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10.1002/pbc.25970

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C4877189!4877189 !26990031
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suck abstract from ncbi


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pmid26990031
      Pediatr+Blood+Cancer 2016 ; 63 (7 ): 1163-7
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  • Fibrolamellar Hepatocellular Carcinoma: Mechanistic Distinction From Adult Hepatocellular Carcinoma #MMPMID26990031
  • Riggle KM ; Turnham R ; Scott JD ; Yeung RS ; Riehle KJ
  • Pediatr Blood Cancer 2016[Jul]; 63 (7 ): 1163-7 PMID26990031 show ga
  • 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.
  • |*Base Sequence [MESH]
  • |*Sequence Deletion [MESH]
  • |Adolescent [MESH]
  • |Adult [MESH]
  • |Carcinoma, Hepatocellular/classification/*genetics/pathology [MESH]
  • |Child [MESH]
  • |Child, Preschool [MESH]
  • |Cyclic AMP-Dependent Protein Kinase Catalytic Subunits/*genetics [MESH]
  • |Female [MESH]
  • |HSP40 Heat-Shock Proteins/*genetics [MESH]
  • |Humans [MESH]
  • |Liver Neoplasms/classification/*genetics/pathology [MESH]
  • |Male [MESH]


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