Mouse Genome Informatics
hm
    Notch3tm1.1Dwr/Notch3tm1.1Dwr
involves: 129S/SvEv * Swiss
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
       
behavior/neurological
• in 9 of 73 mice develop overt permanent motor disability, ataxia or both
• in 9 of 73 mice, 3 of which show staggering ataxic gait
• of one or more limb in 8 of 9 mice with motor disabilities

cardiovascular system
N
• mice exhibit normal vascular smooth muscle cell remodeling or growth and normal cerebral capillary densities (J:191454)
• mice exhibit arteriopathy with granular osminophilic material deposits in the basal lamina of the arterial smooth muscle cells and surrounding matrix in brain and tail arteries

nervous system
• micro-bleeds, hemosiderin deposits or hemosiderin-containing macrophages, perivascular inflammatory infiltration, gliosis, thrombosis, perivascular fibrin(ogen) deposition, microinfarctions characterized by foci with pallor and cell loss around small cystic cavities, and enlargement of the Virchow-Robin spaces creating perivascular lacunae
• in some mice

homeostasis/metabolism
• in the brain vasculature of some mice

Mouse Models of Human Disease
OMIM IDRef(s)
Cerebral Arteriopathy, Autosomal Dominant, with Subcortical Infarcts and Leukoencephalopathy; CADASIL 125310 J:191454