Role of Aire and Notch4 Allelic Mutations in Alopecia Areata in C3H/HeJ Mice

This study investigated the impact of Aire and Notch4 mutations on alopecia areata (AA) in C3H/HeJ mice. Two congenic strains were developed: C3.129S2(B6)-Aire tm1.1Doi /J, a null mutant, and C3.129S1(B6)-Notch4 tm1Grid /J, a truncated allele. The Aire mutation led to a decrease in AA frequency, while the Notch4 mutation did not result in any AA cases, likely due to its close linkage to the H2 b resistance gene. These findings suggest that Aire and Notch4 mutations may influence AA onset and severity, supporting their potential role in distinct subtypes of the disease.