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    Area of Science:

    • Neuroscience
    • Genetics
    • Cell Biology

    Background:

    • Frontotemporal Dementia (FTD) is a neurodegenerative disorder often caused by C9orf72 repeat expansions.
    • Current FTD models have limitations in capturing dynamic disease processes.
    • Understanding C9orf72's role in FTD pathogenesis is crucial.

    Purpose of the Study:

    • To investigate FTD hallmarks in undifferentiated patient-derived induced pluripotent stem cells (iPSCs).
    • To explore the role of lysosome dysfunction in FTD.
    • To identify novel therapeutic targets for FTD.

    Main Methods:

    • Utilized patient-derived iPSCs without neuronal differentiation.
    • Assessed lysosomal function (pH, cathepsin activity).
    • Analyzed TDP-43 proteinopathy and TFEB levels.
    • Performed RNA-sequencing to identify dysregulated transcripts.
    • Confirmed protein expression changes for key genes.

    Main Results:

    • Undifferentiated FTD iPSCs exhibit increased lysosome pH, decreased cathepsin activity, TDP-43 proteinopathy, and increased nuclear TFEB.
    • Reducing lysosome pH ameliorated TDP-43 proteinopathy in FTD iPSCs.
    • RNA-seq revealed dysregulated genes impacting calcium signaling, cell death, and neuronal development.
    • Identified novel protein expression differences in CNTFR, Annexin A2, NANOG, and moesin.

    Conclusions:

    • Undifferentiated FTD iPSCs serve as a robust model for studying cellular pathology.
    • Lysosome dysfunction is a critical factor in C9orf72-linked FTD.
    • FTD iPSCs offer a platform for drug screening and therapeutic development.