Article
Active immunotherapy reduces NOTCH3 deposition in brain capillaries in a CADASIL mouse model.
EMBO molecular medicine - 8 Feb 2023
Oliveira Daniel V, Coupland Kirsten G, Shao Wenchao, Jin Shaobo, Del Gaudio Francesca, Wang Sailan, Fox Rhys, Rutten Julie W, Sandin Johan, Zetterberg Henrik, Lundkvist Johan, Lesnik Oberstein Saskia Aj, Lendahl Urban, Karlström Helena
Abstract excerpt
Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is the most common monogenic form of familial small vessel disease; no preventive or curative therapy is available. CADASIL is caused by mutations in the NOTCH3 gene, resulting in a mutated NOTCH3 receptor, with aggregation of the NOTCH3 extracellular domain (ECD) around vascular smooth muscle cells. In this...
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