EZH2 and hyperinsulinemic hypoglycemia, familial, 4: Other variants, such as the enhancer of zeste homolog 2 (EZH2), nuclear factor (erythroid-derived 2)-like 2 (NRF2), x-ray repair cross-complementing group (XRCC1), ATP binding cassette subfamily C member 2 (ABCB2), ATPase Phospholipid Transporting 8B1 (ATP8B1), natural killer cell receptor G2D (NKG2D), and alpha1-antitrypsin (α1AT) deficiency Z heterozygosity, have been linked to increased risk of CCA or associated with the outcome of patients with bile duct tumors [64,67,68].