title: Fibrolamellar Hepatocellular Carcinoma: Mechanistic Distinction From Adult Hepatocellular Carcinoma.
abstract: Fibrolamellar hepatocellular carcinoma (FL-HCC) has historically been classified as a rare subtype of HCC. However, unlike "classic" HCC, it occurs in children and young adults without underlying liver disease. The recent discovery of a deletion mutation in all FL-HCCs represented a major advancement in understanding the pathogenesis of this disease. This deletion results in the fusion of the genes encoding a heat shock protein (DNAJB1) and the catalytic subunit of protein kinase A (PKA, PRKACA), and overexpression of PRKACA and enhanced cAMP-dependent PKA activity. This review summarizes recent advancements in FL-HCC pathogenesis and characteristics of the HSP40-PKA C protein.
authors.affiliation: Department of Surgery, University of Washington, Seattle, Washington.|Division of General and Thoracic Surgery, Seattle Children's Hospital, Seattle, Washington.
authors.forename: Kevin M
authors.initials: KM
authors.lastname: Riggle
authors.affiliation: Howard Hughes Medical Institute, University of Washington, Seattle, Washington.|Department of Pharmacology, University of Washington, Seattle, Washington.
authors.forename: Rigney
authors.initials: R
authors.lastname: Turnham
authors.affiliation: Howard Hughes Medical Institute, University of Washington, Seattle, Washington.|Department of Pharmacology, University of Washington, Seattle, Washington.
authors.forename: John D
authors.initials: JD
authors.lastname: Scott
authors.affiliation: Department of Surgery, University of Washington, Seattle, Washington.|Northwest Liver Research Program, University of Washington, Seattle, Washington.
authors.forename: Raymond S
authors.initials: RS
authors.lastname: Yeung
authors.affiliation: Department of Surgery, University of Washington, Seattle, Washington.|Division of General and Thoracic Surgery, Seattle Children's Hospital, Seattle, Washington.|Northwest Liver Research Program, University of Washington, Seattle, Washington.|Department of Pathology, University of Washington, Seattle, Washington.
authors.forename: Kimberly J
authors.initials: KJ
authors.lastname: Riehle
mesh_terms: D000293:Adolescent; D000328:Adult; D001483:Base Sequence; D006528:Carcinoma, Hepatocellular / Q000145:classification / Q000235:genetics* / Q000473:pathology; D002648:Child; D002675:Child, Preschool; D054751:Cyclic AMP-Dependent Protein Kinase Catalytic Subunits / Q000235:genetics*; D005260:Female; D050956:HSP40 Heat-Shock Proteins / Q000235:genetics*; D006801:Humans; D008113:Liver Neoplasms / Q000145:classification / Q000235:genetics* / Q000473:pathology; D008297:Male; D015514:Oncogene Proteins, Fusion / Q000235:genetics*; D017384:Sequence Deletion
chemical_list: C494647:DNAJB1 protein, human; D050956:HSP40 Heat-Shock Proteins; D015514:Oncogene Proteins, Fusion; D054751:Cyclic AMP-Dependent Protein Kinase Catalytic Subunits; C517757:PRKACA protein, human
keywords: carcinoma; fibrolamellar; hepatocellular; protein kinase A