Role of Delta-Notch signaling in cerebral cavernous malformations

Neurosurgical Review
Souvik KarHelmut Bertalanffy

Abstract

Cerebral cavernous malformations (CCM) commonly known as cavernous hemangioma are associated with abnormally enlarged thin-walled blood vessels. As a result, these dilated capillaries are prone to leakage and result in hemorrhages. Clinically, such hemorrhages lead to severe headaches, focal neurological deficits, and epileptic seizures. CCM is caused by loss of function mutations in one of the three well-known CCM genes: Krev interaction trapped 1 (KRIT1), OSM, and programmed cell death 10 (PDCD10). Loss of CCM genes have been shown to be synergistically related to decreased Notch signaling and excessive angiogenesis. Despite recent evidences indicating that Notch signaling plays a pivotal role in regulating angiogenesis, the role of Notch in CCM development and progression is still not clear. Here, we provide an update literature review on the current knowledge of the structure of Notch receptor and its ligands, its relevance to angiogenesis and more precisely to CCM pathogenesis. In addition to reviewing the current literatures, this review will also focus on the cross talk between Delta-Notch and vascular endothelial growth factor (VEGF) signaling in angiogenesis and in CCM pathogenesis. Understanding the role of Notch sign...Continue Reading

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Citations

Feb 18, 2017·Current Opinion in Hematology·Maria Grazia LampugnaniNoemi Rudini
Feb 10, 2017·Journal of Molecular Neuroscience : MN·Souvik KarHelmut Bertalanffy
Oct 1, 2017·Circulation Research·Sara I CunhaMaria Grazia Lampugnani
Oct 9, 2019·International Journal of Molecular Sciences·Francesco Vieceli Dalla SegaSaverio Francesco Retta
Jan 15, 2021·Journal of Cell Science·Oscar M J A StassenCecilia M Sahlgren

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