NOTCH3 CADASIL Variant Receptor Aggregation Requires NOTCH3 Wild-Type Receptors: Identification of Highly Selective Inhibitors That Block the Process - PubMed
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- #Therapeutic Inhibitors
- #NOTCH3
- #CADASIL
- CADASIL is a monogenic autosomal dominant small vessel disease caused by mutations in NOTCH3 receptor.
- Pathogenic NOTCH3 CADASIL variants require wild-type NOTCH3 receptors to form high molecular mass assemblies.
- CADASIL mutations in EGFr 4 of NOTCH3 enhance JAGGED1 (JAG1)-dependent NOTCH3 transactivation.
- Novel NOTCH3 inhibitors can block receptor aggregation and mutant NOTCH3 signaling, offering potential therapy.