Journal article
Recent insights into cerebral cavernous malformations: animal models of CCM and the human phenotype
The FEBS journal, Vol.277(5), pp.1076-1083
01/22/2010
DOI: 10.1111/j.1742-4658.2009.07536.x
PMCID: PMC2859824
PMID: 20096037
Abstract
Cerebral cavernous malformations are common vascular lesions of the central nervous system that predispose to seizures, focal neurologic deficits and potentially fatal hemorrhagic stroke. Human genetic studies have identified three genes associated with the disease and biochemical studies of these proteins have identified interaction partners and possible signaling pathways. A variety of animal models of CCM have been described to help translate the cellular and biochemical insights into a better understanding of disease mechanism. In this minireview, we discuss the contributions of animal models to our growing understanding of the biology of cavernous malformations, including the elucidation of the cellular context of CCM protein actions and the
in vivo
confirmation of abnormal endothelial cell–cell interactions. Challenges and progress towards developing a faithful model of CCM biology are reviewed.
Details
- Title: Subtitle
- Recent insights into cerebral cavernous malformations: animal models of CCM and the human phenotype
- Creators
- Aubrey C. Chan - University of UtahDean Y. Li - University of UtahMichel J. Berg - University of Rochester Medical CenterKevin J. Whitehead - University of Utah
- Resource Type
- Journal article
- Publication Details
- The FEBS journal, Vol.277(5), pp.1076-1083
- DOI
- 10.1111/j.1742-4658.2009.07536.x
- PMID
- 20096037
- PMCID
- PMC2859824
- NLM abbreviation
- FEBS J
- ISSN
- 1742-464X
- eISSN
- 1742-4658
- Language
- English
- Date published
- 01/22/2010
- Academic Unit
- Psychiatry; Internal Medicine
- Record Identifier
- 9984280879202771
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