Fibrolamellar Cancer Foundation

Proteo-metabolomics and patient tumor slice experiments point to amino acid centrality for rewired mitochondria in fibrolamellar carcinoma

Proteomics studies assess the composition, structure, expression, and the interactions and connections between proteins at an overall level in tissue. Metabolomics studies focus on characterizing metabolites, the small molecules generated by specific cellular processes. Both types of studies can yield valuable insights into the complex biochemical pathways, networks, and molecular systems that control important cell …

DNAJB1-PRKACA fusion protein-regulated LINC00473 promotes tumor growth and alters mitochondrial fitness in fibrolamellar carcinoma

While the role of the DNAJB1-PRKACA (DP) fusion protein in fibrolamellar carcinoma (FLC) was discovered a decade ago, it has been difficult to directly inhibit the function of this fusion protein. In this study, the investigators set out to identify downstream targets of the oncogene that could be exploited for new therapies. The investigators report …

DNAJB1-PRKACA fusion drives fibrolamellar liver cancer through impaired SIK signaling and CRTC2/p300-mediated transcriptional reprogramming

Decoding the Cancer Mechanisms of Fibrolamellar Carcinoma: How DNAJB1-PRKACA drives Cancer Growth Critical cellular functions such as growth, survival, and communication with other cell types in the body are mediated by networks of proteins. These networks are normally tightly regulated by signaling molecules, which serve as on/off switches. Genetic changes can cause these signaling molecules …

Reversing immunosuppression in the tumor microenvironment of fibrolamellar carcinoma via PD‑1 and IL‑10 blockade

Due to limited knowledge about the immune response to FLC, developing effective immunotherapy is challenging for the disease. In this study, the team investigated the immune environment of fibrolamellar carcinoma (FLC) using several techniques including multiplex immunohistochemistry and flow cytometry. Key findings of the effort include: Implications: The key takeaway of this study is that …

DNAJB1-PRKACA fusion neoantigens elicit rare endogenous T cell responses that potentiate cell therapy for fibrolamellar carcinoma

In this study, published in March 2024, the researchers looked at how a patient’s immune system responds to the protein produced by the fusion of the genes DNAJB1 and PRKACA in FLC. Specifically, the team aimed to understand how the immune system’s “killer” T cells (CD8s) respond to this mutation and evaluate T cell receptors …