Article
Longevity-Associated Variant of BPIFB4 Confers Neuroprotection in the STHdh Cell Model of Huntington Disease.
International journal of molecular sciences - 5 Dec 2022
Cattaneo Monica, Maciag Anna, Milella Maria Serena, Ciaglia Elena, Bruno Antonino, Puca Annibale Alessandro
Abstract excerpt
Huntington's disease (HD) is caused by the production of mutant Huntingtin (mHTT), characterized by long polyglutamine repeats with toxic effects. There are currently no clinically validated therapeutic agents that slow or halt HD progression, resulting in a significant clinical unmet need. The striatum-derived STHdh cell line, generated from mHTT knock-in mouse embryos (STHdhQ111/Q111), represents a useful model...
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