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Article

Fibrolamellar carcinoma in the Carney complex: PRKAR1A loss instead of the classic DNAJB1-PRKACA fusion.

Hepatology (Baltimore, Md.) - 1 Oct 2018

Graham Rondell P, Lackner Carolin, Terracciano Luigi, González-Cantú Yessica, Maleszewski Joseph J, Greipp Patricia T, Simon Sanford M, Torbenson Michael S

Abstract excerpt

Fibrolamellar carcinomas are characterized by activation of protein kinase A, a kinase composed of catalytic and regulatory subunits. PRKACA encodes a catalytic subunit of protein kinase A, and almost all fibrolamellar carcinomas have a heterozygous 400-kb deletion that leads to the fusion of DNAJB1 and PRKACA. The resulting DNAJB1-PRKACA fusion transcript is believed to activate protein kinase A by dysregulation...

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Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

Share to Cyclic AMP-Dependent Protein Kinase RIalpha Subunit

Share to In Situ Hybridization, Fluorescence

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Share to Oncogene Proteins, Fusion

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Source

Open in PubMed Open full text

Identifiers

PMID: 29222914
DOI: 10.1002/hep.29719
PMCID: PMC6151295

Genes

PRKACA
DNAJB1

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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