{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,23]],"date-time":"2026-09-23T19:35:01Z","timestamp":1790192101512,"version":"4.1.0"},"reference-count":77,"publisher":"MDPI AG","issue":"8","license":[{"start":{"date-parts":[[2022,4,7]],"date-time":"2022-04-07T00:00:00Z","timestamp":1649289600000},"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":["SCHE 2092\/1-2"],"award-info":[{"award-number":["SCHE 2092\/1-2"]}],"id":[{"id":"10.13039\/501100001659","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001659","name":"Deutsche Forschungsgemeinschaft","doi-asserted-by":"publisher","award":["SCHE 2092\/3-1"],"award-info":[{"award-number":["SCHE 2092\/3-1"]}],"id":[{"id":"10.13039\/501100001659","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001659","name":"Deutsche Forschungsgemeinschaft","doi-asserted-by":"publisher","award":["SCHE 2092\/4-1 (RP9, CP2, CP3)"],"award-info":[{"award-number":["SCHE 2092\/4-1 (RP9, CP2, CP3)"]}],"id":[{"id":"10.13039\/501100001659","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001659","name":"Deutsche Forschungsgemeinschaft","doi-asserted-by":"publisher","award":["CRU329"],"award-info":[{"award-number":["CRU329"]}],"id":[{"id":"10.13039\/501100001659","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001659","name":"Deutsche Forschungsgemeinschaft","doi-asserted-by":"publisher","award":["Project-ID 43198400 \u2013 SFB1453"],"award-info":[{"award-number":["Project-ID 43198400 \u2013 SFB1453"]}],"id":[{"id":"10.13039\/501100001659","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100003042","name":"Else Kr\u00f6ner-Fresenius-Stiftung","doi-asserted-by":"publisher","award":["EKFS A_09"],"award-info":[{"award-number":["EKFS A_09"]}],"id":[{"id":"10.13039\/501100003042","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Freiburg Institute for Advanced Studies (FRIAS)","award":["MatrixCode research group"],"award-info":[{"award-number":["MatrixCode research group"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Cells"],"abstract":"<jats:p>Steroid-resistant nephrotic syndrome (SRNS) frequently leads to end-stage renal disease, ultimately requiring kidney replacement therapies. SRNS is often caused by hereditary monogenic mutations, specifically affecting specialized epithelial cells (podocytes) of the glomerular filtration barrier. Mutations in several components of the nuclear pore complex, including NUP133 and NUP107, have been recently identified to cause hereditary SRNS. However, underlying pathomechanisms, eliciting podocyte-specific manifestations of these nucleoporopathies, remained largely elusive. Here, we generated an in vitro model of NUP133-linked nucleoporopathies using CRISPR\/Cas9-mediated genome editing in human podocytes. Transcriptome, nuclear pore assembly, and cytoskeleton regulation of NUP133 loss-of-function, mutant, and wild-type podocytes were analyzed. Loss of NUP133 translated into a disruption of the nuclear pore, alterations of the podocyte-specific transcriptome, and impaired cellular protrusion generation. Surprisingly, comparative analysis of the described SRNS-related NUP133 mutations revealed only mild defects. Am impaired protein interaction in the Y-complex and decrease of NUP133 protein levels might be the primary and unifying consequence of mutant variants, leading to a partial loss-of-function phenotype and disease manifestation in susceptible cell types, such as podocytes.<\/jats:p>","DOI":"10.3390\/cells11081259","type":"journal-article","created":{"date-parts":[[2022,4,7]],"date-time":"2022-04-07T13:39:51Z","timestamp":1649338791000},"page":"1259","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":21,"title":["NUP133 Controls Nuclear Pore Assembly, Transcriptome Composition, and Cytoskeleton Regulation in Podocytes"],"prefix":"10.3390","volume":"11","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-6658-9159","authenticated-orcid":false,"given":"Manuel","family":"Rogg","sequence":"first","affiliation":[{"name":"Institute of Surgical Pathology, Faculty of Medicine, Medical Center-University of Freiburg, 79106 Freiburg, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jasmin I.","family":"Maier","sequence":"additional","affiliation":[{"name":"Institute of Surgical Pathology, Faculty of Medicine, Medical Center-University of Freiburg, 79106 Freiburg, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Markus","family":"Ehle","sequence":"additional","affiliation":[{"name":"Institute of Surgical Pathology, Faculty of Medicine, Medical Center-University of Freiburg, 79106 Freiburg, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alena","family":"Sammarco","sequence":"additional","affiliation":[{"name":"Institute of Surgical Pathology, Faculty of Medicine, Medical Center-University of Freiburg, 79106 Freiburg, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7678-7653","authenticated-orcid":false,"given":"Oliver","family":"Schilling","sequence":"additional","affiliation":[{"name":"Institute of Surgical Pathology, Faculty of Medicine, Medical Center-University of Freiburg, 79106 Freiburg, Germany"},{"name":"Freiburg Institute for Advanced Studies (FRIAS), University of Freiburg, 79106 Freiburg, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Martin","family":"Werner","sequence":"additional","affiliation":[{"name":"Institute of Surgical Pathology, Faculty of Medicine, Medical Center-University of Freiburg, 79106 Freiburg, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christoph","family":"Schell","sequence":"additional","affiliation":[{"name":"Institute of Surgical Pathology, Faculty of Medicine, Medical Center-University of Freiburg, 79106 Freiburg, Germany"},{"name":"Freiburg Institute for Advanced Studies (FRIAS), University of Freiburg, 79106 Freiburg, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2022,4,7]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"1437","DOI":"10.1056\/NEJMra1808786","article-title":"Insights into glomerular filtration and albuminuria","volume":"384","author":"Benzing","year":"2021","journal-title":"N. 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