Article
Claudin-1 involved in neonatal ichthyosis sclerosing cholangitis syndrome regulates hepatic paracellular permeability.
Hepatology (Baltimore, Md.) - 1 Apr 2012
Grosse Brigitte, Cassio Doris, Yousef Nadya, Bernardo Céline, Jacquemin Emmanuel, Gonzales Emmanuel
Abstract excerpt
UNLABELLED: Neonatal ichthyosis and sclerosing cholangitis (NISCH) syndrome is a liver disease caused by mutations of CLDN1 encoding Claudin-1, a tight-junction (TJ) protein. In this syndrome, it is speculated that cholestasis is caused by Claudin-1 absence, leading to increased paracellular permeability and liver injuries secondary to paracellular bile regurgitation. We studied the role of claudin-1 in hepatic...
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