The NOTCH3 score: a pre-clinical CADASIL biomarker in a novel human genomic NOTCH3 transgenic mouse model with early progressive vascular NOTCH3 accumulation

[1] Joutel A(1996)Notch3 mutations in CADASIL, a hereditary adult-onset condition causing stroke and dementia Nature 383 707-10

[2] Corpechot C(2000)The ectodomain of the Notch3 receptor accumulates within the cerebrovasculature of CADASIL patients J Clin Invest 105 597-605

[3] Ducros A(1995)Systemic vascular smooth muscle cell impairment in cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy Acta Neuropathol 89 500-12

[4] Vahedi K(2001)Reduced cerebrovascular CO(2) reactivity in CADASIL: A transcranial Doppler sonography study Stroke 32 17-21

[5] Chabriat H(2009)Cadasil Lancet Neurol 8 643-53

[6] Mouton P(2011)PNAS Plus: Hypomorphic Notch 3 alleles link Notch signaling to ischemic cerebral small-vessel disease Proc Natl Acad Sci U S A 108 E128-35

[7] Joutel A(2009)Distinct phenotypic and functional features of CADASIL mutations in the Notch3 ligand binding domain Brain 132 1601-12

[8] Andreux F(2003)Transgenic mice expressing mutant Notch3 develop vascular alterations characteristic of cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy Am J Pathol 162 329-42

[9] Gaulis S(2010)Cerebrovascular dysfunction and microcirculation rarefaction precede white matter lesions in a mouse genetic model of cerebral ischemic small vessel disease J Clin Invest 120 433-45

[10] Domenga V(2005)Mice carrying a R142C Notch 3 knock-in mutation do not develop a CADASIL-like phenotype Genesis 41 13-22

[1] Joutel A(1996)Notch3 mutations in CADASIL, a hereditary adult-onset condition causing stroke and dementia Nature 383 707-10

[2] Corpechot C(2000)The ectodomain of the Notch3 receptor accumulates within the cerebrovasculature of CADASIL patients J Clin Invest 105 597-605

[3] Ducros A(1995)Systemic vascular smooth muscle cell impairment in cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy Acta Neuropathol 89 500-12

[4] Vahedi K(2001)Reduced cerebrovascular CO(2) reactivity in CADASIL: A transcranial Doppler sonography study Stroke 32 17-21

[5] Chabriat H(2009)Cadasil Lancet Neurol 8 643-53

[6] Mouton P(2011)PNAS Plus: Hypomorphic Notch 3 alleles link Notch signaling to ischemic cerebral small-vessel disease Proc Natl Acad Sci U S A 108 E128-35

[7] Joutel A(2009)Distinct phenotypic and functional features of CADASIL mutations in the Notch3 ligand binding domain Brain 132 1601-12

[8] Andreux F(2003)Transgenic mice expressing mutant Notch3 develop vascular alterations characteristic of cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy Am J Pathol 162 329-42

[9] Gaulis S(2010)Cerebrovascular dysfunction and microcirculation rarefaction precede white matter lesions in a mouse genetic model of cerebral ischemic small vessel disease J Clin Invest 120 433-45

[10] Domenga V(2005)Mice carrying a R142C Notch 3 knock-in mutation do not develop a CADASIL-like phenotype Genesis 41 13-22