Role of Delta-Notch signaling in cerebral cavernous malformations

2016 ◽  
Vol 39 (4) ◽  
pp. 581-589 ◽  
Author(s):  
Souvik Kar ◽  
Arpita Baisantry ◽  
Arya Nabavi ◽  
Helmut Bertalanffy
Keyword(s):  
Notch Signaling ◽  
Cerebral Cavernous Malformations ◽  
Cavernous Malformations

scholarly journals Developmental timing of CCM2 loss influences cerebral cavernous malformations in mice

2011 ◽  
Vol 208 (9) ◽  
pp. 1835-1847 ◽  
Author(s):  
Gwénola Boulday ◽  
Noemi Rudini ◽  
Luigi Maddaluno ◽  
Anne Blécon ◽  
Minh Arnould ◽  
...  
Keyword(s):  
Autosomal Dominant ◽  
Vascular Malformations ◽  
Vascular Lesions ◽  
Developmental Timing ◽  
Cerebral Cavernous Malformations ◽  
Loss Of Function ◽  
Cavernous Malformations ◽  
The Central Nervous System ◽  
Dominant Condition

Cerebral cavernous malformations (CCM) are vascular malformations of the central nervous system (CNS) that lead to cerebral hemorrhages. Familial CCM occurs as an autosomal dominant condition caused by loss-of-function mutations in one of the three CCM genes. Constitutive or tissue-specific ablation of any of the Ccm genes in mice previously established the crucial role of Ccm gene expression in endothelial cells for proper angiogenesis. However, embryonic lethality precluded the development of relevant CCM mouse models. Here, we show that endothelial-specific Ccm2 deletion at postnatal day 1 (P1) in mice results in vascular lesions mimicking human CCM lesions. Consistent with CCM1/3 involvement in the same human disease, deletion of Ccm1/3 at P1 in mice results in similar CCM lesions. The lesions are located in the cerebellum and the retina, two organs undergoing intense postnatal angiogenesis. Despite a pan-endothelial Ccm2 deletion, CCM lesions are restricted to the venous bed. Notably, the consequences of Ccm2 loss depend on the developmental timing of Ccm2 ablation. This work provides a highly penetrant and relevant CCM mouse model.

scholarly journals Radiation diagnostics of cerebral cavernous malformations

2021 ◽  
Vol 2 (1) ◽  
pp. 39-48
Author(s):  
Elena N. Girya ◽  
Valentin E. Sinitsyn ◽  
Alexey S. Tokarev
Keyword(s):  
Pulse Sequences ◽  
Vascular Pathology ◽  
Cerebral Cavernous Malformations ◽  
Cavernous Malformations ◽  
Imaging Sequence ◽  
Magnetic Resonance Imaging Mri ◽  
The Moment ◽  
Ct And Mri ◽  
Selection Of

Cerebral cavernous malformations are a fairly common vascular pathology at the moment, with the number of detected cases increasing dramatically in recent years. This is because modern neuroimaging methods such as computed tomography (CT) and magnetic resonance imaging (MRI) have been introduced into clinical practice and are widely available. Prior to the advent of CT and MRI technologies, it was extremely difficult to diagnose this pathology, and the diagnosis was usually made intraoperatively or based on autopsy data. Further, the literature review is devoted to the radiological diagnosis of cerebral cavernous malformations (CM). The role of neuroimaging methods in the diagnosis of cavernous malformations, as well as the use of MRI for CM visualization, was analyzed. The advantages of MRI over other neuroimaging methods for this pathology have been demonstrated. Pulse sequences of MRI and signaling characteristics of various foci were characterized, depending on the morphological substrate. The significance of the susceptibility-weighted imaging sequence was also evaluated for the detection of multifocal lesions in cases of familial CM. The study of the main pulse sequences of MRI for visualization of CM will improve the protocol algorithm for the timely diagnosis of this pathology and the selection of therapeutic approach.

scholarly journals The role of MRI in assessing the effectiveness of cerebral cavernous malformations stereotaxic radiosurgical treatment

2021 ◽  
Vol 2 (2S) ◽  
pp. 34-35
Author(s):  
E. N. Girya ◽  
O. L. Evdokimova ◽  
A. A. Kochakova ◽  
V. A. Rak ◽  
E. N. Rozhnova
Keyword(s):  
Cerebral Cavernous Malformations ◽  
Cavernous Malformations

.

Role of aspirin and statin therapy in patients with cerebral cavernous malformations

2020 ◽  
Vol 78 ◽  
pp. 246-251
Author(s):  
Santiago Gomez-Paz ◽  
Mohamed M. Salem ◽  
Georgios A. Maragkos ◽  
Luis C. Ascanio ◽  
Alejandro Enriquez-Marulanda ◽  
...  
Keyword(s):  
Statin Therapy ◽  
Cerebral Cavernous Malformations ◽  
Cavernous Malformations

The Role of Activated NOTCH Signaling in Childhood T-ALL

2010 ◽  
Vol 223 (01) ◽  
Author(s):  
AE Kulozik ◽  
C Kox ◽  
M Remke ◽  
S Breit ◽  
M Zimmermann ◽  
...  
Keyword(s):  
Notch Signaling

The Role of miR-124 in Drosophila Alzheimer's Disease Model by Targeting Delta in Notch Signaling Pathway

2015 ◽  
Vol 15 (10) ◽  
pp. 980-989 ◽  
Author(s):  
Y. Kong ◽  
J. Wu ◽  
D. Zhang ◽  
C. Wan ◽  
L. Yuan
Keyword(s):  
Notch Signaling ◽  
Signaling Pathway ◽  
Disease Model ◽  
Notch Signaling Pathway

scholarly journals A Series of 14 Representative Presentations of Cerebral Cavernous Malformations

2021 ◽  
pp. 101298
Author(s):  
Ryan Hudnall ◽  
Eric X. Chen ◽  
Patrick J Opperman ◽  
Sean Kelly ◽  
Justin A. Cramer ◽  
...  
Keyword(s):  
Cerebral Cavernous Malformations ◽  
Cavernous Malformations

scholarly journals Crosstalk between NOTCH and AKT signaling during murine megakaryocyte lineage specification

Blood ◽  
2011 ◽  
Vol 118 (5) ◽  
pp. 1264-1273 ◽  
Author(s):  
Melanie G. Cornejo ◽  
Vinciane Mabialah ◽  
Stephen M. Sykes ◽  
Tulasi Khandan ◽  
Cristina Lo Celso ◽  
...  
Keyword(s):  
Stem Cell ◽  
Notch Signaling ◽  
Signaling Pathway ◽  
Hematopoietic Stem Cell ◽  
Stem Cell Differentiation ◽  
Notch Signaling Pathway ◽  
Developmental Pathways ◽  
Lineage Specification ◽  
Hematopoietic Stem

Abstract The NOTCH signaling pathway is implicated in a broad range of developmental processes, including cell fate decisions. However, the molecular basis for its role at the different steps of stem cell lineage commitment is unclear. We recently identified the NOTCH signaling pathway as a positive regulator of megakaryocyte lineage specification during hematopoiesis, but the developmental pathways that allow hematopoietic stem cell differentiation into the erythro-megakaryocytic lineages remain controversial. Here, we investigated the role of downstream mediators of NOTCH during megakaryopoiesis and report crosstalk between the NOTCH and PI3K/AKT pathways. We demonstrate the inhibitory role of phosphatase with tensin homolog and Forkhead Box class O factors on megakaryopoiesis in vivo. Finally, our data annotate developmental mechanisms in the hematopoietic system that enable a decision to be made either at the hematopoietic stem cell or the committed progenitor level to commit to the megakaryocyte lineage, supporting the existence of 2 distinct developmental pathways.

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