Hepatocellular carcinoma associated with tight‐junction protein 2 deficiency

Mutations in TJP2 (also named ZO-2), encoding tight-junction protein 2 (TJP2), cause an autosomal-recessive form of progressive intrahepatic cholestasis.1 Association of TJP2 deficiency with hepatocellular carcinoma (HCC) in childhood has not been recognized.