These GV mutant mice conditionally express a mutant form of HRAS (Harvey rat sarcoma virus oncogene), which encodes a member of the Ras oncogene family involved in signal transduction pathways. Mutations in Hras are associated with Costello syndrome, a rare disorder associated with delayed development, craniofacial abnormalities, extra folds of skin, and multiple heart defects. The amino acid substitution, G12V, has the lowest GTPase activity found in codon 12 substitutions and is the predominant codon 12 mutation found in human Costello syndrome patients. When bred to mice that express tissue-specific Cre recombinase, resulting offspring will express the mutant G12V protein in cre-expressing tissues. Mice heterozygous for the allele are viable and fertile.

A G12V mutant form of protein is conditionally expressed from the Hras1 (Harvey rat sarcoma virus oncogene 1) gene when a neomycin cassette is excised by Cre recombinase. This oncogene is involved with the development of Costello syndrome, a neuro-cardio-facio-cutaneous developmental syndrome.

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