":20,"title":["The HDL mimetic CER\u2010001 remodels plasma lipoproteins and reduces kidney lipid deposits in inherited lecithin:cholesterol acyltransferase deficiency"],"prefix":"10.1111","volume":"291","author":[{"given":"Chiara","family":"Pavanello","sequence":"first","affiliation":[{"name":"Dipartimento di Scienze Farmacologiche e Biomolecolari Centro E. Grossi Paoletti Universit\u00e0 degli Studi di Milano  Milan Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marta","family":"Turri","sequence":"additional","affiliation":[{"name":"Dipartimento di Scienze Farmacologiche e Biomolecolari Centro E. Grossi Paoletti Universit\u00e0 degli Studi di Milano  Milan Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Arianna","family":"Strazzella","sequence":"additional","affiliation":[{"name":"Dipartimento di Scienze Farmacologiche e Biomolecolari Centro E. Grossi Paoletti Universit\u00e0 degli Studi di Milano  Milan Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Patrizia","family":"Tulissi","sequence":"additional","affiliation":[{"name":"Unit of Nephrology, Dialysis and Renal Transplantation, S. Maria della Misericordia Hospital Azienda Sanitaria Universitaria Friuli Centrale (ASUFC)  Udine Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stefano","family":"Pizzolitto","sequence":"additional","affiliation":[{"name":"Unit of Pathology, S. Maria della Misericordia Hospital Azienda Sanitaria Universitaria Friuli Centrale (ASUFC)  Udine Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Giovanna","family":"De Maglio","sequence":"additional","affiliation":[{"name":"Unit of Pathology, S. Maria della Misericordia Hospital Azienda Sanitaria Universitaria Friuli Centrale (ASUFC)  Udine Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Riccardo","family":"Nappi","sequence":"additional","affiliation":[{"name":"Unit of Nephrology, Dialysis and Renal Transplantation, S. 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