Finally, although p53 has traditionally been viewed through the prism of tumor suppression, the fascinating new area of research that has begun to implicate aberrant activation of p53 in a number of developmental syndromes [143,162,163] raises the tantalizing question of whether defective RiBi or unscheduled p53 activation accounts for any cholangiopathies that are currently without a known etiology. Here, TP53 is linked to neoplasm.