Target intelligence / Profile preview

DNAJ heat shock protein family member B1-protein kinase cAMP-dependent catalytic subunit alpha fusion protein (DNAJB1-PRKACA fusion protein)

Target
DNAJB1-PRKACA fusion protein
Molecular classification
Fusion Protein, Chimeric Protein, Kinase, Molecular Chaperone
01

Overview

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.

Other names
DNAJB1-PRKACAChimeric DnaJ-PKAHSP40-PKA Fusion Protein
02

Mechanism of action

Inhibiting kinase activity of the fusion protein, disruption of protein-protein interactions.

03

Biological functions

Protein foldingStress responseSignal transduction via cAMPSerine/threonine kinase activityCell proliferationOncogenesis
04

Disease associations

CancerFibrolamellar Hepatocellular Carcinoma
05

Safety considerations

Potential for off-target effects with kinase inhibitorsDevelopment of resistance to targeted therapies
06

Biomarkers

DNAJB1-PRKACA fusion transcript (diagnostic)DNAJB1-PRKACA fusion protein (diagnostic)

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