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2016 ; 63
(7
): 1163-7
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English Wikipedia
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.