Journal publication Archives - 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 …

Fibrolamellar carcinoma: An entity all its own

This publication summarizes the current understanding of fibrolamellar carcinoma based on a review of currently available literature. It outlines the epidemiology, pathology, and biology of FLC, plus provides a high-level overview of the evidence supporting existing therapies for the disease — including surgical, radiotherapy, interventional radiology and systemic treatments. The full article can be read …

DNAJB1-PRKACA in HEK293T cells induces LINC00473 overexpression that depends on PKA signaling

While FLC in almost all patients is caused by the DNAJB1-PRKACA gene fusion, the mechanism by which that fusion causes the cancer is still unclear. In order to get insight into that question, this team engineered the DNAJB1-PRKACADP fusion gene into “immortalized” liver cells and measured the impact that the change had on the functions …

Identification of Novel Therapeutic Targets for Fibrolamellar Carcinoma Using Patient Derived Xenografts and Direct from Patient Screening

This publication describes the results of the first large scale screening of known drugs and compounds for their effectiveness against FLC. It identified six new PDX lines derived by direct implantation of tumor specimens, both from the liver (one primary and one recurrent tumor) and metastases. These facilitated the first reported large-scale screening of compounds …

Clinical Outcomes in Fibrolamellar Hepatocellular Carcinoma Treated with Immune Checkpoint Inhibitors

Although immune checkpoint inhibitors (ICIs) are becoming the standard of care in some types of cancers, few studies have looked at the safety and efficacy of ICIs for treating FLC. This multicenter, retrospective analysis aimed to determine if treatment with ICIs could be helpful for FLC patients. Patient data included in this study was identified …

Defining incidence and complications of fibrolamellar liver cancer through tiered computational analysis of clinical data

Rare diseases like fibrolamellar are often poorly understood because of the difficulty in assembling accurate patient data and the lack of ICD billing codes specific to the disease. The most cited study of the incidence of FLC in the US, using data from the National Cancer Institute’s Surveillance, Epidemiology, and End Results (SEER) Program, reports …

Oncogenic Addiction of Fibrolamellar Hepatocellular Carcinoma to the Fusion Kinase DNAJB1-PRKACA

Based on another study from the Simon lab, we have known that a gene fusion called DNAJB1-PRKACA produced a mutated protein kinase A (PKA) that causes 99% of cases of fibrolamellar carcinoma. In this study, the researchers found that this gene is not only important for the growth of FLC tumors but also that blocking …

CDK7 is a novel therapeutic target in fibrolamellar carcinoma

Fibrolamellar carcinoma (FLC) is a rare, aggressive liver cancer primarily affecting young individuals, with limited treatment options and poor prognosis. The discovery of the DNAJB1-PRKACA gene fusion as the primary oncogenic driver in FLC over a decade ago has reshaped research efforts. The exact way the DNAJ-PKAc protein causes cancer is still unclear, but research …

Disruption of proteome by an oncogenic fusion kinase alters metabolism in fibrolamellar hepatocellular carcinoma

In this study, the researchers used a method called mass spectrometry to study the proteins and metabolites inside FLC tumors and compared them to normal liver tissue. They found that the proteins in FLC tumors are different from those in healthy liver tissue, which could explain why FLC cells react differently to certain drugs and …

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