{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,16]],"date-time":"2026-04-16T06:40:58Z","timestamp":1776321658450,"version":"3.50.1"},"reference-count":47,"publisher":"MDPI AG","issue":"22","license":[{"start":{"date-parts":[[2020,11,11]],"date-time":"2020-11-11T00:00:00Z","timestamp":1605052800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100001659","name":"Deutsche Forschungsgemeinschaft","doi-asserted-by":"publisher","award":["DFG-GR 4403\/2-1"],"award-info":[{"award-number":["DFG-GR 4403\/2-1"]}],"id":[{"id":"10.13039\/501100001659","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001659","name":"Deutsche Forschungsgemeinschaft","doi-asserted-by":"publisher","award":["DFG-GR 4403\/5-1"],"award-info":[{"award-number":["DFG-GR 4403\/5-1"]}],"id":[{"id":"10.13039\/501100001659","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001659","name":"Deutsche Forschungsgemeinschaft","doi-asserted-by":"publisher","award":["DFG-PA 1844\/3-1"],"award-info":[{"award-number":["DFG-PA 1844\/3-1"]}],"id":[{"id":"10.13039\/501100001659","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["IJMS"],"abstract":"<jats:p>Stargardt macular degeneration is an inherited retinal disease caused by mutations in the ATP-binding cassette subfamily A member 4 (ABCA4) gene. Here, we characterized the complement expression profile in ABCA4\u2212\/\u2212 retinae and aligned these findings with morphological markers of retinal degeneration. We found an enhanced retinal pigment epithelium (RPE) autofluorescence, cell loss in the inner retina of ABCA4\u2212\/\u2212 mice and demonstrated age-related differences in complement expression in various retinal cell types irrespective of the genotype. However, 24-week-old ABCA4\u2212\/\u2212 mice expressed more c3 in the RPE and fewer cfi transcripts in the microglia compared to controls. At the protein level, the decrease of complement inhibitors (complement factor I, CFI) in retinae, as well as an increased C3b\/C3 ratio in the RPE\/choroid and retinae of ABCA4\u2212\/\u2212, mice was confirmed. We showed a corresponding increase of the C3d\/C3 ratio in the serum of ABCA4\u2212\/\u2212 mice, while no changes were observed for CFI. Our findings suggest an overactive complement cascade in the ABCA4\u2212\/\u2212 retinae that possibly contributes to pathological alterations, including microglial activation and neurodegeneration. Overall, this underpins the importance of well-balanced complement homeostasis to maintain retinal integrity.<\/jats:p>","DOI":"10.3390\/ijms21228468","type":"journal-article","created":{"date-parts":[[2020,11,11]],"date-time":"2020-11-11T19:08:28Z","timestamp":1605121708000},"page":"8468","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":10,"title":["Cell-Type-Specific Complement Profiling in the ABCA4\u2212\/\u2212 Mouse Model of Stargardt Disease"],"prefix":"10.3390","volume":"21","author":[{"given":"Yassin","family":"Jabri","sequence":"first","affiliation":[{"name":"Department of Experimental Ophthalmology, Eye Clinic, University Hospital Regensburg, D-93053 Regensburg, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Josef","family":"Biber","sequence":"additional","affiliation":[{"name":"Department of Physiological Genomics, Biomedical Center, Ludwig Maximilians University Munich, D-82152 Planegg-Martinsried, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Nundehui","family":"Diaz-Lezama","sequence":"additional","affiliation":[{"name":"Department of Physiological Genomics, Biomedical Center, Ludwig Maximilians University Munich, D-82152 Planegg-Martinsried, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0338-7530","authenticated-orcid":false,"given":"Antje","family":"Grosche","sequence":"additional","affiliation":[{"name":"Department of Physiological Genomics, Biomedical Center, Ludwig Maximilians University Munich, D-82152 Planegg-Martinsried, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1563-1601","authenticated-orcid":false,"given":"Diana","family":"Pauly","sequence":"additional","affiliation":[{"name":"Experimental Ophthalmology, Philipps-University Marburg, D-35043 Marburg, Germany"},{"name":"Department of Ophthalmology, University Hospital Regensburg, D-93053 Regensburg, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2020,11,11]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"415","DOI":"10.1016\/bs.pmbts.2015.06.008","article-title":"Insights into the Molecular Properties of ABCA4 and Its Role in the Visual Cycle and Stargardt Disease","volume":"134","author":"Molday","year":"2015","journal-title":"Prog. 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