Novel Murine Models of Cerebral Cavernous Malformations

Novel Chronic Mouse Model of Cerebral Cavernous Malformations

(2020) Cécile Cardoso et al.   STROKE

Symptomatic Brain Hemorrhages from Cavernous Angioma After Botulinum Toxin Injections, a Role of TLR/MEKK3 Mechanism? Case Report and Review of the Literature

(2020) Janne Koskimäki et al.   World Neurosurgery

Rho Kinase Inhibition Blunts Lesion Development and Hemorrhage in Murine Models of Aggressive Pdcd10/Ccm3 Disease

(2019) Robert Shenkar et al.   STROKE

A Brain-Targeted Orally Available ROCK2 Inhibitor Benefits Mild and Aggressive Cavernous Angioma Disease

(2019) Lisa McKerracher et al.   Translational Stroke Research

VEGF signalling enhances lesion burden in KRIT1 deficient mice

(2019) Peter V. DiStefano et al.   JOURNAL OF CELLULAR AND MOLECULAR MEDICINE

Phenotypic characterization of murine models of cerebral cavernous malformations

(2018) Hussein A. Zeineddine et al.   LABORATORY INVESTIGATION

Systematic pharmacological screens uncover novel pathways involved in cerebral cavernous malformations

(2018) Cécile Otten et al.   EMBO Molecular Medicine

Cerebral cavernous malformations form an anticoagulant vascular domain in humans and mice

(2018) Miguel Alejandro Lopez-Ramirez et al.   BLOOD

Ponatinib (AP24534) inhibits MEKK3-KLF signaling and prevents formation and progression of cerebral cavernous malformations

(2018) Jaesung P. Choi et al.   Science Advances

Thrombospondin1 (TSP1) replacement prevents cerebral cavernous malformations

(2017) Miguel Alejandro Lopez-Ramirez et al.   JOURNAL OF EXPERIMENTAL MEDICINE

Endothelial TLR4 and the microbiome drive cerebral cavernous malformations

(2017) Alan T. Tang et al.   NATURE

Combined HMG-COA reductase and prenylation inhibition in treatment of CCM

(2017) Sayoko Nishimura et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Plasma Biomarkers of Inflammation Reflect Seizures and Hemorrhagic Activity of Cerebral Cavernous Malformations

(2017) Romuald Girard et al.   Translational Stroke Research

B-Cell Depletion Reduces the Maturation of Cerebral Cavernous Malformations in Murine Models

(2016) Changbin Shi et al.   Journal of Neuroimmune Pharmacology

Propranolol stops progressive multiple cerebral cavernoma in an adult patient

(2016) Matthias Reinhard et al.   JOURNAL OF THE NEUROLOGICAL SCIENCES

Clinical course of untreated cerebral cavernous malformations: a meta-analysis of individual patient data

(2016) Margaret A Horne et al.   LANCET NEUROLOGY

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

(2016) Zinan Zhou et al.   NATURE

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

(2016) Huanjiao Jenny Zhou et al.   NATURE MEDICINE

RhoA Kinase Inhibition With Fasudil Versus Simvastatin in Murine Models of Cerebral Cavernous Malformations

(2016) Robert Shenkar et al.   STROKE

Propranolol Treatment of Cavernous Malformations with Symptomatic Hemorrhage

(2016) Joseph M. Zabramski et al.   World Neurosurgery

Defective autophagy is a key feature of cerebral cavernous malformations

(2015) Saverio Marchi et al.   EMBO Molecular Medicine

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

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

Sulindac metabolites decrease cerebrovascular malformations inCCM3-knockout mice

(2015) Luca Bravi et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

CCM-3/STRIPAK promotes seamless tube extension through endocytic recycling

(2015) Benjamin Lant et al.   Nature Communications

Exceptional aggressiveness of cerebral cavernous malformation disease associated with PDCD10 mutations

(2014) Robert Shenkar et al.   GENETICS IN MEDICINE

Lack of CCM1 induces hypersprouting and impairs response to flow

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

Lesions from patients with sporadic cerebral cavernous malformations harbor somatic mutations in the CCM genes: evidence for a common biochemical pathway for CCM pathogenesis

(2014) David A. McDonald et al.   HUMAN MOLECULAR GENETICS

CCM1–ICAP-1 complex controls β1 integrin–dependent endothelial contractility and fibronectin remodeling

(2013) Eva Faurobert et al.   JOURNAL OF CELL BIOLOGY

EndMT contributes to the onset and progression of cerebral cavernous malformations

(2013) Luigi Maddaluno et al.   NATURE

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

Ccm3 functions in a manner distinct from Ccm1 and Ccm2 in a zebrafish model of CCM vascular disease

(2011) Bilge Yoruk et al.   DEVELOPMENTAL BIOLOGY

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

TAK1 in brain endothelial cells mediates fever and lethargy

(2011) Dirk A. Ridder et al.   JOURNAL OF EXPERIMENTAL MEDICINE

Loss ofcerebral cavernous malformation 3(Ccm3) in neuroglia leads to CCM and vascular pathology

(2011) Angeliki Louvi et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Fasudil Decreases Lesion Burden in a Murine Model of Cerebral Cavernous Malformation Disease

(2011) David A. McDonald et al.   STROKE

A novel mouse model of cerebral cavernous malformations based on the two-hit mutation hypothesis recapitulates the human disease

(2010) D. A. McDonald et al.   HUMAN MOLECULAR GENETICS

Rho Kinase Inhibition Rescues the Endothelial Cell Cerebral Cavernous Malformation Phenotype

(2010) Asya L. Borikova et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Cerebral cavernous malformations proteins inhibit Rho kinase to stabilize vascular integrity

(2010) Rebecca A. Stockton et al.   JOURNAL OF EXPERIMENTAL MEDICINE

KRIT1 Regulates the Homeostasis of Intracellular Reactive Oxygen Species

(2010) Luca Goitre et al.   PLoS One

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

Tissue-specific conditional CCM2 knockout mice establish the essential role of endothelial CCM2 in angiogenesis: implications for human cerebral cavernous malformations

(2009) G. Boulday et al.   Disease Models & Mechanisms

Functional analyses of human and zebrafish 18-amino acid in-frame deletion pave the way for domain mapping of the cerebral cavernous malformation 3 protein

(2009) Katrin Voss et al.   HUMAN MUTATION

Cerebral Cavernous Malformation 2 Protein Promotes Smad Ubiquitin Regulatory Factor 1-mediated RhoA Degradation in Endothelial Cells

(2009) Lisa E. S. Crose et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

The cerebral cavernous malformation signaling pathway promotes vascular integrity via Rho GTPases

(2009) Kevin J Whitehead et al.   NATURE MEDICINE

Regulation of cardiovascular development and integrity by the heart of glass–cerebral cavernous malformation protein pathway

(2009) Benjamin Kleaveland et al.   NATURE MEDICINE

CEREBRAL CAVERNOUS MALFORMATIONS

(2009) Judith Gault et al.   NEUROSURGERY

Immune Response in Human Cerebral Cavernous Malformations

(2009) Changbin Shi et al.   STROKE

Efficient, inducible Cre-recombinase activation in vascular endothelium

(2008) Suzanne Claxton et al.   GENESIS

ccm1 cell autonomously regulates endothelial cellular morphogenesis and vascular tubulogenesis in zebrafish

(2008) Benjamin M. Hogan et al.   HUMAN MOLECULAR GENETICS

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