North American Indian childhood cirrhosis
North American Indian childhood cirrhosis is a disease in humans that can affect Ojibway-Cree children in northwestern Quebec, Canada. The disease is due to an autosomal recessive abnormality of the CIRH1A gene, which codes for cirhin.
NAIC is a ribosomopathy. An R565W mutation of CIRH1A leads to partial impairment of cirhin interaction with NOL11.
Initial transient neonatal jaundice advances over time to biliary cirrhosis with severe liver fibrosis. Eventually, liver failure occurs, and requires liver transplantation.
