Article
Pathogenic sequence for dissecting aneurysm formation in a hypomorphic polycystic kidney disease 1 mouse model.
Arteriosclerosis, thrombosis, and vascular biology - 1 Oct 2007
Hassane Sabrine, Claij Nanna, Lantinga-van Leeuwen Irma S, Van Munsteren J Conny, Van Lent Natascha, Hanemaaijer Roeland, Breuning Martijn H, Peters Dorien J M, DeRuiter Marco C
Abstract excerpt
OBJECTIVE: Autosomal Dominant Polycystic Kidney Disease (ADPKD) is a multi-system disorder characterized by progressive cyst formation in the kidneys. Serious complications of ADPKD are intracranial and aortic aneurysms. The condition is mainly caused by mutations in the PKD1 or PKD2 gene. We have carefully analyzed vascular remodeling in hypomorphic Pkd1(nl/nL) mouse model with dissecting aneurysms in the aorta....
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