AbstractCerebral cavernous malformations (CCMs) are vascular lesions of the central nervous system that affect 1 in 200 people. CCM lesions are prone to bleeds and are the major cause of stroke and sudden death in children and young adults. Current treatment for CCM is limited to high-risk neurosurgery; but most lesions are surgically inaccessible. As such there is an urgent need for novel non-invasive, druggable treatment options. Mouse models of CCM disease have been established by deleting Ccm genes in postnatal animals. These mouse models provide invaluable tools to investigate molecular mechanism and therapeutic approaches for CCM disease. However, the full value of these animal models is limited by the lack of an accurate and quantitative method to assess lesion burden and progression. To overcome the limitation, we have adopted micro-CT imaging to measure CCM lesion burden in mouse brains. As this study utilized a voxel dimension of 9.5μm (leading to a minimum feature size of approximately 25μm), it is therefore sufficient to measure CCM lesion volume and number globally and accurately and provide high-resolution 3-D mapping of CCM lesions in mouse brains. Using micro-CT, we have quantitatively demonstrated various genes and therapeutic approaches can modify CCM lesion burden. Hence, micro-CT imaging enhanced the value of the established mouse models to study the molecular basis and potential therapies for CCM and other cerebrovascular diseases.
Centenary Institute, University of Technology Sydney
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