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journal publication
This retrospective study, led by Dr. Paul Kent and colleagues at Rush University Medical Center, evaluated whether a three-drug combination known as “triple therapy (TT)”, or “triple immunochemotherapy”, could help control advanced fibrolamellar carcinoma (FLC). This treatment combines: This is the same therapy investigated by M.D. Anderson Cancer center in a prospective clinical trial. Study …
This article describes work conducted at the University Hospital Tübingen on the creation of a therapeutic peptide vaccine for fibrolamellar carcinoma. Using FLC’s characteristic DNAJB1-PRKACA fusion protein, they designed a personalized peptide vaccine using fragments of that DNAJB1-PRKACA protein and administered it to a patient with FLC. The patient, a recipient of a liver transplant, …
Fibrolamellar carcinoma (FLC) is a rare liver cancer that mostly affects young people and currently has no standard, effective treatment. Over the last decade, many studies have been conducted that have analyzed multiple types of biological data from the same cells or tissue to get a more complete picture of how the disease works, but …
This study explored how fibrolamellar carcinoma (FLC) rewires the way its cells generate energy, focusing on calcium inside mitochondria—the cell’s “power plants.” Researchers found that FLC cells have abnormally high mitochondrial calcium levels, which changes how they process nutrients like branched‑chain amino acids. A key driver of this is the mitochondrial calcium uniporter, a channel …
This article, published in Nature Reviews Gastroenterology & Hepatology, summarizes the key advances made in understanding the disease since the 2014 discovery that nearly all FLC tumors contain the DNAJB1-PRKACA chimeric fusion transcript. The paper outlines the advances made in understanding the molecular pathways driven by the fusion protein and the potential to identify actionable …
This study, led by a team from Cornell University, looked at the molecular features of fibrolamellar carcinoma (FLC), a rare liver cancer that mainly affects teenagers and young adults. Until now, most studies analyzing biological data from fibrolamellar tissue have examined tumors in bulk, mixing together all the different cell types found in a tumor. …
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 …
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 …
Rare tumors contribute significantly to the burden of cancer in the United States, yet are often poorly understood and have inadequate research support. To address this situation, the National Cancer Institute’s (NCI) My Pediatric and Adult Rare Tumor Network (MyPART) was developed. MyPART’s goals include connecting participants and investigators through shared infrastructure and networks; accelerating …