Mesenchymal Cell Replacement Corrects Thymic Hypoplasia in Murine Models of 22q11.2 Deletion Syndrome

    September 2022 in “ Journal of Clinical Investigation
    Pratibha Bhalla, Qiumei Du, Ashwani Kumar, Chao Xing, Angela Moses, Igor Dozmorov, Christian Wysocki, Ondine Cleaver, Timothy J. Pirolli, M. Louise Markert, M. Teresa de la Morena, Antonio Baldini, Nicolai S. C. van Oers
    TLDR Replacing defective mesenchymal cells with normal ones fixes thymic growth issues in 22q11.2DS mouse models.
    The study explored the role of mesenchymal cells in thymic hypoplasia in mouse models of 22q11.2 deletion syndrome (22q11.2DS). Researchers found that replacing defective mesenchymal cells with normal ones restored thymic growth, highlighting their crucial role in thymus development. Increased collagen deposition and altered ECM protein expression were identified as contributing factors to hypoplasia. Treatment with minoxidil improved thymic tissue expansion by reducing collagen cross-linking. The findings suggested that mesenchymal cell replacement could be a potential strategy for regenerating thymuses in patients with thymic aplasia or those who have undergone thymectomies, offering a novel approach for thymus organoid technologies.
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