Homozygotes: Homozygous ROSA NOTCH3-1031 mice are viable, fertile, with the CADASIL (cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy)-associated R1031C mutation inserted into the Gt(ROSA)26Sor locus. Expression of the mutated human NOTCH3 is blocked by an upstream loxP-flanked STOP sequence. When bred to cre expressing mice, the STOP sequence is deleted in the tissues of offspring where Cre recombinase is present, resulting in mutated human NOTCH3 expression and the parallel expression of enhanced green fluorescent protein (GFP) targeted to the nucleus. Expression in vascular smooth muscle cells is characterized by cell degeneration and the presence of granular osmiophilic deposits. These mice allow inducible expression of a human mutation associated with ischemic stroke.

Heterozygote: Not evaluated

In this ROSA NOTCH3-1031 knockin strain, the human NOTCH3 gene is altered by the CADASIL-associated R1031C mutation and expression is blocked by an upstream loxP-flanked STOP sequence. Cre-mediated excision results in expression of the mutated NOTCH3 and GFP targeted to the nucleus. Expression in vascular smooth muscle cells is characterized by cell degeneration and the presence of granular osmiophilic deposits. These mice allow inducible expression of a human mutation associated with ischemic stroke.

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