A model of human neural networks reveals NPTX2 pathology in ALS and FTLD.

ABSTRACT: Human cellular models of neurodegeneration require reproducibility and longevity, which is necessary for simulating age-dependent diseases. Such systems are particularly needed for TDP-43 proteinopathies¹, which involve human-specific mechanisms^2-5 that cannot be directly studied in animal models. Here, to explore the emergence and consequences of TDP-43 pathologies, we generated induced pluripotent stem cell-derived, colony morphology neural stem cells (iCoMoNSCs) via manual selection of neural precursors⁶. Single-cell transcriptomics and comparison to independent neural stem cells⁷ showed that iCoMoNSCs are uniquely homogenous and self-renewing. Differentiated iCoMoNSCs formed a self-organized multicellular system consisting of synaptically con

SUBMITTER: Hruska-Plochan M 

PROVIDER: S-EPMC10901740 | biostudies-literature | 2024 Feb

REPOSITORIES: biostudies-literature