VEGFR3 modulates brain microvessel branching in a mouse model of 22q11.2 deletion syndrome.

ABSTRACT: The loss of a single copy of TBX1 accounts for most of the clinical signs and symptoms of 22q11.2 deletion syndrome, a common genetic disorder that is characterized by multiple congenital anomalies and brain-related clinical problems, some of which likely have vascular origins. Tbx1 mutant mice have brain vascular anomalies, thus making them a useful model to gain insights into the human disease. Here, we found that the main morphogenetic function of TBX1 in the mouse brain is to suppress vessel branching morphogenesis through regulation of Vegfr3 We demonstrate that inactivating Vegfr3 in the Tbx1 expression domain on a Tbx1 mutant background enhances brain vessel branching and filopodia formation, whereas increasing Vegfr3 expression in th

SUBMITTER: Cioffi S 

PROVIDER: S-EPMC9553901 | biostudies-literature | 2022 Dec

REPOSITORIES: biostudies-literature