NIH R01 · 2025
Bromodomain and extra-terminal motif (BET) inhibitors in Solitary fibrous tumor (SFT)
Project Summary Solitary fibrous tumor (SFT), historically also called hemangiopericytoma (HPC), is a soft-tissue sarcoma occurring in adults and infants. This nonhereditary cancer is a result of an environmental intrachromosomal gene fusion between NAB2 and STAT6 on chromosome 12, which fuses the activation domain of STAT6 with the repression domain of NAB2. Consequently, instead of NAB2 repressing early growth response (EGR-1) target genes, the fusion protein activates them leading to tumorigenesis. There are at least 6 distinct fusion variants that account for pathologic variation and tumor aggressiveness in SFTs. Anatomically, these blood vessel-derived tumors can occur anywhere;…
From the public funding record at NIH RePORTER. Describes the funded project, not the reviews below.