Analysis of CCN Protein Expression and Activities in Vasoproliferative Retinopathies.

The retina is a complex neurovascular structure that conveys light/visual image through the optic nerve to the visual cortex of the brain. Neuronal and vascular activities in the retina are physically and functionally intertwined, and vascular alterations are consequential to the proper function of the entire visual system. In particular, alteration of the structure and barrier function of the retinal vasculature is commonly associated with the development of vasoproliferative ischemic retinopathy, a set of clinically well-defined chronic ocular microvascular complications causing blindness in all age groups. Experimentally, the retinal tissue provides researchers with a convenient, easily accessible, and directly observable model suitable to investigate whether and how newly identified genes regulate vascular development and regeneration. The six mammalian CCN gene-encoded proteins are part of an extracellular network of bioactive molecules that regulate various aspects of organ system development and diseases. Whether and how these molecules regulate the fundamental aspects of blood vessel development and pathology and subsequently the neurovascular link in the retina are open-ended questions. Sophisticated methods have been developed to gain insight into the pathogenesis of retinal vasculopathy. This chapter describes several useful methodologies and animal models to investigate the regulation and potential relevance of the CCN proteins in vasoproliferative diseases of the retina.