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Mutations in peroxisomal biogenesis proteins cause Zellweger spectrum disorders (ZSD), rare multisystem disorders often associated with neurological phenotypes. Unlike other peroxisome biogenesis proteins, PEX11\u03b2 regulates peroxisomal fission, and PEX11\u03b2 mutations result in milder metabolic phenotypes but persistent neurodevelopmental abnormalities, suggesting a role for PEX11\u03b2 in neurodevelopment. To model PEX11\u03b2 deficiency during human neurogenesis, we generated PEX11\u03b2 knockout human iPSCs and differentiated them into neural progenitors and neural rosettes. PEX11\u03b2 loss caused elongated peroxisomal morphology, reduced fission, and impaired recruitment of fission proteins, without affecting mitochondrial morphology or respiration. Elongated peroxisomal morphology was independent of the peroxisome-endoplasmic reticulum tether. Lipidomic analysis revealed reduced ether-linked phospholipids in PEX11\u03b2-deficient neural progenitors, suggesting impaired peroxisomal function. Finally, PEX11\u03b2 deficiency led to increased neural rosette lumen size and neural progenitor number.<\/jats:p>","DOI":"10.64898\/2026.02.13.705842","type":"posted-content","created":{"date-parts":[[2026,2,16]],"date-time":"2026-02-16T15:20:13Z","timestamp":1771255213000},"source":"Crossref","is-referenced-by-count":0,"title":["Modeling the cell biology of PEX11\u03b2 deficiency during human neurogenesis"],"prefix":"10.64898","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-3682-3469","authenticated-orcid":false,"given":"C","family":"Bodnya","sequence":"first","affiliation":[{"name":"Department of Cell and Developmental Biology, Vanderbilt University, Nashville, TN, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"RP","family":"Theart","sequence":"additional","affiliation":[{"name":"Department of Electrical and Electronic Engineering, Stellenbosch University, Stellenbosch 7602, South Africa"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1201-1394","authenticated-orcid":false,"given":"V","family":"Gama","sequence":"additional","affiliation":[{"name":"Department of Cell and Developmental Biology, Vanderbilt University, Nashville, TN, USA"},{"name":"Vanderbilt Brain Institute, Vanderbilt University, Nashville, TN, USA"},{"name":"Vanderbilt Center for Stem Cell Biology, Vanderbilt University, Nashville, TN, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"54368","reference":[{"key":"2026021816150507000_2026.02.13.705842v1.1","doi-asserted-by":"publisher","DOI":"10.1002\/jimd.12582"},{"key":"2026021816150507000_2026.02.13.705842v1.2","doi-asserted-by":"publisher","DOI":"10.1038\/cdd.2016.36"},{"key":"2026021816150507000_2026.02.13.705842v1.3","doi-asserted-by":"publisher","DOI":"10.1242\/dmm.007708"},{"key":"2026021816150507000_2026.02.13.705842v1.4","doi-asserted-by":"publisher","DOI":"10.1126\/science.aah4701"},{"key":"2026021816150507000_2026.02.13.705842v1.5","doi-asserted-by":"publisher","DOI":"10.1101\/2024.08.23.609462"},{"key":"2026021816150507000_2026.02.13.705842v1.6","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0053424"},{"key":"2026021816150507000_2026.02.13.705842v1.7","doi-asserted-by":"publisher","DOI":"10.1002\/glia.21027"},{"key":"2026021816150507000_2026.02.13.705842v1.8","doi-asserted-by":"publisher","DOI":"10.1016\/j.bbalip.2003.12.010"},{"key":"2026021816150507000_2026.02.13.705842v1.9","doi-asserted-by":"publisher","DOI":"10.1074\/mcp.M114.044305"},{"key":"2026021816150507000_2026.02.13.705842v1.10","doi-asserted-by":"publisher","DOI":"10.1038\/nbt.1529"},{"key":"2026021816150507000_2026.02.13.705842v1.11","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0313445"},{"key":"2026021816150507000_2026.02.13.705842v1.12","doi-asserted-by":"publisher","DOI":"10.1083\/jcb.201607055"},{"key":"2026021816150507000_2026.02.13.705842v1.13","doi-asserted-by":"publisher","DOI":"10.1038\/s42003-020-01442-x"},{"key":"2026021816150507000_2026.02.13.705842v1.14","doi-asserted-by":"publisher","DOI":"10.1242\/jcs.062109"},{"key":"2026021816150507000_2026.02.13.705842v1.15","doi-asserted-by":"publisher","DOI":"10.4331\/wjbc.v6.i4.301"},{"key":"2026021816150507000_2026.02.13.705842v1.16","doi-asserted-by":"publisher","DOI":"10.1136\/jmedgenet-2012-100778"},{"key":"2026021816150507000_2026.02.13.705842v1.17","doi-asserted-by":"publisher","DOI":"10.1101\/gad.1616208"},{"key":"2026021816150507000_2026.02.13.705842v1.18","doi-asserted-by":"publisher","DOI":"10.1016\/j.neuroscience.2015.11.005"},{"key":"2026021816150507000_2026.02.13.705842v1.19","doi-asserted-by":"publisher","DOI":"10.1194\/jlr.R900009-JLR200"},{"key":"2026021816150507000_2026.02.13.705842v1.20","doi-asserted-by":"crossref","first-page":"142","DOI":"10.1016\/0005-2760(87)90180-9","article-title":"Significance of catalase in peroxisomal fatty acyl-CoA beta-oxidation","volume":"921","year":"1987","journal-title":"Biochim. 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