Rare Inherited Cholestatic Disorders and Molecular Links to Hepatocarcinogenesis

Hepatocellular carcinoma (HCC) is the most common primary liver cancer affecting adults and the second most common primary liver cancer affecting children. Recent years have seen a significant increase in our understanding of the molecular changes associated with HCC. However, HCC is a complex disea...

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Bibliographic Details
Published in:Cells (Basel, Switzerland) Switzerland), 2022-08, Vol.11 (16), p.2570
Main Authors: Jeyaraj, Rebecca, Kelly, Deirdre
Format: Article
Language:English
Subjects:
ABCB11 gene
Adults
Bile
Causes of
Cell cycle
Children
Cholangiocarcinoma
Cholestasis
Complications and side effects
Development and progression
Gallbladder diseases
Genes
Genetic disorders
Hepatitis B
hepatocarcinogenesis
Hepatocellular carcinoma
Infections
inherited cholestasis
Jaundice, Obstructive
Liver cancer
Liver diseases
Metabolism
Pediatrics
Proteins
Response rates
Review
Risk factors
TJP2 gene
Tumors
VPS33B gene
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Summary:Hepatocellular carcinoma (HCC) is the most common primary liver cancer affecting adults and the second most common primary liver cancer affecting children. Recent years have seen a significant increase in our understanding of the molecular changes associated with HCC. However, HCC is a complex disease, and its molecular pathogenesis, which likely varies by aetiology, remains to be fully elucidated. Interestingly, some inherited cholestatic disorders that manifest in childhood are associated with early HCC development. This review will thus explore how three genes that are associated with liver disease in childhood (ABCB11, TJP2 and VPS33B) might play a role in the initiation and progression of HCC. Specifically, chronic bile-induced damage (caused by ABCB11 changes), disruption of intercellular junction formation (caused by TJP2 changes) and loss of normal apical–basal cell polarity (caused by VPS33B changes) will be discussed as possible mechanisms for HCC development.
ISSN:2073-4409
2073-4409
DOI:10.3390/cells11162570