NOTCH3 Mouse Models

Why Model NOTCH3

Notch3 is the mouse ortholog of human NOTCH3 and encodes a Notch-family transmembrane receptor expressed mainly in vascular smooth muscle and pericytes. NOTCH3 signaling has been associated with maintenance of small blood vessels.

NOTCH3 mutations cause CADASIL, an inherited cerebral small-vessel disease causing stroke and dementia, and NOTCH3-directed approaches are under study. Notch3-deficient and mutant mice show vascular changes relevant to the disease.

Model Applications

Humanized NOTCH3 models, including those carrying CADASIL-associated alleles, have been used to study NOTCH3 biology and to evaluate NOTCH3-directed approaches in defined experimental settings.

Custom NOTCH3 Model Options

Engineered to your specifications, with molecular validation of the targeted allele and germline transmission guaranteed.

• Conventional knockout
• Conditional knockout
• Point mutation / knockin
• Reporter knockin
• Humanized
• Targeted transgenic

Sources

• Most common NOTCH3 mutations causing CADASIL or CADASIL-like cerebral small vessel disease: a systematic review. Cereb Circ Cogn Behav. 2024. doi:10.1016/j.cccb.2024.100227
• Joutel A et al. Notch3 mutations in CADASIL, a hereditary adult-onset condition causing stroke and dementia. Nature. 1996. doi:10.1038/383707a0

Last reviewed: October 22, 2026 · Reviewed by: Paul Sheiffele

Related gene targets: Notch1 · Jag1 · Dll4 · Hey1