SOX10 and Aganglionic megacolon: It was reported that homozygotes of Dominant megacolon mice with SOX10 mutation (SOX10Dom/Dom) die in utero prior to 13 days of gestation, and a significant proportion of heterozygous Dom mice were lost before weaning because of the occurrence of megacolon [18, 21], while SOX10-knockout mice (SOX10Lacz/Lacz) have phenotypes similar to those of Dom mice [22].