TY - JOUR
T1 - There and back again
T2 - Autoimmune Polyendocrinopathy Syndrome Type I and the Aire knockout mouse
AU - Liston, Adrian
PY - 2006
Y1 - 2006
N2 - Autoimmune Polyendocrinopathy Syndrome Type I (APS1) is a severe human multiorgan autoimmune disorder, caused by mutations in the gene AIRE. Following this determination, a mouse model for the disease was rationally designed by knocking out the murine homologue, Aire. The Aire knockout mouse has demonstrated that Aire initiates immune tolerance via promiscuous transcription of organ-specific genes in the thymus. Recent studies using the Aire knockout mouse have demonstrated gene-dose dependency and disease modifier loci, returning the onus of exploration to human genetics.
AB - Autoimmune Polyendocrinopathy Syndrome Type I (APS1) is a severe human multiorgan autoimmune disorder, caused by mutations in the gene AIRE. Following this determination, a mouse model for the disease was rationally designed by knocking out the murine homologue, Aire. The Aire knockout mouse has demonstrated that Aire initiates immune tolerance via promiscuous transcription of organ-specific genes in the thymus. Recent studies using the Aire knockout mouse have demonstrated gene-dose dependency and disease modifier loci, returning the onus of exploration to human genetics.
UR - http://www.scopus.com/inward/record.url?scp=33645889003&partnerID=8YFLogxK
U2 - 10.1016/j.ddmod.2006.03.006
DO - 10.1016/j.ddmod.2006.03.006
M3 - Review article
SN - 1740-6757
VL - 3
SP - 33
EP - 40
JO - Drug Discovery Today: Disease Models
JF - Drug Discovery Today: Disease Models
IS - 1
ER -