Endothelial Notch signaling is upregulated in human brain arteriovenous malformations and a mouse model of the disease.

ABSTRACT: Brain arteriovenous malformations (BAVMs) can cause lethal hemorrhagic stroke and have no effective treatment. The cellular and molecular basis for this disease is largely unknown. We have previously shown that expression of constitutively-active Notch4 receptor in the endothelium elicits and maintains the hallmarks of BAVM in mice, thus establishing a mouse model of the disease. Our work suggested that Notch pathway could be a critical molecular mediator of BAVM pathogenesis. Here, we investigated the hypothesis that upregulated Notch activation contributes to the pathogenesis of human BAVM. We examined the expression of the canonical Notch downstream target Hes1 in the endothelium of human BAVMs by immunofluorescence, and showed increased levels relative to either autopsy or surgical bio

SUBMITTER: Murphy PA 

PROVIDER: S-EPMC3095492 | biostudies-literature | 2009 Sep

REPOSITORIES: biostudies-literature