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

ccm2-like is required for cardiovascular development as a novel component of the Heg-CCM pathway

(2013) Jonathan N. Rosen et al.   DEVELOPMENTAL BIOLOGY

Focal Defects in Single-Celled Tubes Mutant for Cerebral Cavernous Malformation 3, GCKIII, or NSF2

(2013) Yanjun Song et al.   DEVELOPMENTAL CELL

A Network of Interactions Enables CCM3 and STK24 to Coordinate UNC13D-Driven Vesicle Exocytosis in Neutrophils

(2013) Yong Zhang et al.   DEVELOPMENTAL CELL

Calcium and Calmodulin-dependent Serine/Threonine Protein Kinase Type II (CaMKII)-mediated Intramolecular Opening of Integrin Cytoplasmic Domain-associated Protein-1 (ICAP-1α) Negatively Regulates β1 Integrins

(2013) Angélique Millon-Frémillon et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

The Structure of the Ternary Complex of Krev Interaction Trapped 1 (KRIT1) Bound to Both the Rap1 GTPase and the Heart of Glass (HEG1) Cytoplasmic Tail

(2013) Alexandre R. Gingras et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

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

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

Loss of CCM3 impairs DLL4-Notch signalling: implication in endothelial angiogenesis and in inherited cerebral cavernous malformations

(2013) Chao You et al.   JOURNAL OF CELLULAR AND MOLECULAR MEDICINE

Mechanism for KRIT1 Release of ICAP1-Mediated Suppression of Integrin Activation

(2013) Weizhi Liu et al.   MOLECULAR CELL

EndMT contributes to the onset and progression of cerebral cavernous malformations

(2013) Luigi Maddaluno et al.   NATURE

Two-hit mechanism in cerebral cavernous malformation? A case of monozygotic twins with a CCM1/KRIT1 germline mutation

(2013) Philipp Dammann et al.   NEUROSURGICAL REVIEW

The C-terminal extension of human RTEL1, mutated in Hoyeraal-Hreidarsson syndrome, contains Harmonin-N-like domains

(2013) Guilhem Faure et al.   PROTEINS-STRUCTURE FUNCTION AND BIOINFORMATICS

Structural Basis for the Unique Heterodimeric Assembly between Cerebral Cavernous Malformation 3 and Germinal Center Kinase III

(2013) Xueyong Xu et al.   STRUCTURE

Structural Mechanism of CCM3 Heterodimerization with GCKIII Kinases

(2013) Meng Zhang et al.   STRUCTURE

Cerebral cavernous malformation is a vascular disease associated with activated RhoA signaling

(2012) Bryan T. Richardson et al.   BIOLOGICAL CHEMISTRY

Genetic and cellular basis of cerebral cavernous malformations: implications for clinical management

(2012) S Bacigaluppi et al.   CLINICAL GENETICS

Cell Adhesion: A FERM Grasp of the Tail Sorts Out Integrins

(2012) Nina N. Brahme et al.   CURRENT BIOLOGY

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

(2012) Xiangjian Zheng et al.   DEVELOPMENTAL CELL

Structural studies of cerebral cavernous malformations 2 (CCM2) reveal a folded helical domain at its C-terminus

(2012) Oriana S. Fisher et al.   FEBS LETTERS

Structural Basis for Small G Protein Effector Interaction of Ras-related Protein 1 (Rap1) and Adaptor Protein Krev Interaction Trapped 1 (KRIT1)

(2012) Xiaofeng Li et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

STK25 Protein Mediates TrkA and CCM2 Protein-dependent Death in Pediatric Tumor Cells of Neural Origin

(2012) Barbara Costa et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Structural basis of the junctional anchorage of the cerebral cavernous malformations complex

(2012) Alexandre R. Gingras et al.   JOURNAL OF CELL BIOLOGY

Sorting nexin 17 prevents lysosomal degradation of β1 integrins by binding to the β1-integrin tail

(2012) Ralph Thomas Böttcher et al.   NATURE CELL BIOLOGY

Identification of the Kelch Family Protein Nd1-L as a Novel Molecular Interactor of KRIT1

(2012) Paolo Guazzi et al.   PLoS One

Conditional deletion of Ccm2 causes hemorrhage in the adult brain: a mouse model of human cerebral cavernous malformations

(2011) Kirk Cunningham et al.   HUMAN MOLECULAR GENETICS

Molecular Recognition of Leucine-Aspartate Repeat (LD) Motifs by the Focal Adhesion Targeting Homology Domain of Cerebral Cavernous Malformation 3 (CCM3)

(2011) Xiaofeng Li et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

CCM3/PDCD10 Heterodimerizes with Germinal Center Kinase III (GCKIII) Proteins Using a Mechanism Analogous to CCM3 Homodimerization

(2011) Derek F. Ceccarelli et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Structure-Function Analysis of Core STRIPAK Proteins

(2011) Michelle J. Kean et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Osteoblast mineralization requires β1 integrin/ICAP-1–dependent fibronectin deposition

(2011) Molly Brunner et al.   JOURNAL OF CELL 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

Cerebral cavernous malformations: from genes to proteins to disease

(2011) Daniel D. Cavalcanti et al.   JOURNAL OF NEUROSURGERY

A mechanism of Rap1-induced stabilization of endothelial cell–cell junctions

(2011) Jian J. Liu et al.   MOLECULAR BIOLOGY OF THE CELL

Rac1/osmosensing scaffold for MEKK3 contributes via phospholipase C- 1 to activation of the osmoprotective transcription factor NFAT5

(2011) X. Zhou et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

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

Crystal structure of human programmed cell death 10 complexed with inositol-(1,3,4,5)-tetrakisphosphate: A novel adaptor protein involved in human cerebral cavernous malformation

(2010) Jingjin Ding et al.   BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS

Integrin Cytoplasmic Domain–Associated Protein-1 Attenuates Sprouting Angiogenesis

(2010) René Brütsch et al.   CIRCULATION RESEARCH

Rap1, a mercenary among the Ras-like GTPases

(2010) E.W. Frische et al.   DEVELOPMENTAL BIOLOGY

Recent insights into cerebral cavernous malformations: the molecular genetics of CCM

(2010) Florence Riant et al.   FEBS Journal

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

Crystal Structure of CCM3, a Cerebral Cavernous Malformation Protein Critical for Vascular Integrity

(2010) Xiaofeng Li et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Rho Kinase Inhibition Rescues the Endothelial Cell Cerebral Cavernous Malformation Phenotype

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

CCM1 regulates vascular-lumen organization by inducing endothelial polarity

(2010) M. G. Lampugnani et al.   JOURNAL OF CELL SCIENCE

CCM3/PDCD10 stabilizes GCKIII proteins to promote Golgi assembly and cell orientation

(2010) M. Fidalgo et al.   JOURNAL OF CELL SCIENCE

CCM3 signaling through sterile 20–like kinases plays an essential role during zebrafish cardiovascular development and cerebral cavernous malformations

(2010) Xiangjian Zheng et al.   JOURNAL OF CLINICAL INVESTIGATION

Cerebral cavernous malformations proteins inhibit Rho kinase to stabilize vascular integrity

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

Ccm1 Regulates Microvascular Morphogenesis during Angiogenesis

(2010) Huiling Liu et al.   JOURNAL OF VASCULAR RESEARCH

The pathogenetic features of cerebral cavernous malformations: a comprehensive review with therapeutic implications

(2010) Khaled M. Krisht et al.   Neurosurgical Focus

Cerebral cavernous malformations as a disease of vascular permeability: from bench to bedside with caution

(2010) Sanjay Yadla et al.   Neurosurgical Focus

KRIT1 Regulates the Homeostasis of Intracellular Reactive Oxygen Species

(2010) Luca Goitre et al.   PLoS One

Defining the Functional Domain of Programmed Cell Death 10 through Its Interactions with Phosphatidylinositol-3,4,5-Trisphosphate

(2010) Christopher F. Dibble 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

Control of cell adhesion dynamics by Rap1 signaling

(2009) Benjamin Boettner et al.   CURRENT OPINION IN CELL BIOLOGY

Rap1 and its effector KRIT1/CCM1 regulate  -catenin signaling

(2009) A. J. Glading et al.   Disease Models & Mechanisms

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

CCM2 Mediates Death Signaling by the TrkA Receptor Tyrosine Kinase

(2009) Liraz Harel et al.   NEURON

Assembling stable hair cell tip link complex via multidentate interactions between harmonin and cadherin 23

(2009) L. Pan et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Biosignaling of mammalian Ste20-related kinases

(2008) Pin Ling et al.   CELLULAR SIGNALLING

Structural and functional differences between KRIT1A and KRIT1B isoforms: A framework for understanding CCM pathogenesis

(2008) Floriana Francalanci et al.   EXPERIMENTAL CELL RESEARCH

Novel CCM1, CCM2, and CCM3 mutations in patients with cerebral cavernous malformations: in-frame deletion in CCM2 prevents formation of a CCM1/CCM2/CCM3 protein complex

(2008) Sonja Stahl et al.   HUMAN MUTATION

Cell adaptive response to extracellular matrix density is controlled by ICAP-1–dependent β1-integrin affinity

(2008) Angélique Millon-Frémillon et al.   JOURNAL OF CELL BIOLOGY

A PP2A Phosphatase High Density Interaction Network Identifies a Novel Striatin-interacting Phosphatase and Kinase Complex Linked to the Cerebral Cavernous Malformation 3 (CCM3) Protein

(2008) Marilyn Goudreault et al.   MOLECULAR & CELLULAR PROTEOMICS

krit1 Modulates β1-integrin-mediated Endothelial Cell Proliferation

(2008) Jun Zhang et al.   NEUROSURGERY