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Nature, ISSN 0028-0836, 04/2016, Volume 532, Issue 7597, pp. 122 - 126
Journal Article
Journal of Experimental Medicine, ISSN 0022-1007, 04/2010, Volume 207, Issue 4, pp. 881 - 896
Endothelial cell-cell junctions regulate vascular permeability, vasculogenesis, and angiogenesis. Familial cerebral cavernous malformations (CCMs) in humans... 
CELL PERMEABILITY | PATHOGENESIS | MEDICINE, RESEARCH & EXPERIMENTAL | IMMUNE-RESPONSE | P38 MAPK ACTIVATION | ACUTE LUNG INJURY | VE-CADHERIN | CCM2 | IMMUNOLOGY | 2-HIT MECHANISM | BRAIN | ENDOTHELIAL BARRIER FUNCTION | RNA, Small Interfering - genetics | Humans | Capillary Permeability - drug effects | Endothelium, Vascular - drug effects | rhoA GTP-Binding Protein - metabolism | Animal Structures - blood supply | Mutation - physiology | Phosphorylation - genetics | rho-Associated Kinases - metabolism | Edema, Cardiac - pathology | Phosphorylation - drug effects | Endothelial Cells - metabolism | 1-(5-Isoquinolinesulfonyl)-2-Methylpiperazine - therapeutic use | Hemangioma, Cavernous, Central Nervous System - drug therapy | Edema, Cardiac - genetics | Hemangioma, Cavernous, Central Nervous System - pathology | Signal Transduction - drug effects | Endothelium, Vascular - metabolism | Endothelium, Vascular - pathology | Mice | Myosin Light Chains - metabolism | Animal Structures - metabolism | Protein Binding - physiology | Capillary Permeability - physiology | KRIT1 Protein | Microtubule-Associated Proteins - genetics | Microtubule-Associated Proteins - metabolism | 1-(5-Isoquinolinesulfonyl)-2-Methylpiperazine - analogs & derivatives | Hemangioma, Cavernous, Central Nervous System - metabolism | rho-Associated Kinases - antagonists & inhibitors | Brain Edema - genetics | Edema, Cardiac - drug therapy | Mice, Mutant Strains | Pulmonary Edema - pathology | Microfilament Proteins - metabolism | Microfilament Proteins - genetics | Proto-Oncogene Proteins - metabolism | Pulmonary Edema - genetics | Brain Edema - pathology | Mice, Inbred C57BL | Proto-Oncogene Proteins - genetics | Carrier Proteins - genetics | Animals | Carrier Proteins - metabolism | Protein Kinase Inhibitors - therapeutic use | 1-(5-Isoquinolinesulfonyl)-2-Methylpiperazine - pharmacology | Signal Transduction - physiology | Brain Edema - drug therapy | Protein Kinase Inhibitors - pharmacology | Endothelial Cells - drug effects | Index Medicus
Journal Article
Proceedings of the National Academy of Sciences, ISSN 0027-8424, 07/2015, Volume 112, Issue 27, pp. 8421 - 8426
Cerebral cavernous malformation (CCM) is a disease of the central nervous system causing hemorrhage-prone multiple lumen vascular malformations and very severe... 
Cerebral cavernous malformation | Sulindac metabolites | catenin | Endothelial cells | Vascular pathology | cerebral cavernous malformation | beta-catenin | endothelial cells | SIGNALING CONTROLS | MULTIDISCIPLINARY SCIENCES | BLOOD-BRAIN-BARRIER | EFFECTOR | CEREBRAL CAVERNOUS MALFORMATIONS | vascular pathology | PATHWAY | RAP1 | DISEASE | JUNCTIONS | GROWTH | sulindac metabolites | Immunohistochemistry | Central Nervous System Neoplasms - metabolism | Endothelial Cells - metabolism | Central Nervous System Neoplasms - genetics | Intracellular Signaling Peptides and Proteins - metabolism | Signal Transduction - genetics | Reverse Transcriptase Polymerase Chain Reaction | Hemangioma, Cavernous, Central Nervous System - drug therapy | beta Catenin - metabolism | Hemangioma, Cavernous, Central Nervous System - genetics | Hemangioma, Cavernous, Central Nervous System - metabolism | beta Catenin - genetics | Anti-Inflammatory Agents, Non-Steroidal - pharmacology | Mice, Knockout | Intracellular Signaling Peptides and Proteins - deficiency | Sulindac - analogs & derivatives | Animals | Signal Transduction - drug effects | Gene Expression Regulation, Neoplastic - drug effects | Central Nervous System Neoplasms - drug therapy | Intracellular Signaling Peptides and Proteins - genetics | Sulindac - pharmacology | Transforming Growth Factor beta - metabolism | Disease Models, Animal | Usage | Sulindac | Genetic aspects | Research | Drug therapy | Health aspects | Cerebrovascular disease | Nervous system | Pharmacology | Rodents | Medical treatment | Cells | Index Medicus | Biological Sciences | β-catenin | Other Medical Sciences not elsewhere specified | Other Medical Sciences | Medical and Health Sciences | Medicin och hälsovetenskap | Annan medicin och hälsovetenskap | Övrig annan medicin och hälsovetenskap
Journal Article
Journal of Experimental Medicine, ISSN 0022-1007, 11/2017, Volume 214, Issue 11, pp. 3331 - 3346
Journal Article
Nature Medicine, ISSN 1078-8956, 09/2016, Volume 22, Issue 9, pp. 1033 - 1042
Journal Article
EMBO Molecular Medicine, ISSN 1757-4676, 01/2016, Volume 8, Issue 1, pp. 6 - 24
Cerebral cavernous malformations ( CCM s) are vascular malformations located within the central nervous system often resulting in cerebral hemorrhage.... 
endothelial cells | TGF | β | KLF | 4 | BMP | MT | End | CCM | KLF4 | EndMT | Endothelial cells | TGFβ-BMP | MEDICINE, RESEARCH & EXPERIMENTAL | RHO KINASE | BLOOD-BRAIN-BARRIER | KRUPPEL-LIKE FACTORS | ENDOTHELIAL-MESENCHYMAL TRANSITION | TGF beta-BMP | SIGNALING PATHWAY | VASCULAR INTEGRITY | SHEAR-STRESS | VE-CADHERIN | GROWTH-FACTOR | TRANSCRIPTION FACTOR | Cell Proliferation | KRIT1 Protein | Microtubule-Associated Proteins - genetics | Microtubule-Associated Proteins - metabolism | Humans | Hemangioma, Cavernous, Central Nervous System - genetics | Hemangioma, Cavernous, Central Nervous System - metabolism | Bone Morphogenetic Protein 6 - genetics | RNA Interference | Kruppel-Like Transcription Factors - metabolism | HEK293 Cells | Disease Models, Animal | Proto-Oncogene Proteins - metabolism | Proto-Oncogene Proteins - antagonists & inhibitors | Signal Transduction | Endothelial Cells - metabolism | Mice, Inbred C57BL | Bone Morphogenetic Protein 6 - antagonists & inhibitors | Proto-Oncogene Proteins - genetics | Microtubule-Associated Proteins - antagonists & inhibitors | Mitogen-Activated Protein Kinase 7 - metabolism | Disease Progression | Mice, Knockout | Animals | Bone Morphogenetic Protein 6 - metabolism | Hemangioma, Cavernous, Central Nervous System - pathology | Endothelial Cells - cytology | Smad1 Protein - metabolism | Mice | Mutation | Kruppel-Like Transcription Factors - antagonists & inhibitors | Kruppel-Like Transcription Factors - genetics | Transforming Growth Factor beta - metabolism | Brain | Bone morphogenetic proteins | Transforming growth factors | Mortality | Stem cells | Index Medicus | s | TGFβ‐BMP | Vascular Biology & Angiogenesis | Genetics, Gene Therapy & Genetic Disease | Cardiovascular System | Basic Medicine | Medical and Health Sciences | Medicin och hälsovetenskap | Medicinska och farmaceutiska grundvetenskaper
Journal Article
Proceedings of the National Academy of Sciences of the United States of America, ISSN 0027-8424, 7/2010, Volume 107, Issue 28, pp. 12640 - 12645
Cerebral cavernous malformations (CCM) are frequent vascular abnormalities caused by mutations in one of the CCM genes. CCM1 (also known as KRIT1) stabilizes... 
Angiogenesis | Phosphorylation | Human umbilical vein endothelial cells | Sprouting | Messenger RNA | Gene expression regulation | Lesions | Down regulation | Endothelial cells | Apoptosis | Vascular malformations | KRIT1 | CCM | PATHOGENESIS | MORPHOGENESIS | endothelial cells | MULTIDISCIPLINARY SCIENCES | ENDOTHELIAL-CELLS | vascular malformations | AKT | 2-HIT MECHANISM | Capillaries - pathology | Humans | Hemangioma, Cavernous, Central Nervous System - genetics | Hemangioma, Cavernous, Central Nervous System - metabolism | Proto-Oncogene Proteins c-akt - genetics | Microvessels | Female | Proto-Oncogene Proteins c-akt - metabolism | Capillaries - metabolism | Endothelial Cells - metabolism | Intercellular Signaling Peptides and Proteins | Signal Transduction - genetics | Mice, SCID | Proteins - genetics | Animals | Proteins - metabolism | Hemangioma, Cavernous, Central Nervous System - pathology | Mitogen-Activated Protein Kinases - pharmacology | Mitogen-Activated Protein Kinases - genetics | Mice | Proteins - pharmacology | Mutation | Endothelial Cells - pathology | Endothelial Cells - drug effects | Mitogen-Activated Protein Kinases - metabolism | Brain | Abnormalities | Physiological aspects | Genetic aspects | Neovascularization | Properties | Risk factors | Endothelium | Proteins | Genes | Rodents | Morphology | Biochemistry | Kinases | Embryos | Index Medicus | Biological Sciences
Journal Article
Journal of Biological Chemistry, ISSN 0021-9258, 04/2010, Volume 285, Issue 16, pp. 11760 - 11764
Cerebral cavernous malformations (CCM) are vascular lesions causing seizures and stroke. Mutations causing inactivation of one of three genes, ccm1, -2, or -3,... 
ACTIVATION | FASUDIL | GTPASES | INTEGRITY | PATHWAY | BIOCHEMISTRY & MOLECULAR BIOLOGY | CCM1 | KRIT1 Protein | Microtubule-Associated Proteins - genetics | Humans | rhoA GTP-Binding Protein - metabolism | Hemangioma, Cavernous, Central Nervous System - genetics | Hemangioma, Cavernous, Central Nervous System - metabolism | rho-Associated Kinases - antagonists & inhibitors | RNA Interference | rho-Associated Kinases - metabolism | Apoptosis Regulatory Proteins - genetics | Cardiac Myosins - metabolism | Amides - pharmacology | Cell Line | Proto-Oncogene Proteins - antagonists & inhibitors | Endothelial Cells - metabolism | Membrane Proteins - genetics | Carrier Proteins - antagonists & inhibitors | Proto-Oncogene Proteins - genetics | Microtubule-Associated Proteins - antagonists & inhibitors | Hemangioma, Cavernous, Central Nervous System - drug therapy | Carrier Proteins - genetics | Phenotype | Hemangioma, Cavernous, Central Nervous System - pathology | Membrane Proteins - antagonists & inhibitors | Apoptosis Regulatory Proteins - antagonists & inhibitors | Biosensing Techniques | Myosin Light Chains - metabolism | Protein Kinase Inhibitors - pharmacology | Pyridines - pharmacology | Endothelial Cells - pathology | Endothelial Cells - drug effects | Index Medicus | Molecular Bases of Disease | Brain | Extracellular Matrix | shRNA | Cerebral Cavernous Malformation | RhoA Biosensor | Reports | Drug Action | ROCK Inhibitor | Rho Kinase | Cell Permeabilization | RhoA
Journal Article
Free Radical Biology and Medicine, ISSN 0891-5849, 02/2018, Volume 115, pp. 202 - 218
( ) is a disease gene responsible for Cerebral Cavernous Malformations (CCM), a major cerebrovascular disease of proven genetic origin affecting 0.3–0.5% of... 
Oxidative stress | Heme oxygenase-1 (HO-1) | Argpyrimidine-modified heat-shock proteins | Redox signaling | Cerebral Cavernous Malformations | Antioxidant defense | Glyoxalase 1 (Glo1) | c-Jun | Oxidative DNA damage and apoptosis | Nuclear factor erythroid 2-related factor 2 (Nrf2) | Adaptive redox homeostasis | CCM1/KRIT1 | Cerebrovascular disease | GLYCATION END-PRODUCT | GLYOXALASE I | METHYLGLYOXAL MODIFICATION | ARGPYRIMIDINE-MODIFIED HSP70 | ENDOCRINOLOGY & METABOLISM | NF-KAPPA-B | AORTIC ENDOTHELIAL-CELLS | BIOCHEMISTRY & MOLECULAR BIOLOGY | CRYSTALLINE SILICA | DEFENSE PATHWAY | SIGNALING PATHWAY | VASCULAR INTEGRITY | Humans | Homeostasis | Hemangioma, Cavernous, Central Nervous System - genetics | Hemangioma, Cavernous, Central Nervous System - metabolism | Pyruvaldehyde - metabolism | Autophagy - genetics | Endothelial Cells - physiology | Central Nervous System Neoplasms - metabolism | Oxidation-Reduction | Lactoylglutathione Lyase - metabolism | Central Nervous System Neoplasms - genetics | KRIT1 Protein - metabolism | Cells, Cultured | Oxidative Stress - genetics | KRIT1 Protein - genetics | Mutation - genetics | HSP70 Heat-Shock Proteins - metabolism | Mice, Knockout | Animals | NF-E2-Related Factor 2 - metabolism | Brain - pathology | HSP27 Heat-Shock Proteins - metabolism | Mice | Protein Processing, Post-Translational | Apoptosis | Antioxidants | Proteins | Brain | Heme | Heat shock proteins | Index Medicus | PTM, post-translational modification | hBMEC, human brain microvascular endothelial cells | Casp-3, Caspase-3 | COX-2, cycloxygenase-2 | BBB, blood-brain barrier | NVU, neurovascular unit | Cyt c, cytochrome c | ICH, intracerebral hemorrhage | MG, methylglyoxal | HO-1, Heme oxygenase-1 | CCM, Cerebral Cavernous Malformation | AP, argpyrimidine (Nẟ-(5-hydroxy-4,6-dimethylpyrimidine-2-yl)-l-ornithine) | Glo1, Glyoxalase 1 | ARE, antioxidant-response element | KRIT1 | ROS, reactive oxygen species | KRIT1, Krev interaction trapped 1 | AGEs, advanced glycation end-products | TUNEL, TdT-mediated dUTP nick-end labeling | EndMT, endothelial-to-mesenchymal transition | Nrf2, nuclear factor erythroid 2-related factor 2 | Keap1, Kelch-like ECH-associated protein 1 | SOD, superoxide dismutase | Hsp, heat-shock protein | CNS, central nervous system | JNK, c-Jun NH2-terminal kinase | MEF, mouse embryonic fibroblast | CCM1
Journal Article
Journal Article