{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,7]],"date-time":"2026-08-07T20:18:39Z","timestamp":1786133919626,"version":"build-2736575974"},"reference-count":71,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"1","content-domain":{"domain":["lww.com","ovid.com"],"crossmark-restriction":true},"short-container-title":["JASN"],"published-print":{"date-parts":[[2026,1]]},"abstract":"<jats:p>\n                    Alport syndrome is a progressive, hereditary disorder of basement membranes caused by variants in genes encoding the\n                    <jats:italic toggle=\"yes\">\u03b1<\/jats:italic>\n                    3,\n                    <jats:italic toggle=\"yes\">\u03b1<\/jats:italic>\n                    4, or\n                    <jats:italic toggle=\"yes\">\u03b1<\/jats:italic>\n                    5 chains of type IV collagen (\n                    <jats:italic toggle=\"yes\">COL4A3, COL4A4<\/jats:italic>\n                    , and\n                    <jats:italic toggle=\"yes\">COL4A5<\/jats:italic>\n                    ) leading to glomerulopathy, kidney failure, hearing loss, and eye abnormalities. The absence or dysfunction of the\n                    <jats:italic toggle=\"yes\">\u03b1<\/jats:italic>\n                    3-\n                    <jats:italic toggle=\"yes\">\u03b1<\/jats:italic>\n                    4-\n                    <jats:italic toggle=\"yes\">\u03b1<\/jats:italic>\n                    5 (IV) heterotrimer triggers multiple compensatory and detrimental pathways within all layers of the glomerular filtration barrier. Developing a therapeutic strategy for patients with Alport syndrome depends on understanding these mechanisms of disease progression that are predominant at different times throughout the disease course. These strategies may include reconstitution of the\n                    <jats:italic toggle=\"yes\">\u03b1<\/jats:italic>\n                    3-\n                    <jats:italic toggle=\"yes\">\u03b1<\/jats:italic>\n                    4-\n                    <jats:italic toggle=\"yes\">\u03b1<\/jats:italic>\n                    5 (IV) network in the glomerular basement membrane, reducing biomechanical strain and glomerular hyperfiltration, chaperone therapy, blocking aberrant signaling between the glomerular basement membrane and podocytes, reducing endothelial cell injury, reducing inflammation, and blocking fibrosis pathways.\n                  <\/jats:p>","DOI":"10.1681\/asn.0000000897","type":"journal-article","created":{"date-parts":[[2025,9,12]],"date-time":"2025-09-12T16:00:30Z","timestamp":1757692830000},"page":"172-179","update-policy":"https:\/\/doi.org\/10.1097\/lww.0000000000001000","source":"Crossref","is-referenced-by-count":4,"title":["Treatment Approaches for Alport Syndrome"],"prefix":"10.1681","volume":"37","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-2494-3970","authenticated-orcid":false,"given":"Michelle N.","family":"Rheault","sequence":"first","affiliation":[{"name":"Division of Nephrology, Department of Pediatrics, University of Minnesota Medical School, Minneapolis, Minnesota"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"276","published-online":{"date-parts":[[2025,9,12]]},"reference":[{"issue":"4","key":"B1-20260717","doi-asserted-by":"crossref","first-page":"1948","DOI":"10.1172\/JCI118241","article-title":"Comparative distribution of the alpha 1(IV), alpha 5(IV), and alpha 6(IV) collagen chains in normal human adult and fetal tissues and in kidneys from X-linked Alport syndrome patients","volume":"96","author":"Peissel","year":"1995","journal-title":"J Clin Invest."},{"issue":"25","key":"B2-20260717","doi-asserted-by":"crossref","first-page":"2543","DOI":"10.1056\/NEJMra022296","article-title":"Alport\u2019s syndrome, Goodpasture\u2019s syndrome, and type IV collagen","volume":"348","author":"Hudson","year":"2003","journal-title":"N Engl J Med."},{"issue":"2","key":"B3-20260717","doi-asserted-by":"crossref","first-page":"112","DOI":"10.1038\/s41581-020-0329-y","article-title":"Complexities of the glomerular basement membrane","volume":"17","author":"Naylor","year":"2021","journal-title":"Nat Rev Nephrol."},{"issue":"1","key":"B4-20260717","doi-asserted-by":"crossref","first-page":"13","DOI":"10.1097\/00041552-199801000-00003","article-title":"Developmental biology of glomerular basement membrane components","volume":"7","author":"Miner","year":"1998","journal-title":"Curr Opin Nephrol Hypertens."},{"issue":"3","key":"B5-20260717","doi-asserted-by":"crossref","first-page":"879","DOI":"10.1083\/jcb.127.3.879","article-title":"Collagen IV alpha 3, alpha 4, and alpha 5 chains in rodent basal laminae: sequence, distribution, association with laminins, and developmental switches","volume":"127","author":"Miner","year":"1994","journal-title":"J Cell Biol."},{"issue":"10","key":"B6-20260717","doi-asserted-by":"crossref","first-page":"2470","DOI":"10.1172\/JCI119431","article-title":"Isoform switching of type IV collagen is developmentally arrested in X-linked Alport syndrome leading to increased susceptibility of renal basement membranes to endoproteolysis","volume":"99","author":"Kalluri","year":"1997","journal-title":"J Clin Invest."},{"issue":"7","key":"B7-20260717","doi-asserted-by":"crossref","first-page":"1471","DOI":"10.1681\/ASN.2008101086","article-title":"Cellular origins of type IV collagen networks in developing glomeruli","volume":"20","author":"Abrahamson","year":"2009","journal-title":"J Am Soc Nephrol."},{"key":"B8-20260717","doi-asserted-by":"crossref","first-page":"e01149","DOI":"10.7554\/eLife.01149","article-title":"Nanoscale protein architecture of the kidney glomerular basement membrane","volume":"2","author":"Suleiman","year":"2013","journal-title":"eLife."},{"issue":"15","key":"B9-20260717","doi-asserted-by":"crossref","first-page":"8767","DOI":"10.1074\/jbc.273.15.8767","article-title":"Glomerular basement membrane. Identification of a novel disulfide-cross-linked network of alpha3, alpha4, and alpha5 chains of type IV collagen and its implications for the pathogenesis of Alport syndrome","volume":"273","author":"Gunwar","year":"1998","journal-title":"J Biol Chem."},{"issue":"9","key":"B10-20260717","doi-asserted-by":"crossref","first-page":"e0161802","DOI":"10.1371\/journal.pone.0161802","article-title":"X-linked and autosomal recessive Alport syndrome: pathogenic variant features and further genotype-phenotype correlations","volume":"11","author":"Savige","year":"2016","journal-title":"PLoS One."},{"issue":"6","key":"B11-20260717","doi-asserted-by":"crossref","first-page":"1605","DOI":"10.1016\/j.kint.2020.06.038","article-title":"Genotype-phenotype correlations influence the response to angiotensin-targeting drugs in Japanese patients with male X-linked Alport syndrome","volume":"98","author":"Yamamura","year":"2020","journal-title":"Kidney Int."},{"issue":"4","key":"B12-20260717","doi-asserted-by":"crossref","first-page":"688","DOI":"10.1093\/ndt\/gfae182","article-title":"Genotype and X-chromosome inactivation are associated with disease severity in females with X-linked Alport syndrome","volume":"40","author":"Suzuki","year":"2025","journal-title":"Nephrol Dial Transplant."},{"issue":"10","key":"B13-20260717","doi-asserted-by":"crossref","first-page":"1510","DOI":"10.34067\/KID.0000000000000547","article-title":"Clinical, pathological, and genetic characteristics of patients with digenic Alport syndrome","volume":"5","author":"Inoki","year":"2024","journal-title":"Kidney360."},{"issue":"7","key":"B14-20260717","doi-asserted-by":"crossref","first-page":"1175","DOI":"10.1007\/s00467-018-3985-4","article-title":"Expert consensus guidelines for the genetic diagnosis of Alport syndrome","volume":"34","author":"Savige","year":"2019","journal-title":"Pediatr Nephrol."},{"issue":"4","key":"B15-20260717","doi-asserted-by":"crossref","first-page":"1633","DOI":"10.1016\/S0002-9440(10)63520-1","article-title":"A human-mouse chimera of the alpha3alpha4alpha5(IV) collagen protomer rescues the renal phenotype in Col4a3\u2212\/\u2212 Alport mice","volume":"163","author":"Heidet","year":"2003","journal-title":"Am J Pathol."},{"issue":"4","key":"B16-20260717","doi-asserted-by":"crossref","first-page":"687","DOI":"10.1681\/ASN.2013070798","article-title":"Feasibility of repairing glomerular basement membrane defects in Alport syndrome","volume":"25","author":"Lin","year":"2014","journal-title":"J Am Soc Nephrol."},{"issue":"4","key":"B17-20260717","doi-asserted-by":"crossref","first-page":"480","DOI":"10.1038\/s41431-019-0537-8","article-title":"New frontiers to cure Alport syndrome: COL4A3 and COL4A5 gene editing in podocyte-lineage cells","volume":"28","author":"Daga","year":"2020","journal-title":"Eur J Hum Genet."},{"issue":"11","key":"B18-20260717","doi-asserted-by":"crossref","first-page":"663","DOI":"10.1038\/s41581-018-0047-x","article-title":"Precision gene editing technology and applications in nephrology","volume":"14","author":"WareJoncas","year":"2018","journal-title":"Nat Rev Nephrol."},{"issue":"1","key":"B19-20260717","doi-asserted-by":"crossref","first-page":"10728","DOI":"10.1038\/s41467-024-54475-9","article-title":"Enhancing gene transfer to renal tubules and podocytes by context-dependent selection of AAV capsids","volume":"15","author":"Furusho","year":"2024","journal-title":"Nat Commun."},{"issue":"2","key":"B20-20260717","doi-asserted-by":"crossref","first-page":"249","DOI":"10.1038\/s41591-018-0326-x","article-title":"Identification of preexisting adaptive immunity to Cas9 proteins in humans","volume":"25","author":"Charlesworth","year":"2019","journal-title":"Nat Med."},{"issue":"9791","key":"B21-20260717","doi-asserted-by":"crossref","first-page":"595","DOI":"10.1016\/S0140-6736(11)60756-3","article-title":"Exon skipping and dystrophin restoration in patients with Duchenne muscular dystrophy after systemic phosphorodiamidate morpholino oligomer treatment: an open-label, phase 2, dose-escalation study","volume":"378","author":"Cirak","year":"2011","journal-title":"Lancet."},{"issue":"1","key":"B22-20260717","doi-asserted-by":"crossref","first-page":"2777","DOI":"10.1038\/s41467-020-16605-x","article-title":"Development of an exon skipping therapy for X-linked Alport syndrome with truncating variants in COL4A5","volume":"11","author":"Yamamura","year":"2020","journal-title":"Nat Commun."},{"issue":"2","key":"B23-20260717","doi-asserted-by":"crossref","first-page":"264","DOI":"10.1124\/jpet.120.265595","article-title":"ELX-02 generates protein via premature stop codon read-through without inducing native stop codon read-through proteins","volume":"374","author":"Crawford","year":"2020","journal-title":"J Pharmacol Exp Ther."},{"issue":"12","key":"B24-20260717","doi-asserted-by":"crossref","first-page":"e0223954","DOI":"10.1371\/journal.pone.0223954","article-title":"The novel aminoglycoside, ELX-02, permits CTNS W138X translational read-through and restores lysosomal cystine efflux in cystinosis","volume":"14","author":"Brasell","year":"2019","journal-title":"PLoS One."},{"issue":"4","key":"B25-20260717","doi-asserted-by":"crossref","first-page":"102334","DOI":"10.1016\/j.omtn.2024.102334","article-title":"Novel readthrough agent suppresses nonsense mutations and restores functional type VII collagen and laminin 332 in epidermolysis bullosa","volume":"35","author":"Levian","year":"2024","journal-title":"Mol Ther Nucleic Acids."},{"issue":"4","key":"B26-20260717","doi-asserted-by":"crossref","first-page":"649","DOI":"10.1681\/ASN.V114649","article-title":"X-linked Alport syndrome: natural history in 195 families and genotype- phenotype correlations in males","volume":"11","author":"Jais","year":"2000","journal-title":"J Am Soc Nephrol."},{"issue":"5","key":"B27-20260717","doi-asserted-by":"crossref","first-page":"1426","DOI":"10.1681\/ASN.2017060690","article-title":"Laminin-521 protein therapy for glomerular basement membrane and podocyte abnormalities in a model of pierson syndrome","volume":"29","author":"Lin","year":"2018","journal-title":"J Am Soc Nephrol."},{"issue":"9","key":"B28-20260717","doi-asserted-by":"crossref","first-page":"968","DOI":"10.1038\/ki.2009.324","article-title":"Biomechanical strain causes maladaptive gene regulation, contributing to Alport glomerular disease","volume":"76","author":"Meehan","year":"2009","journal-title":"Kidney Int."},{"key":"B29-20260717","doi-asserted-by":"crossref","first-page":"87","DOI":"10.1016\/j.exer.2013.05.008","article-title":"Mechanical response of wild-type and Alport murine lens capsules during osmotic swelling","volume":"113","author":"Gyoneva","year":"2013","journal-title":"Exp Eye Res."},{"issue":"2","key":"B30-20260717","doi-asserted-by":"crossref","first-page":"438","DOI":"10.1046\/j.1523-1755.2003.00779.x","article-title":"Preemptive ramipril therapy delays renal failure and reduces renal fibrosis in COL4A3-knockout mice with Alport syndrome","volume":"63","author":"Gross","year":"2003","journal-title":"Kidney Int."},{"issue":"5","key":"B31-20260717","doi-asserted-by":"crossref","first-page":"494","DOI":"10.1038\/ki.2011.407","article-title":"Early angiotensin-converting enzyme inhibition in Alport syndrome delays renal failure and improves life expectancy","volume":"81","author":"Gross","year":"2012","journal-title":"Kidney Int."},{"issue":"11","key":"B32-20260717","doi-asserted-by":"crossref","first-page":"2485","DOI":"10.1093\/ndt\/gfad105","article-title":"Effectiveness of renin-angiotensin-aldosterone system blockers in patients with Alport syndrome: a systematic review and meta-analysis","volume":"38","author":"Zeng","year":"2023","journal-title":"Nephrol Dial Transplant."},{"issue":"6","key":"B33-20260717","doi-asserted-by":"crossref","first-page":"1275","DOI":"10.1016\/j.kint.2019.12.015","article-title":"A multicenter, randomized, placebo-controlled, double-blind phase 3 trial with open-arm comparison indicates safety and efficacy of nephroprotective therapy with ramipril in children with Alport's syndrome","volume":"97","author":"Gross","year":"2020","journal-title":"Kidney Int."},{"issue":"6","key":"B34-20260717","doi-asserted-by":"crossref","first-page":"1091","DOI":"10.1093\/ndt\/gfae265","article-title":"Diagnosis, management and treatment of the Alport syndrome - 2024 guideline on behalf of ERKNet, ERA and ESPN","volume":"40","author":"Torra","year":"2025","journal-title":"Nephrol Dial Transplant."},{"issue":"3","key":"B35-20260717","doi-asserted-by":"crossref","first-page":"711","DOI":"10.1007\/s00467-020-04819-6","article-title":"Clinical practice recommendations for the diagnosis and management of Alport syndrome in children, adolescents, and young adults-an update for 2020","volume":"36","author":"Kashtan","year":"2021","journal-title":"Pediatr Nephrol."},{"issue":"5","key":"B36-20260717","doi-asserted-by":"crossref","first-page":"1360","DOI":"10.1016\/j.ekir.2025.02.036","article-title":"Prospective cohort study in Alport syndrome patients under standard therapy","volume":"10","author":"Gross","year":"2025","journal-title":"Kidney Int Rep."},{"issue":"12","key":"B37-20260717","doi-asserted-by":"crossref","first-page":"1763","DOI":"10.2215\/CJN.02400222","article-title":"Effects of bardoxolone methyl in Alport syndrome","volume":"17","author":"Warady","year":"2022","journal-title":"Clin J Am Soc Nephrol."},{"issue":"6","key":"B38-20260717","doi-asserted-by":"crossref","first-page":"749","DOI":"10.1093\/ckj\/sfy090","article-title":"The potential for improving cardio-renal outcomes by sodium-glucose co-transporter-2 inhibition in people with chronic kidney disease: a rationale for the EMPA-KIDNEY study","volume":"11","author":"Herrington","year":"2018","journal-title":"Clin Kidney J."},{"issue":"2","key":"B39-20260717","doi-asserted-by":"crossref","first-page":"117","DOI":"10.1056\/NEJMoa2204233","article-title":"Empagliflozin in patients with chronic kidney disease","volume":"388","author":"Herrington","year":"2023","journal-title":"N Engl J Med."},{"issue":"1","key":"B40-20260717","doi-asserted-by":"crossref","first-page":"51","DOI":"10.1016\/S2213-8587(23)00322-4","article-title":"Impact of primary kidney disease on the effects of empagliflozin in patients with chronic kidney disease: secondary analyses of the EMPA-KIDNEY trial","volume":"12","author":"Abat","year":"2024","journal-title":"Lancet Diabetes Endocrinol."},{"issue":"7","key":"B41-20260717","doi-asserted-by":"crossref","first-page":"1815","DOI":"10.3390\/cells10071815","article-title":"Sodium-glucose cotransporter-2 inhibitors in patients with hereditary podocytopathies, Alport syndrome, and FSGS: a case series to better plan a large-scale study","volume":"10","author":"Boeckhaus","year":"2021","journal-title":"Cells."},{"issue":"12","key":"B42-20260717","doi-asserted-by":"crossref","first-page":"3490","DOI":"10.1016\/j.ekir.2024.09.014","article-title":"SGLT2-Inhibition in patients with Alport syndrome","volume":"9","author":"Boeckhaus","year":"2024","journal-title":"Kidney Int Rep."},{"issue":"4","key":"B43-20260717","doi-asserted-by":"crossref","first-page":"679","DOI":"10.1093\/ndt\/gfae180","article-title":"Protocol and rationale for a randomized controlled SGLT2 inhibitor trial in paediatric and young adult populations with chronic kidney disease: DOUBLE PRO-TECT Alport","volume":"40","author":"Gross","year":"2025","journal-title":"Nephrol Dial Transplant."},{"issue":"2","key":"B44-20260717","doi-asserted-by":"crossref","first-page":"260","DOI":"10.1681\/ASN.2012121217","article-title":"Evidence for activation of the unfolded protein response in collagen IV nephropathies","volume":"25","author":"Pieri","year":"2014","journal-title":"J Am Soc Nephrol."},{"issue":"4","key":"B45-20260717","doi-asserted-by":"crossref","first-page":"739","DOI":"10.1016\/j.ekir.2017.03.004","article-title":"The chemical chaperone, PBA, reduces ER stress and autophagy and increases collagen IV \u03b15 expression in cultured fibroblasts from men with X-linked Alport syndrome and missense mutations","volume":"2","author":"Wang","year":"2017","journal-title":"Kidney Int Rep."},{"issue":"4","key":"B46-20260717","doi-asserted-by":"crossref","first-page":"609","DOI":"10.1016\/j.kint.2025.05.016","article-title":"Chemical chaperone 4-phenylbutyrate treatment alleviates the kidney phenotype in a mouse model of Alport syndrome with a pathogenic variant in Col4a3","volume":"108","author":"Ioannou","year":"2025","journal-title":"Kidney Int."},{"issue":"7","key":"B47-20260717","doi-asserted-by":"crossref","first-page":"ar62","DOI":"10.1091\/mbc.E21-11-0578","article-title":"Distinct proteostasis states drive pharmacologic chaperone susceptibility for cystic fibrosis transmembrane conductance regulator misfolding mutants","volume":"33","author":"McDonald","year":"2022","journal-title":"Mol Biol Cell."},{"issue":"3","key":"B48-20260717","doi-asserted-by":"crossref","first-page":"157","DOI":"10.1038\/s41581-024-00906-1","article-title":"Integrins in the kidney - beyond the matrix","volume":"21","author":"Bock","year":"2025","journal-title":"Nat Rev Nephrol."},{"issue":"2","key":"B49-20260717","doi-asserted-by":"crossref","first-page":"617","DOI":"10.1016\/S0002-9440(10)63326-3","article-title":"Lack of integrin alpha1beta1 leads to severe glomerulosclerosis after glomerular injury","volume":"165","author":"Chen","year":"2004","journal-title":"Am J Pathol."},{"issue":"5","key":"B50-20260717","doi-asserted-by":"crossref","first-page":"1649","DOI":"10.1016\/S0002-9440(10)64802-X","article-title":"Integrin alpha1beta1 and transforming growth factor-beta1 play distinct roles in Alport glomerular pathogenesis and serve as dual targets for metabolic therapy","volume":"157","author":"Cosgrove","year":"2000","journal-title":"Am J Pathol."},{"key":"B51-20260717","doi-asserted-by":"crossref","first-page":"13","DOI":"10.1016\/j.matbio.2014.01.006","article-title":"Collagen receptors integrin alpha2beta1 and discoidin domain receptor 1 regulate maturation of the glomerular basement membrane and loss of integrin alpha2beta1 delays kidney fibrosis in COL4A3 knockout mice","volume":"34","author":"Rubel","year":"2014","journal-title":"Matrix Biol."},{"issue":"6","key":"B52-20260717","doi-asserted-by":"crossref","first-page":"1027","DOI":"10.1681\/ASN.2011040367","article-title":"Inhibition of integrin \u03b12\u03b21 ameliorates glomerular injury","volume":"23","author":"Borza","year":"2012","journal-title":"J Am Soc Nephrol."},{"issue":"11","key":"B53-20260717","doi-asserted-by":"crossref","first-page":"2648","DOI":"10.1097\/01.ASN.0000032419.13208.0C","article-title":"Discoidin domain receptor 1 controls growth and adhesion of mesangial cells","volume":"13","author":"Curat","year":"2002","journal-title":"J Am Soc Nephrol."},{"key":"B54-20260717","doi-asserted-by":"crossref","first-page":"185","DOI":"10.1016\/j.matbio.2013.12.002","article-title":"Discoidin domain receptors in disease","volume":"34","author":"Borza","year":"2014","journal-title":"Matrix Biol."},{"key":"B55-20260717","doi-asserted-by":"crossref","first-page":"103162","DOI":"10.1016\/j.ebiom.2020.103162","article-title":"Discoidin domain receptor 1 activation links extracellular matrix to podocyte lipotoxicity in Alport syndrome","volume":"63","author":"Kim","year":"2021","journal-title":"EBioMedicine."},{"issue":"5","key":"B56-20260717","doi-asserted-by":"crossref","first-page":"346","DOI":"10.1016\/j.matbio.2010.03.002","article-title":"Loss of collagen-receptor DDR1 delays renal fibrosis in hereditary type IV collagen disease","volume":"29","author":"Gross","year":"2010","journal-title":"Matrix Biol."},{"issue":"1","key":"B57-20260717","doi-asserted-by":"crossref","first-page":"37","DOI":"10.1021\/acschembio.8b00866","article-title":"DNA-encoded library-derived DDR1 inhibitor prevents fibrosis and renal function loss in a genetic mouse model of Alport syndrome","volume":"14","author":"Richter","year":"2019","journal-title":"ACS Chem Biol."},{"issue":"2","key":"B58-20260717","doi-asserted-by":"crossref","first-page":"300","DOI":"10.1016\/j.kint.2016.02.018","article-title":"Endothelin A receptor activation on mesangial cells initiates Alport glomerular disease","volume":"90","author":"Dufek","year":"2016","journal-title":"Kidney Int."},{"issue":"12","key":"B59-20260717","doi-asserted-by":"crossref","first-page":"e0168343","DOI":"10.1371\/journal.pone.0168343","article-title":"X-linked Alport dogs demonstrate mesangial filopodial invasion of the capillary tuft as an early event in glomerular damage","volume":"11","author":"Clark","year":"2016","journal-title":"PLoS One"},{"key":"B60-20260717","doi-asserted-by":"crossref","first-page":"35068","DOI":"10.1038\/srep35068","article-title":"Three-dimensional electron microscopy reveals the evolution of glomerular barrier injury","volume":"6","author":"Randles","year":"2016","journal-title":"Sci Rep."},{"issue":"26","key":"B61-20260717","doi-asserted-by":"crossref","first-page":"2436","DOI":"10.1056\/NEJMoa2308550","article-title":"Sparsentan versus irbesartan in focal segmental glomerulosclerosis","volume":"389","author":"Rheault","year":"2023","journal-title":"N Engl J Med."},{"issue":"10417","key":"B62-20260717","doi-asserted-by":"crossref","first-page":"2077","DOI":"10.1016\/S0140-6736(23)02302-4","article-title":"Efficacy and safety of sparsentan versus irbesartan in patients with IgA nephropathy (PROTECT): 2-year results from a randomised, active-controlled, phase 3 trial","volume":"402","author":"Rovin","year":"2023","journal-title":"Lancet"},{"issue":"3","key":"B63-20260717","doi-asserted-by":"crossref","first-page":"353","DOI":"10.1002\/path.6087","article-title":"Dual inhibition of the endothelin and angiotensin receptor ameliorates renal and inner ear pathologies in Alport mice","volume":"260","author":"Cosgrove","year":"2023","journal-title":"J Pathol."},{"issue":"6","key":"B64-20260717","doi-asserted-by":"crossref","first-page":"460","DOI":"10.1038\/s41577-025-01131-y","article-title":"Kidney immunology from pathophysiology to clinical translation","volume":"25","author":"Kurts","year":"2025","journal-title":"Nat Rev Immunol."},{"issue":"1","key":"B65-20260717","doi-asserted-by":"crossref","first-page":"7053","DOI":"10.1038\/s41598-021-86109-1","article-title":"Metformin ameliorates the severity of experimental Alport syndrome","volume":"11","author":"Omachi","year":"2021","journal-title":"Sci Rep."},{"issue":"3","key":"B66-20260717","doi-asserted-by":"crossref","first-page":"284","DOI":"10.1038\/labinvest.3700715","article-title":"Lymphocytes are dispensable for glomerulonephritis but required for renal interstitial fibrosis in matrix defect-induced Alport renal disease","volume":"88","author":"Lebleu","year":"2008","journal-title":"Lab Invest."},{"issue":"2","key":"B67-20260717","doi-asserted-by":"crossref","first-page":"214","DOI":"10.1093\/ndt\/gfx246","article-title":"STAT3 inhibition attenuates the progressive phenotypes of Alport syndrome mouse model","volume":"33","author":"Yokota","year":"2018","journal-title":"Nephrol Dial Transplant."},{"issue":"4","key":"B68-20260717","doi-asserted-by":"crossref","first-page":"582","DOI":"10.1016\/j.humpath.2009.08.024","article-title":"Interstitial inflammation in Alport syndrome","volume":"41","author":"Jedlicka","year":"2010","journal-title":"Hum Pathol."},{"issue":"4","key":"B69-20260717","doi-asserted-by":"crossref","first-page":"269","DOI":"10.1007\/s004670050451","article-title":"Chronology of renal scarring in males with Alport syndrome","volume":"12","author":"Kashtan","year":"1998","journal-title":"Pediatr Nephrol."},{"issue":"4","key":"B70-20260717","doi-asserted-by":"crossref","first-page":"594","DOI":"10.3390\/cells11040594","article-title":"Anti-microRNA-21 therapy on top of ACE inhibition delays renal failure in Alport syndrome mouse models","volume":"11","author":"Rubel","year":"2022","journal-title":"Cells."},{"issue":"8","key":"B71-20260717","doi-asserted-by":"crossref","first-page":"995","DOI":"10.2215\/CJN.0000000000000458","article-title":"A randomized controlled clinical trial testing effects of lademirsen on Kidney Function decline in adults with Alport Syndrome","volume":"19","author":"Gale","year":"2024","journal-title":"Clin J Am Soc Nephrol."}],"container-title":["Journal of the American Society of Nephrology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.ovid.com\/10.1681\/ASN.0000000897","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,8,7]],"date-time":"2026-08-07T19:42:14Z","timestamp":1786131734000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.ovid.com\/10.1681\/ASN.0000000897"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,9,12]]},"references-count":71,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2026]]}},"URL":"https:\/\/doi.org\/10.1681\/asn.0000000897","relation":{},"ISSN":["1046-6673","1533-3450"],"issn-type":[{"value":"1046-6673","type":"print"},{"value":"1533-3450","type":"electronic"}],"subject":[],"published":{"date-parts":[[2025,9,12]]}}}