CADASIL brain vessels show a HTRA1 loss-of-function profile

Therapeutic antibody targeting of Notch3 signaling prevents mural cell loss in CADASIL

(2017) Arturo I. Machuca-Parra et al.   JOURNAL OF EXPERIMENTAL MEDICINE

Serum amyloid P component: A novel potential player in vessel degeneration in CADASIL

(2017) Akihito Nagatoshi et al.   JOURNAL OF THE NEUROLOGICAL SCIENCES

New Insight Into the Pathogenesis of Cerebral Small-Vessel Diseases

(2017) Kristin Müller et al.   STROKE

Neuropathological diagnosis of vascular cognitive impairment and vascular dementia with implications for Alzheimer’s disease

(2016) Raj N. Kalaria   ACTA NEUROPATHOLOGICA

Reducing Timp3 or vitronectin ameliorates disease manifestations in CADASIL mice

(2016) Carmen Capone et al.   ANNALS OF NEUROLOGY

Therapeutic NOTCH3 cysteine correction in CADASIL using exon skipping:in vitroproof of concept

(2016) Julie W. Rutten et al.   BRAIN

Sequestration of cellular interacting partners by protein aggregates: implication in a loss-of-function pathology

(2016) Hui Yang et al.   FEBS Journal

Increased Notch3 Activity Mediates Pathological Changes in Structure of Cerebral ArteriesNovelty and Significance

(2016) Celine Baron-Menguy et al.   HYPERTENSION

Distinct molecular mechanisms ofHTRA1mutants in manifesting heterozygotes with CARASIL

(2016) Hiroaki Nozaki et al.   NEUROLOGY

Systems proteomic analysis reveals that clusterin and tissue inhibitor of metalloproteinases 3 increase in leptomeningeal arteries affected by cerebral amyloid angiopathy

(2016) A. Manousopoulou et al.   NEUROPATHOLOGY AND APPLIED NEUROBIOLOGY

Mechanistic insights into a TIMP3-sensitive pathway constitutively engaged in the regulation of cerebral hemodynamics

(2016) Carmen Capone et al.   eLife

Pericytes are involved in the pathogenesis of cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy

(2015) Mitrajit Ghosh et al.   ANNALS OF NEUROLOGY

HeterozygousHTRA1mutations are associated with autosomal dominant cerebral small vessel disease

(2015) Edgard Verdura et al.   BRAIN

Homozygous NOTCH3 null mutation and impaired NOTCH3 signaling in recessive early-onset arteriopathy and cavitating leukoencephalopathy

(2015) T. Pippucci et al.   EMBO Molecular Medicine

Perturbations of the cerebrovascular matrisome: A convergent mechanism in small vessel disease of the brain?

(2015) Anne Joutel et al.   JOURNAL OF CEREBRAL BLOOD FLOW AND METABOLISM

Genetic factors in cerebral small vessel disease and their impact on stroke and dementia

(2015) Christof Haffner et al.   JOURNAL OF CEREBRAL BLOOD FLOW AND METABOLISM

A Human Platelet Receptor Protein Microarray Identifies the High Affinity Immunoglobulin E Receptor Subunit α (FcεR1α) as an Activating Platelet Endothelium Aggregation Receptor 1 (PEAR1) Ligand

(2015) Yi Sun et al.   MOLECULAR & CELLULAR PROTEOMICS

Mouse model of CADASIL reveals novel insights into Notch3 function in adult hippocampal neurogenesis

(2015) Fanny Ehret et al.   NEUROBIOLOGY OF DISEASE

Clusterin/Apolipoprotein J immunoreactivity is associated with white matter damage in cerebral small vessel diseases

(2015) Lucinda Craggs et al.   NEUROPATHOLOGY AND APPLIED NEUROBIOLOGY

Cysteine-Sparing CADASIL Mutations inNOTCH3Show Proaggregatory Properties In Vitro

(2015) Frank Arne Wollenweber et al.   STROKE

Combined deficiency of Notch1 and Notch3 causes pericyte dysfunction, models CADASIL and results in arteriovenous malformations

(2015) Natalie M. Kofler et al.   Scientific Reports

The Small Leucine-Rich Proteoglycan BGN Accumulates in CADASIL and Binds to NOTCH3

(2015) Xiaojie Zhang et al.   Translational Stroke Research

Microvascular Pathology and Morphometrics of Sporadic and Hereditary Small Vessel Diseases of the Brain

(2014) Lucinda J.L. Craggs et al.   BRAIN PATHOLOGY

CADASIL and CARASIL

(2014) Saara Tikka et al.   BRAIN PATHOLOGY

Latent NOTCH3 epitopes unmasked in CADASIL and regulated by protein redox state

(2014) Xiaojie Zhang et al.   BRAIN RESEARCH

Interpretation ofNOTCH3mutations in the diagnosis of CADASIL

(2014) Julie W Rutten et al.   EXPERT REVIEW OF MOLECULAR DIAGNOSTICS

Remodelling the extracellular matrix in development and disease

(2014) Caroline Bonnans et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

Vascular accumulation of the small leucine-rich proteoglycan decorin in CADASIL

(2014) Soo Jung Lee et al.   NEUROREPORT

Cerebral small vessel disease-related protease HtrA1 processes latent TGF-β binding protein 1 and facilitates TGF-β signaling

(2014) Nathalie Beaufort et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Features of Cerebral Autosomal Recessive Arteriopathy With Subcortical Infarcts and Leukoencephalopathy

(2014) Hiroaki Nozaki et al.   STROKE

Archetypal Arg169Cys Mutation in NOTCH3 Does Not Drive the Pathogenesis in Cerebral Autosomal Dominant Arteriopathy With Subcortical Infarcts and Leucoencephalopathy via a Loss-of-Function Mechanism

(2014) Emmanuel Cognat et al.   STROKE

Sequestration of latent TGF-β binding protein 1 into CADASIL-related Notch3-ECD deposits

(2014) Jessica Kast et al.   Acta Neuropathologica Communications

The Emerging Roles of HTRA1 in Musculoskeletal Disease

(2013) André Nicki Tiaden et al.   AMERICAN JOURNAL OF PATHOLOGY

Label-free quantification in clinical proteomics

(2013) Dominik A. Megger et al.   BIOCHIMICA ET BIOPHYSICA ACTA-PROTEINS AND PROTEOMICS

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

(2013) Marie Monet-Leprêtre et al.   BRAIN

Loss-of-function Mutation in the NOTCH3 Gene: Simply a Polymorphism?

(2013) Anne Joutel   HUMAN MUTATION

Hypomorphic NOTCH3 Alleles Do Not Cause CADASIL in Humans

(2013) Julie W. Rutten et al.   HUMAN MUTATION

Review: Cerebral amyloid angiopathy, prion angiopathy, CADASIL and the spectrum of protein elimination failure angiopathies (PEFA) in neurodegenerative disease with a focus on therapy

(2013) R. O. Carare et al.   NEUROPATHOLOGY AND APPLIED NEUROBIOLOGY

KIAA1199, a deafness gene of unknown function, is a new hyaluronan binding protein involved in hyaluronan depolymerization

(2013) H. Yoshida et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

A novel Notch3 deletion mutation in a Chinese patient with cerebral autosomal dominant arteriopathy with subcortical infarcts and leucoencephalopathy (CADASIL)

(2012) Fan Weiming et al.   JOURNAL OF CLINICAL NEUROSCIENCE

Neurodegeneration as a consequence of failed mitochondrial maintenance

(2011) Mariusz Karbowski et al.   ACTA NEUROPATHOLOGICA

Amyloid-like Aggregates Sequester Numerous Metastable Proteins with Essential Cellular Functions

(2011) Heidi Olzscha et al.   CELL

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

(2011) Marco Duering et al.   HUMAN MOLECULAR GENETICS

Cerebral small-vessel disease protein HTRA1 controls the amount of TGF-β1 via cleavage of proTGF-β1

(2011) Atsushi Shiga et al.   HUMAN MOLECULAR GENETICS

HTRA proteases: regulated proteolysis in protein quality control

(2011) Tim Clausen et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

Overexpression of HTRA1 Leads to Ultrastructural Changes in the Elastic Layer of Bruch's Membrane via Cleavage of Extracellular Matrix Components

(2011) Sarah Vierkotten et al.   PLoS One

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

(2011) J. F. Arboleda-Velasquez et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Increased expression of multifunctional serine protease, HTRA1, in retinal pigment epithelium induces polypoidal choroidal vasculopathy in mice

(2011) A. Jones et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Notch3 Is Critical for Proper Angiogenesis and Mural Cell Investment

(2010) Hua Liu et al.   CIRCULATION RESEARCH

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

(2010) Anne Joutel et al.   JOURNAL OF CLINICAL INVESTIGATION

CADASIL mutations enhance spontaneous multimerization of NOTCH3

(2009) C. Opherk et al.   HUMAN MOLECULAR GENETICS

CADASIL

(2009) Hugues Chabriat et al.   LANCET NEUROLOGY

TACE-Mediated Ectodomain Shedding of the Type I TGF-β Receptor Downregulates TGF-β Signaling

(2009) Cheng Liu et al.   MOLECULAR CELL

Universal sample preparation method for proteome analysis

(2009) Jacek R Wiśniewski et al.   NATURE METHODS

Association of HTRA1 Mutations and Familial Ischemic Cerebral Small-Vessel Disease

(2009) Kenju Hara et al.   NEW ENGLAND JOURNAL OF MEDICINE

The Extracellular Matrix: Not Just Pretty Fibrils

(2009) R. O. Hynes   SCIENCE