Further support to Aire haploinsufficiency comes from the report by Liston et al (Liston et al., 2004) demonstrating that the loss of one copy of the Aire allele in TCR-insHEL double transgenic mice caused severe functional defects in negative selection of autoreactive T cells and resulted in pancreatic cell insulitis, with diabetes incidence comparable to the Aire KO mouse. Here, AIRE is linked to diabetes mellitus.