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The DNAJ heat shock protein family member B1-protein kinase cAMP-dependent catalytic subunit alpha fusion protein is a chimeric oncoprotein associated with fibrolamellar hepatocellular carcinoma (FL-HCC). This fusion results from a deletion on chromosome 19, joining the first exon of DNAJB1 to exons 2–10 of PRKACA. The fusion creates a functional chimeric enzyme with altered regulatory control, leading to increased cAMP-dependent signaling and tumorigenesis. The presence of this fusion is pathognomonic for FL-HCC and serves as a diagnostic biomarker and potential therapeutic target.
Inhibiting kinase activity of the fusion protein, disruption of protein-protein interactions.
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