Cerebral cavernous malformations are driven by ADAMTS5 proteolysis of versican

Endothelial cell clonal expansion in the development of cerebral cavernous malformations

(2019) Matteo Malinverno et al.   Nature Communications

Exosites in Hypervariable Loops of ADAMTS Spacer Domains control Substrate Recognition and Proteolysis

(2019) Salvatore Santamaria et al.   Scientific Reports

Adamts5 −/− Mice Exhibit Altered Aggrecan Proteolytic Profiles That Correlate With Ascending Aortic Anomalies

(2019) Loren E. Dupuis et al.   ARTERIOSCLEROSIS THROMBOSIS AND VASCULAR BIOLOGY

Distinct cellular roles for PDCD10 define a gut-brain axis in cerebral cavernous malformation

(2019) Alan T. Tang et al.   Science Translational Medicine

Role of ADAMTS-5 in Aortic Dilatation and Extracellular Matrix RemodelingHighlights

(2018) Marika Fava et al.   ARTERIOSCLEROSIS THROMBOSIS AND VASCULAR BIOLOGY

Control of cardiac jelly dynamics by NOTCH1 and NRG1 defines the building plan for trabeculation

(2018) Gonzalo del Monte-Nieto et al.   NATURE

A molecular atlas of cell types and zonation in the brain vasculature

(2018) Michael Vanlandewijck et al.   NATURE

Cerebral Cavernous Malformations Develop Through Clonal Expansion of Mutant Endothelial Cells

(2018) Matthew R. Detter et al.   CIRCULATION RESEARCH

Versican Proteolysis by ADAMTS Proteases and Its Influence on Sex Steroid Receptor Expression in Uterine Leiomyoma

(2017) Ndeye-Aicha Gueye et al.   JOURNAL OF CLINICAL ENDOCRINOLOGY & METABOLISM

Endothelial TLR4 and the microbiome drive cerebral cavernous malformations

(2017) Alan T. Tang et al.   NATURE

Synopsis of Guidelines for the Clinical Management of Cerebral Cavernous Malformations: Consensus Recommendations Based on Systematic Literature Review by the Angioma Alliance Scientific Advisory Board Clinical Experts Panel

(2017) Amy Akers et al.   NEUROSURGERY

Immunoregulatory roles of versican proteolysis in the myeloma microenvironment

(2016) C. Hope et al.   BLOOD

Versican Deficiency Significantly Reduces Lung Inflammatory Response Induced by Polyinosine-Polycytidylic Acid Stimulation

(2016) Inkyung Kang et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Micro-computed tomography in murine models of cerebral cavernous malformations as a paradigm for brain disease

(2016) Romuald Girard et al.   JOURNAL OF NEUROSCIENCE METHODS

Cerebral cavernous malformations arise from endothelial gain of MEKK3–KLF2/4 signalling

(2016) Zinan Zhou et al.   NATURE

Erratum: Endothelial exocytosis of angiopoietin-2 resulting from CCM3 deficiency contributes to cerebral cavernous malformation

(2016) Huanjiao Jenny Zhou et al.   NATURE MEDICINE

Increased Number of White Matter Lesions in Patients with Familial Cerebral Cavernous Malformations

(2015) M.J. Golden et al.   AMERICAN JOURNAL OF NEURORADIOLOGY

The Cerebral Cavernous Malformation Pathway Controls Cardiac Development via Regulation of Endocardial MEKK3 Signaling and KLF Expression

(2015) Zinan Zhou et al.   DEVELOPMENTAL CELL

KLF4 is a key determinant in the development and progression of cerebral cavernous malformations

(2015) R. Cuttano et al.   EMBO Molecular Medicine

The cerebral cavernous malformation proteins CCM2L and CCM2 prevent the activation of the MAP kinase MEKK3

(2015) Xavier Cullere et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Structural Insights into the Molecular Recognition between Cerebral Cavernous Malformation 2 and Mitogen-Activated Protein Kinase Kinase Kinase 3

(2015) Xiaoyan Wang et al.   STRUCTURE

Structure and vascular function of MEKK3–cerebral cavernous malformations 2 complex

(2015) Oriana S. Fisher et al.   Nature Communications

Lack of CCM1 induces hypersprouting and impairs response to flow

(2014) Tara M. Mleynek et al.   HUMAN MOLECULAR GENETICS

KRIT1 Protein Depletion Modifies Endothelial Cell Behavior via Increased Vascular Endothelial Growth Factor (VEGF) Signaling

(2014) Peter V. DiStefano et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Signaling pathways and the cerebral cavernous malformations proteins: lessons from structural biology

(2013) Oriana S. Fisher et al.   CELLULAR AND MOLECULAR LIFE SCIENCES

Dynamic Contrast-Enhanced MRI Evaluation of Cerebral Cavernous Malformations

(2013) Blaine L. Hart et al.   Translational Stroke Research

Ultrastructural analysis of vascular features in cerebral cavernous malformations

(2012) Gamze Tanriover et al.   CLINICAL NEUROLOGY AND NEUROSURGERY

Dynamic Regulation of the Cerebral Cavernous Malformation Pathway Controls Vascular Stability and Growth

(2012) Xiangjian Zheng et al.   DEVELOPMENTAL CELL

Untreated clinical course of cerebral cavernous malformations: a prospective, population-based cohort study

(2012) Rustam Al-Shahi Salman et al.   LANCET NEUROLOGY

Proteoglycan degradation by the ADAMTS family of proteinases

(2011) Heather Stanton et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

Altered versican cleavage in ADAMTS5 deficient mice; A novel etiology of myxomatous valve disease

(2011) Loren E. Dupuis et al.   DEVELOPMENTAL BIOLOGY

Partially redundant functions of Adamts1 and Adamts4 in the perinatal development of the renal medulla

(2011) Derek Boerboom et al.   DEVELOPMENTAL DYNAMICS

Mutations in 2 distinct genetic pathways result in cerebral cavernous malformations in mice

(2011) Aubrey C. Chan et al.   JOURNAL OF CLINICAL INVESTIGATION

Developmental timing of CCM2 loss influences cerebral cavernous malformations in mice

(2011) Gwénola Boulday et al.   JOURNAL OF EXPERIMENTAL MEDICINE

A Transgenic Approach to Identify Thyroxine Transporter-Expressing Structures in Brain Development

(2011) M.-F. Lang et al.   JOURNAL OF NEUROENDOCRINOLOGY

Cooperation of two ADAMTS metalloproteases in closure of the mouse palate identifies a requirement for versican proteolysis in regulating palatal mesenchyme proliferation

(2010) H. Enomoto et al.   DEVELOPMENT

Versican Facilitates Chondrocyte Differentiation and Regulates Joint Morphogenesis

(2010) Kanyamas Choocheep et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Ephrin-B2 controls VEGF-induced angiogenesis and lymphangiogenesis

(2010) Yingdi Wang et al.   NATURE

Cerebral cavernous malformation protein CCM1 inhibits sprouting angiogenesis by activating DELTA-NOTCH signaling

(2010) Joycelyn Wüstehube et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Stabilization of VEGFR2 Signaling by Cerebral Cavernous Malformation 3 Is Critical for Vascular Development

(2010) Y. He et al.   Science Signaling

ADAMTS Metalloproteases Generate Active Versican Fragments that Regulate Interdigital Web Regression

(2009) Daniel R. McCulloch et al.   DEVELOPMENTAL CELL

Adamts5, the gene encoding a proteoglycan-degrading metalloprotease, is expressed by specific cell lineages during mouse embryonic development and in adult tissues

(2009) Daniel R. McCulloch et al.   GENE EXPRESSION PATTERNS

Endocardial Brg1 Represses ADAMTS1 to Maintain the Microenvironment for Myocardial Morphogenesis

(2008) Kryn Stankunas et al.   DEVELOPMENTAL CELL

Efficient, inducible Cre-recombinase activation in vascular endothelium

(2008) Suzanne Claxton et al.   GENESIS

Biallelic somatic and germline mutations in cerebral cavernous malformations (CCMs): evidence for a two-hit mechanism of CCM pathogenesis

(2008) Amy L. Akers et al.   HUMAN MOLECULAR GENETICS

Hemorrhage From Cavernous Malformations of the Brain

(2008) Rustam Al-Shahi Salman et al.   STROKE

Cerebral cavernous malformations: congruency of histopathological features with the current clinical definition

(2007) J M Frischer et al.   JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY