A phenotypic brain organoid atlas and biobank for neurodevelopmental disorders

Summary

Thousands of genes are associated with neurodevelopmental disorders (NDDs), yet mechanisms and targeted treatments remain elusive. To fill these gaps, we present a California Institute of Regenerative Medicine (CIRM)-initiated NDD biobank of 352 publicly available genetically diverse patient-derived induced pluripotent stem cells (iPSCs), along with clinical details, brain imaging, and genomic data, representing four major categories of disease: microcephaly (MIC), polymicrogyria (PMG), epilepsy (EPI), and intellectual disability (ID). From 35 representative patients, we studied over 6,000 brain organoids for histology and single-cell transcriptomics. Compared with an organoid library from ten neurotypicals, patients showed distinct cellular defects linked to underlying clinical disease categories. MIC showed defects in cell survival and excessive TTR+ cells, PMG showed intermediate progenitor cell junction defects, EPI showed excessive astrogliosis, and ID showed excessive generation of TTR+ cells. Our organoid atlas demonstrates both conserved and divergent NDD category-specific phenotypes, bridging genotype and phenotype. This NDD iPSC biobank can support future disease modeling and therapeutic approaches. Copyright © 2025 Elsevier Inc. All rights reserved.

Authors Wang L, Nakamura Y, Li J, Sievert D, Liu Y, Nguyen T, Jetti PS, Thai E, Zhou RY, Weng J, Meave N, Yadavilli M, Howarth R, Camey K, Banka N, Owusu-Hammond C, Barrows C, Kingsmore SF, Zaki MS, Mukamel E, Gleeson JG
Journal Cell stem cell
Publication Date 2025 Dec 4;32(12):1923-1940.e7
PubMed 41187745
PubMed Central PMC13003759
DOI 10.1016/j.stem.2025.10.006

Research Projects

Cell Lines