Publications by authors named "Ilaria Campus"
hESC-derived striatal progenitors grafted into a Huntington's disease rat model support long-term functional motor recovery by differentiating, self-organizing and connecting into the lesioned striatum.
Roberta Schellino Dario Besusso Roberta Parolisi Gabriela B Gómez-González Sveva Dallere Ilaria Campus
Stem Cell Res Ther
July 2023
Article Synopsis
- * In a study, hESC-derived striatal progenitors were implanted in rats with HD-like lesions, showing survival and integration into the brain, while also enhancing motor skills over six months.
- * Environmental enrichment (EE) helped improve the differentiation of grafted cells and their integration with the host brain but didn’t significantly enhance overall task performance when used alongside grafting.
Article Synopsis
- Researchers have developed a protocol for engineering striatal medium spiny neurons (MSNs) from human pluripotent stem cells (PSCs), which could be useful for understanding and treating neurological diseases, particularly Huntington's disease (HD).
- The protocol achieves high reproducibility and generates functional D1- and D2-MSNs in just 25 days by carefully modulating cell density and specific morphogens.
- Single-cell RNA sequencing confirms that these engineered cells resemble natural fetal MSNs in terms of gene expression and development, and adjustments to the midkine pathway can enhance MSN production.
Article Synopsis
- RUES2 cell lines are the first isogenic human embryonic stem cells with varying CAG lengths in the HTT gene, but their potential for neuronal differentiation hasn't been fully assessed.
- During differentiation, RUES2 shows a preference for medial ganglionic eminence (MGE), and HD-RUES2 cells demonstrate abnormal MGE gene expression and recognizable Huntington's Disease (HD) characteristics.
- The study identifies the transcription factor SP1 as a possible harmful partner to mutated HTT, indicating that mutated HTT could negatively impact early neuronal development through its interaction with SP1.
Article Synopsis
- GSX2 is a transcription factor crucial for developing specific brain regions, particularly the corpus striatum, which is involved in movement and coordination.
- Medium spiny neurons (MSNs), the main type of cells in the striatum, are significantly impacted in Huntington disease, a neurodegenerative disorder.
- Researchers used CRISPR technology to create a special human embryonic stem cell line with a GSX2 reporter, allowing them to observe striatal cell development and focus on cells that are set to become MSNs.
Article Synopsis
- * Researchers explored using human embryonic stem cells to replace damaged cells in HD, and successfully demonstrated that these cells could integrate into the brain and form connections in a rat model.
- * Their findings also showed that these transplanted cells improved the rats' sensory-motor tasks for up to two months, highlighting a promising therapeutic potential for this treatment method.
UV-induced photoproducts are responsible for the pathological effects of sunlight. Mutations in nucleotide excision repair (NER) cause severe pathologies characterized by sunlight sensitivity, coupled to elevated predisposition to cancer and/or neurological dysfunctions. We have previously shown that in UV-irradiated non-cycling cells, only a particular subset of lesions activates the DNA damage response (DDR), and this requires NER and EXO1 activities.
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