Since defective Jagged1/Notch-2 signalling is responsible for transdifferentiation of hepatoblasts to biliary epithelia [15] and mutations in either JAG1 or NOTCH2 are known to cause Alagille syndrome type 1 and 2, respectively, association of JAG1 with biliary atresia reported by Kohsaka et al. [4] would be acceptable. This evidence concerns the gene NOTCH2 and Alagille syndrome due to a JAG1 point mutation.