Cerebral Cavernous Malformations Develop Through Clonal Expansion of Mutant Endothelial Cells.

ABSTRACT:

Rationale

Vascular malformations arise in vessels throughout the entire body. Causative genetic mutations have been identified for many of these diseases; however, little is known about the mutant cell lineage within these malformations.

Objective

We utilize an inducible mouse model of cerebral cavernous malformations (CCMs) coupled with a multicolor fluorescent reporter to visualize the contribution of mutant endothelial cells (ECs) to the malformation.

Methods and results

We combined a Ccm3 mouse model with the confetti fluorescent reporter to simultaneously delete Ccm3 and label the mutant EC with 1 of 4 possible colors. We acquired Z-series confocal images from serial brain sections and created 3-dimensional reconstructions of entire CCMs to visualize mutant ECs d

SUBMITTER: Detter MR 

PROVIDER: S-EPMC6205520 | biostudies-literature | 2018 Oct

REPOSITORIES: biostudies-literature