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Notch3 Arg170Cys Knock-In Mice Display Pathologic and Clinical Features of the Neurovascular Disorder Cerebral Autosomal Dominant Arteriopathy With Subcortical Infarcts and Leukoencephalopathy
Author(s) -
Goedele Wallays,
Dieter Nuyens,
Robert SilasiMansat,
Joris Souffreau,
Zsuzsanna CallaertsVegh,
An Van Nuffelen,
Lieve Moons,
Rudi D’Hooge,
Florea Lupu,
Peter Carmeliet,
Désiré Collen,
Mieke Dewerchin
Publication year - 2011
Publication title -
arteriosclerosis thrombosis and vascular biology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 3.007
H-Index - 270
eISSN - 1524-4636
pISSN - 1079-5642
DOI - 10.1161/atvbaha.111.237859
Subject(s) - cadasil , leukoencephalopathy , pathology , medicine , gliosis , neurovascular bundle , pathogenesis , stroke (engine) , neuroscience , disease , biology , mechanical engineering , engineering
Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is an adult-onset neurovascular disorder caused by stereotyped mutations in the NOTCH3 receptor. Elucidation of its pathobiology is still incomplete and remains a challenge, in part because the available preclinical mouse models to date do not reproduce the full spectrum of CADASIL pathology and clinical disease.

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