Publications by authors named "L Cironi"
The development of targeted therapies offers new hope for patients affected by incurable cancer. However, multiple challenges persist, notably in controlling tumor cell plasticity in patients with refractory and metastatic illness. Neuroblastoma (NB) is an aggressive pediatric malignancy originating from defective differentiation of neural crest-derived progenitors with oncogenic activity due to genetic and epigenetic alterations and remains a clinical challenge for high-risk patients.
View Article and Find Full Text PDFCIC-DUX4-rearranged sarcoma (CDS) is a rare and aggressive soft tissue tumor that occurs most frequently in young adults. The key oncogenic driver of this disease is the expression of the CIC-DUX4 fusion protein as a result of chromosomal rearrangements. CIC-DUX4 displays chromatin binding properties, and is therefore believed to function as an aberrant transcription factor.
View Article and Find Full Text PDFOncogenic fusion proteins generated by chromosomal translocations play major roles in cancer. Among them, fusions between EWSR1 and transcription factors generate oncogenes with powerful chromatin regulatory activities, capable of establishing complex gene expression programs in permissive precursor cells. Here we define the epigenetic and 3D connectivity landscape of Clear Cell Sarcoma, an aggressive cancer driven by the EWSR1-ATF1 fusion gene.
View Article and Find Full Text PDFArticle Synopsis
- Synovial sarcoma (SyS) is an aggressive cancer driven by the SS18-SSX fusion, showing low levels of T cell infiltration, which indicates immune evasion.
- Researchers used single-cell RNA sequencing to analyze 16,872 cells from human SyS tumors, identifying a key malignant subpopulation linked to poorer clinical outcomes and immune-deprived areas.
- The study found that the malignant cell state is influenced by the SS18-SSX fusion and can be targeted with a combination of HDAC and CDK4/CDK6 inhibitors, boosting T cell responses and enhancing treatment effectiveness.
Article Synopsis
- Synovial sarcoma (SyS) is a type of aggressive cancer caused by a specific genetic mutation that fuses the SS18 gene with one of the SSX genes, leading to abnormal gene regulation.
- Researchers developed organoid models of SyS and conducted extensive genome profiling to uncover how this cancer alters chromatin structure and influences gene expression.
- Findings revealed that the SS18-SSX fusion disrupts normal cellular processes by changing the way chromatin is remodeled, making SyS cells reliant on specific regulators, and these changes can be reversed if the SS18-SSX fusion is removed.