However, biliary disease in ΔS6 livers is highly reminiscent of the neonatal intrahepatic cholestasis and jaundice that is seen in patients with North American Indian Childhood Cirrhosis (NAIC) (OMIM #604901), a rare autosomal recessive ribosomopathy associated with homozygous missense mutations (R565W) in the Cirhin1A-encoding gene CIRH1A/UTP4 [80]. Here, UTP4 is linked to hereditary North American Indian childhood cirrhosis.