{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,2]],"date-time":"2026-06-02T07:30:14Z","timestamp":1780385414879,"version":"3.54.1"},"reference-count":30,"publisher":"The Company of Biologists","license":[{"start":{"date-parts":[[2021,5,18]],"date-time":"2021-05-18T00:00:00Z","timestamp":1621296000000},"content-version":"am","delay-in-days":868,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0"}],"funder":[{"name":"Rare Disease Consortium","award":["MU379777\/ 1-1"],"award-info":[{"award-number":["MU379777\/ 1-1"]}]},{"name":"Rare Disease Consortium","award":["SCHI 587\/11-1"],"award-info":[{"award-number":["SCHI 587\/11-1"]}]},{"DOI":"10.13039\/501100002347","name":"Bundesministerium f\u00fcr Bildung und Forschung","doi-asserted-by":"publisher","award":["01GM1518D"],"award-info":[{"award-number":["01GM1518D"]}],"id":[{"id":"10.13039\/501100002347","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000057","name":"National Institute of General Medical Sciences","doi-asserted-by":"publisher","award":["P20GM104318"],"award-info":[{"award-number":["P20GM104318"]}],"id":[{"id":"10.13039\/100000057","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["P20GM0103423"],"award-info":[{"award-number":["P20GM0103423"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"abstract":"<jats:p>Different glomerular diseases that affect podocyte homeostasis can clinically present as nephrotic syndrome with massive proteinuria, hypoalbuminemia, hyperlipidemia and edema. Up to now no drugs that specifically target the actin cytoskeleton of podocytes are on the market and model systems for library screenings to develop anti-proteinuric drugs are of high interest. We developed a standardized proteinuria model in zebrafish using puromycin aminonucleoside (PAN) via treatment in the fish water to allow for further drug testing to develop anti-proteinuric drugs for the treatment of glomerular diseases. We noticed that fish that carry the nacre-mutation show a significantly higher susceptibility for the disruption of the glomerular filtration barrier following PAN treatment, which results in a more pronounced proteinuria phenotype. Nacre zebrafish inherit a mutation yielding a truncated version of microphthalmia-associated transcription factor\/ melanogenesis associated transcription factor (mitf). We hypothesized that the nacre mutation may lead to reduced formin expression and defects in cytoskeletal rearrangement. Based on the observations in zebrafish, we carried out a PAN treatment on cultured human podocytes after knockdown with MITF siRNA causing a rearrangement of the actin cytoskeleton.<\/jats:p>","DOI":"10.1242\/bio.040253","type":"journal-article","created":{"date-parts":[[2019,2,4]],"date-time":"2019-02-04T17:15:41Z","timestamp":1549300541000},"source":"Crossref","is-referenced-by-count":12,"title":["Mutation of microphthalmia-associated transcription factor (mitf) in zebrafish sensitizes for glomerulopathy"],"prefix":"10.1242","author":[{"given":"Janina","family":"M\u00fcller-Deile","sequence":"first","affiliation":[{"name":"Department of Nephrology, Hannover Medical School, Hannover, Germany"},{"name":"Department of Nephrology and Hypertension, University of Erlangen-Nurnberg, Erlangen, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2693-5712","authenticated-orcid":false,"given":"Heiko","family":"Schenk","sequence":"additional","affiliation":[{"name":"Department of Nephrology, Hannover Medical School, Hannover, Germany"},{"name":"Mount Desert Island Biological Laboratory, Salisbury Cove, ME, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Philipp","family":"Niggemann","sequence":"additional","affiliation":[{"name":"Department of Nephrology, Hannover Medical School, Hannover, Germany"},{"name":"Mount Desert Island Biological Laboratory, Salisbury Cove, ME, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Patricia","family":"Bola\u00f1os-Palmieri","sequence":"additional","affiliation":[{"name":"Department of Nephrology, Hannover Medical School, Hannover, Germany"},{"name":"Department of Nephrology and Hypertension, University of Erlangen-Nurnberg, Erlangen, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6291-5300","authenticated-orcid":false,"given":"Beina","family":"Teng","sequence":"additional","affiliation":[{"name":"Department of Nephrology, Hannover Medical School, Hannover, Germany"},{"name":"Department of Nephrology and Hypertension, University of Erlangen-Nurnberg, Erlangen, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alysha","family":"Higgs","sequence":"additional","affiliation":[{"name":"Mount Desert Island Biological Laboratory, Salisbury Cove, ME, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9416-764X","authenticated-orcid":false,"given":"Lynne","family":"Staggs","sequence":"additional","affiliation":[{"name":"Mount Desert Island Biological Laboratory, Salisbury Cove, ME, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8361-6446","authenticated-orcid":false,"given":"Hermann","family":"Haller","sequence":"additional","affiliation":[{"name":"Department of Nephrology, Hannover Medical School, Hannover, Germany"},{"name":"Mount Desert Island Biological Laboratory, Salisbury Cove, ME, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Patricia","family":"Schroder","sequence":"additional","affiliation":[{"name":"Mount Desert Island Biological Laboratory, Salisbury Cove, ME, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8414-1470","authenticated-orcid":false,"given":"Mario","family":"Schiffer","sequence":"additional","affiliation":[{"name":"Department of Nephrology, Hannover Medical School, Hannover, Germany"},{"name":"Department of Nephrology and Hypertension, University of Erlangen-Nurnberg, Erlangen, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"237","published-online":{"date-parts":[[2019,1,1]]},"reference":[{"key":"2021042423393764800_BIO040253C1","doi-asserted-by":"publisher","first-page":"e12626","DOI":"10.1371\/journal.pone.0012626","article-title":"Cofilin-1 inactivation leads to proteinuria--studies in zebrafish, mice and humans","volume":"5","author":"Ashworth","year":"2010","journal-title":"PLoS ONE"},{"key":"2021042423393764800_BIO040253C2","doi-asserted-by":"publisher","first-page":"316","DOI":"10.1038\/ki.2012.349","article-title":"Mutations in the INF2 gene account for a significant proportion of familial but not sporadic focal and segmental glomerulosclerosis","volume":"83","author":"Barua","year":"2013","journal-title":"Kidney Int."},{"key":"2021042423393764800_BIO040253C3","doi-asserted-by":"publisher","first-page":"72","DOI":"10.1038\/ng.505","article-title":"Mutations in the formin gene INF2 cause focal segmental glomerulosclerosis","volume":"42","author":"Brown","year":"2010","journal-title":"Nat. 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