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Cerebral Cavernous Malformation Proteins in Barrier Maintenance and Regulation

1
Graduate Program in Public Health and Preventive Medicine, Wuhan University of Science and Technology, Wuhan 430081, China
2
Department of Pathology, The University of Chicago, Chicago, IL 60615, USA
3
Section of Neurosurgery, Department of Surgery, The University of Chicago, Chicago, IL 60615, USA
*
Author to whom correspondence should be addressed.
These authors contributed equally.
Int. J. Mol. Sci. 2020, 21(2), 675; https://doi.org/10.3390/ijms21020675
Received: 10 December 2019 / Revised: 13 January 2020 / Accepted: 15 January 2020 / Published: 20 January 2020
(This article belongs to the Special Issue The Tight Junction and Its Proteins: More Than Just a Barrier)
Cerebral cavernous malformation (CCM) is a disease characterized by mulberry shaped clusters of dilated microvessels, primarily in the central nervous system. Such lesions can cause seizures, headaches, and stroke from brain bleeding. Loss-of-function germline and somatic mutations of a group of genes, called CCM genes, have been attributed to disease pathogenesis. In this review, we discuss the impact of CCM gene encoded proteins on cellular signaling, barrier function of endothelium and epithelium, and their contribution to CCM and potentially other diseases. View Full-Text
Keywords: cerebral cavernous malformation; endothelial barrier; epithelial barrier; Rho; ROCK; MEKK3 cerebral cavernous malformation; endothelial barrier; epithelial barrier; Rho; ROCK; MEKK3
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MDPI and ACS Style

Wei, S.; Li, Y.; Polster, S.P.; Weber, C.R.; Awad, I.A.; Shen, L. Cerebral Cavernous Malformation Proteins in Barrier Maintenance and Regulation. Int. J. Mol. Sci. 2020, 21, 675.

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