Role of Delta-Notch signaling in cerebral cavernous malformations

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• 作者：Souvik Kar ; Arpita Baisantry ; Arya Nabavi ; Helmut Bertalanffy
• 关键词：Cerebral cavernous malformations ; Hemorrhages ; Notch ; Angiogenesis
• 刊名：Neurosurgical Review
• 出版年：2016
• 出版时间：October 2016
• 年：2016
• 卷：39
• 期：4
• 页码：581-589
• 全文大小：2,541 KB
• 刊物类别：Medicine
• 刊物主题：Medicine & Public Health
  Neurosurgery
  
• 出版者：Springer Berlin / Heidelberg
• ISSN：1437-2320
• 卷排序：39

文摘

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 signaling in CCM development and progression might help provide a better insight for novel anti-angiogenic therapies.