Costello syndrome model mice with a Hras^G12S/+ mutation are susceptible to develop house dust mite-induced atopic dermatitis.

ABSTRACT: Costello syndrome is an autosomal dominant disorder that is caused by germline HRAS mutations. Patients with Costello syndrome present craniofacial abnormalities, cardiac defects, and cancer predisposition, as well as skin abnormalities, including papillomas, keratosis pilaris, and eczematous dermatitis. However, the mechanisms underlying the dermatological abnormalities remain unclear. Here, we demonstrated that knock-in mice expressing an Hras G12S mutation (Hras^G12S/+ mice) are susceptible to develop atopic dermatitis (AD)-like skin lesions, including eczema, pruritus, elevated serum IgE levels, acanthosis, and the infiltration of mast cells, basophils, and type-2 innate lymphoid cells in the dermis, after stimulation with house dust mite allergens (Dermatophagoides farinae,

SUBMITTER: Katata Y 

PROVIDER: S-EPMC7426869 | biostudies-literature | 2020 Aug

REPOSITORIES: biostudies-literature