A murine model of cerebral cavernous malformations with acute hemorrhage

iScience

卷:25

Elisabetta Dejana¹  Federica Pisati²  Claudio Tripodo²  Matteo Malinverno³  Claudio Maderna³ 

[1] Tumour Immunology Unit, University of Palermo, Palermo, Italy;

[2] Tumour and Microenvironment Histopathology Unit, FIRC Institute of Molecular Oncology (IFOM), Milan, Italy;

[3] Vascular Biology Unit, The FIRC Institute of Molecular Oncology Foundation, Milan 20139, Italy;

关键词: Vascular remodeling;    Developmental neuroscience;    Model organism;   

DOI  :  

来源: DOAJ

【 摘 要 】

Summary: Cavernomas are multi-lumen and blood-filled vascular malformations which form in the brain and the spinal cord. They lead to hemorrhage, epileptic seizures, neurological deficits, and paresthesia. An effective medical treatment is still lacking, and the available murine models for cavernomas have several limitations for preclinical studies. These include disease phenotypes that differ from human diseases, such as restriction of the lesions to the cerebellum, and absence of acute hemorrhage. Additional limitations of current murine models include rapid development of lesions, which are lethal before the first month of age. Here, we have characterized a murine model that recapitulates features of the human disease: lesions develop after weaning throughout the entire CNS, including the spinal cord, and undergo acute hemorrhage. This provides a preclinical model to develop new drugs for treatment of acute hemorrhage in the brain and spinal cord, as an unmet medical emergency for patients with cavernomas.

【 授权许可】

Unknown