ArticleCancer discovery2025
DNAJB1-PRKACA Fusion Drives Fibrolamellar Liver Cancer through Impaired SIK Signaling and CRTC2/p300-Mediated Transcriptional Reprogramming.
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Article in Cancer discovery, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. An erratum has been issued. Cited by 16 papers.
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16citing papers in PubMed
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Who cites it
16 citing papers in PubMed.
- Remodeling of mitochondrial dynamics by metabolic pathways couples to oncogenic growth inScience advances · 2026Article
- Clinical guideline for the diagnosis and treatment of fibrolamellar carcinoma.Hepatology (Baltimore, Md.) · 2026Article
- Cancer-associated fusion transcripts: mechanisms, functional roles, and clinical implications.Clinical and experimental medicine · 2026Review
- Article
- DNA-PK-mediated CRTC2 phosphorylation promotes NHEJ and suppresses antitumor immunity via relocation to repair complexes.Nature communications · 2026Article
- DNAJ-PKAc induces metabolic rewiring and enhanced glutamine flux in fibrolamellar HCC.Journal of hepatology · 2026Article
- AI-derived prognostic model identifies high-risk gene signatures in pediatric gliomas.Frontiers in immunology · 2026Article
- Article
- Article
- Targeting LKB1/STK11-mutant cancer: distinct metabolism, microenvironment, and therapeutic resistance.Trends in pharmacological sciences · 2025Review
- Article
- Mitochondrial calcium signaling regulates branched-chain amino acid catabolism in fibrolamellar carcinoma.Science advances · 2025Article
- LKB1 regulates JNK-dependent stress signaling and apoptotic dependency of KRAS-mutant lung cancers.Nature communications · 2025Article
- Correction: DNAJB1-PRKACA Fusion Drives Fibrolamellar Liver Cancer through Impaired SIK Signaling and CRTC2/p300-Mediated Transcriptional Reprogramming.Cancer discovery · 2025Article
- Global, regional, and national burden of liver cancer in adolescents and young adults from 1990 to 2021: an analysis of the global burden of disease study 2021 and forecast to 2040.Frontiers in public health · 2025Article
- Managing Advanced Fibrolamellar Carcinoma at Stage IV: A Case Report.GE Portuguese journal of gastroenterologyArticle
Corrections and comments
- Erratum issued
Authors and funding
24 authors.
Ilaria Gritti *Krantz Family Center for Cancer Research, Massachusetts General Hospital, Boston, Massachusetts.ORCID 0000-0002-7313-0450
Jinkai Wan *Krantz Family Center for Cancer Research, Massachusetts General Hospital, Boston, Massachusetts.ORCID 0000-0001-6736-0332
Vajira WeeresekaraKrantz Family Center for Cancer Research, Massachusetts General Hospital, Boston, Massachusetts.ORCID 0000-0002-9668-7091
Joel M VazDivision of Human Biology, Fred Hutchinson Cancer Center, Seattle, Washington.ORCID 0009-0008-7177-3525
Giuseppe TarantinoDepartment of Medicine, Harvard Medical School, Boston, Massachusetts.ORCID 0000-0002-6078-6856
Tenna Holgersen BrydeNovo Nordisk Foundation Center for Basic Metabolic Research, University of Copenhagen, Copenhagen, Denmark.ORCID 0000-0003-3422-2122
Vindhya VijayKrantz Family Center for Cancer Research, Massachusetts General Hospital, Boston, Massachusetts.ORCID 0000-0002-7886-7443
Ashwin V KammulaKrantz Family Center for Cancer Research, Massachusetts General Hospital, Boston, Massachusetts.ORCID 0000-0001-5284-721X
Prabhat KattelKrantz Family Center for Cancer Research, Massachusetts General Hospital, Boston, Massachusetts.ORCID 0009-0006-3099-0780
Songli ZhuDivision of Human Biology, Fred Hutchinson Cancer Center, Seattle, Washington.ORCID 0000-0001-8560-5444
Phuong VuKrantz Family Center for Cancer Research, Massachusetts General Hospital, Boston, Massachusetts.ORCID 0009-0006-3846-3002
Marina ChanDivision of Human Biology, Fred Hutchinson Cancer Center, Seattle, Washington.ORCID 0009-0004-0332-4454
Meng-Ju WuKrantz Family Center for Cancer Research, Massachusetts General Hospital, Boston, Massachusetts.ORCID 0000-0001-6847-7065
John D GordanDivision of Hematology and Oncology, University of California, San Francisco, San Francisco, California.ORCID 0000-0001-8997-5725
Krushna C PatraUniversity of Cincinnati College of Medicine, Cincinnati, Ohio.ORCID 0000-0002-2386-6435
Vanessa S SilveiraKrantz Family Center for Cancer Research, Massachusetts General Hospital, Boston, Massachusetts.ORCID 0000-0001-5633-9490
Robert T MangusoKrantz Family Center for Cancer Research, Massachusetts General Hospital, Boston, Massachusetts.ORCID 0000-0003-1336-413X
Marc N WeinDepartment of Medicine, Harvard Medical School, Boston, Massachusetts.ORCID 0000-0002-6015-8147
Christopher J OttKrantz Family Center for Cancer Research, Massachusetts General Hospital, Boston, Massachusetts.ORCID 0000-0001-6142-7241
Jun QiDepartment of Medicine, Harvard Medical School, Boston, Massachusetts.ORCID 0000-0002-1461-3356
David LiuDepartment of Medicine, Harvard Medical School, Boston, Massachusetts.ORCID 0000-0003-0346-5033
Kei SakamotoNovo Nordisk Foundation Center for Basic Metabolic Research, University of Copenhagen, Copenhagen, Denmark.ORCID 0000-0001-8839-5980
Taranjit S GujralDivision of Human Biology, Fred Hutchinson Cancer Center, Seattle, Washington.ORCID 0000-0002-4453-3031
Nabeel BardeesyKrantz Family Center for Cancer Research, Massachusetts General Hospital, Boston, Massachusetts.ORCID 0000-0003-3867-0416
Funding
The RAS and P13K Pathways in Pancreatic AdenocarcinomaP01CA117969 · NCI · UNIVERSITY OF TX MD ANDERSON CAN CTR · PI KALLURI, RAGHU · 2006 to 2025
$41.7MTissue and Pathology ResourcesP50CA127003 · NCI · DANA-FARBER CANCER INST · PI SHIVDASANI, RAMESH A · 2007 to 2023
$33.2MTargeting novel therapeutic vulnerabilities in LKB1 mutant tumors.R01CA219670 · NCI · MASSACHUSETTS GENERAL HOSPITAL · PI EL-BARDEESY, NABEEL, WONG, KWOK KIN · 2018 to 2022
$3.4MFunctions of the LKB1 tumor suppressor in control in metabolism and epigenticsR01CA215498 · NCI · MASSACHUSETTS GENERAL HOSPITAL · PI EL-BARDEESY, NABEEL · 2018 to 2022
$2.7MTargeting PLK1 signaling for the treatment of fibrolamellar carcinomaR01CA273081 · NCI · FRED HUTCHINSON CANCER CENTER · PI Taran Singh Gujral · 2023 to 2026
$1.9MTargeting Oncogenic PKA signaling mechanismsR01CA279997 · NCI · UNIVERSITY OF CALIFORNIA, SAN FRANCISCO · PI John D Gordan, John D Scott · 2024 to 2026
$1.9MInvestigating the molecular mechanisms of growth in GNAS mutant pancreatic cancer.R37CA272854 · NCI · UNIVERSITY OF CINCINNATI · PI Krushna Chandra Patra · 2022 to 2026
$1.8MAlex's Lemonade Stand Foundation for Childhood Cancer (ALSF) 21-24687American-Italian Cancer Foundation (AICF)Bertarelli Rare Cancers FundFibrolamellar Cancer Foundation (FCF)National Cancer Institute (NCI) P50CA127003National Cancer Institute (NCI) R01CA273081National Cancer Institute (NCI) R01CA279997NCI NIH HHS P01 CA117969NCI NIH HHS P50 CA127003NCI NIH HHS R01 CA215498NCI NIH HHS R01 CA219670NCI NIH HHS R01 CA273081NCI NIH HHS R01 CA279997NCI NIH HHS R37 CA272854Novo Nordisk Fonden (NNF) NNF18CC0034900
Abstract
significanceThis work combines functional studies in model systems and examination of human tumor specimens to define a central oncogenic pathway driven by DNAJB1-PRKACA fusions in FLC. DNAJB1-PRKACA-mediated inactivation of the SIK stimulates CRTC2-p300-mediated transcription to drive tumor growth. The findings illuminate pathogenic mechanisms and inform therapeutic development.
Indexed as
Carcinoma, HepatocellularCyclic AMP-Dependent Protein Kinase Catalytic SubunitsE1A-Associated p300 ProteinHSP40 Heat-Shock ProteinsLiver NeoplasmsOncogene Proteins, FusionProtein Serine-Threonine KinasesTranscription FactorsAnimalsCell Line, TumorHumansMiceSignal TransductionCyclic AMP-Dependent Protein Kinase Catalytic SubunitsDNAJB1 protein, humanE1A-Associated p300 ProteinEP300 protein, humanHSP40 Heat-Shock ProteinsOncogene Proteins, FusionPRKACA protein, humanProtein Serine-Threonine KinasesTranscription Factors
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