Abnormal recruitment of extracellular matrix proteins by excess Notch3 ECD: a new pathomechanism in CADASIL (Q36904906)

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scientific article published on 6 May 2013

Abnormal recruitment of extracellular matrix proteins by excess Notch3ECD: a new pathomechanism in CADASIL

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English	Abnormal recruitment of extracellular matrix proteins by excess Notch3 ECD: a new pathomechanism in CADASIL	scientific article published on 6 May 2013	Abnormal recruitment of extracellular matrix proteins by excess Notch3ECD: a new pathomechanism in CADASIL

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scholarly article

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29 February 2020

Abnormal recruitment of extracellular matrix proteins by excess Notch3 ECD: a new pathomechanism in CADASIL (English)

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29 February 2020

extracellular matrix

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Emmanuel Cognat

8

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Marie Monet-Leprêtre

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Iman Haddad

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Céline Baron-Menguy

3

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Maï Fouillot-Panchal

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Meriem Riani

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29 February 2020

Valérie Domenga-Denier

6

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Claire Dussaule

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6 May 2013

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29 February 2020

136

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Pt 6

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1830-1845

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describes a project that uses

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Notch signalling in smooth muscle cells during development and disease

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Hypomorphic Notch 3 alleles link Notch signaling to ischemic cerebral small-vessel disease

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Increased neovascularization in mice lacking tissue inhibitor of metalloproteinases-3.

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Amyloid-like aggregates sequester numerous metastable proteins with essential cellular functions

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Notch3 is critical for proper angiogenesis and mural cell investment

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Cerebrovascular dysfunction and microcirculation rarefaction precede white matter lesions in a mouse genetic model of cerebral ischemic small vessel disease

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29 September 2017

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29 September 2017

CADASIL mutations enhance spontaneous multimerization of NOTCH3.

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29 September 2017

The canonical Notch signaling pathway: unfolding the activation mechanism

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29 September 2017

Distinct phenotypic and functional features of CADASIL mutations in the Notch3 ligand binding domain

1 reference

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29 September 2017

Congruence between NOTCH3 mutations and GOM in 131 CADASIL patients

1 reference

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29 September 2017

Tissue inhibitors of metalloproteinases in cell signaling: metalloproteinase-independent biological activities.

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Identification of the extracellular matrix (ECM) binding motifs of tissue inhibitor of metalloproteinases (TIMP)-3 and effective transfer to TIMP-1.

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Notch3 is required for arterial identity and maturation of vascular smooth muscle cells

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29 September 2017

Pathogenic mutations associated with cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy differently affect Jagged1 binding and Notch3 activity via the RBP/JK signaling Pathway

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A novel function for tissue inhibitor of metalloproteinases-3 (TIMP3): inhibition of angiogenesis by blockage of VEGF binding to VEGF receptor-2

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Skin biopsy immunostaining with a Notch3 monoclonal antibody for CADASIL diagnosis

1 reference

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The ectodomain of the Notch3 receptor accumulates within the cerebrovasculature of CADASIL patients

1 reference

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Strong clustering and stereotyped nature of Notch3 mutations in CADASIL patients

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Notch3 mutations in CADASIL, a hereditary adult-onset condition causing stroke and dementia

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29 September 2017

Mutations in the tissue inhibitor of metalloproteinases-3 (TIMP3) in patients with Sorsby's fundus dystrophy

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Systemic vascular smooth muscle cell impairment in cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy

1 reference

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CADASIL-associated Notch3 mutations have differential effects both on ligand binding and ligand-induced Notch3 receptor signaling through RBP-Jk

1 reference

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27 June 2018

Transgenic mice expressing mutant Notch3 develop vascular alterations characteristic of cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy

1 reference

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The archetypal R90C CADASIL-NOTCH3 mutation retains NOTCH3 function in vivo.

1 reference

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Evaluation of diagnostic NOTCH3 immunostaining in CADASIL.

1 reference

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4 September 2018

Systemic vascular smooth muscle cell impairment in cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy

1 reference

https://api.crossref.org/works/10.1093%2FBRAIN%2FAWT092

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1 reference

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Co-aggregate formation of CADASIL-mutant NOTCH3: a single-particle analysis

1 reference

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based on heuristic

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Spectrum of mutations in biopsy-proven CADASIL: implications for diagnostic strategies

1 reference

https://pubmed.ncbi.nlm.nih.gov/23649698

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Identifiers

10.1093/BRAIN/AWT092

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29 February 2020

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Europe PubMed Central

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29 February 2020

1 reference

Europe PubMed Central

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29 February 2020

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