期刊
FEBS JOURNAL
卷 277, 期 5, 页码 1076-1083出版社
WILEY
DOI: 10.1111/j.1742-4658.2009.07536.x
关键词
animal model; cavernous angioma; CCM; CCM2; cerebral cavernous malformation; Krit1; mouse model; OSM; PDCD10; zebrafish
资金
- US National Institutes of Health [T32-GM007464]
- American Heart Association
- Edna Benning Foundation
- Juvenile Diabetes Research Foundation
- Burroughs Wellcome Fund
- Flight Attendants Medical Research Institute
- NATIONAL HEART, LUNG, AND BLOOD INSTITUTE [R01HL084516, R01HL065648, R01HL077671, K08HL079095, R01HL068873] Funding Source: NIH RePORTER
- NATIONAL INSTITUTE OF GENERAL MEDICAL SCIENCES [T32GM007464] Funding Source: NIH RePORTER
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.
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