The unique structural and functional characteristics of glomerular endothelial cell fenestrations and their potential as a therapeutic target in kidney disease

The glomerular endothelial cell (GEnC) fenestrations are a critical component of the glomerular filtration barrier. Their unique non-diaphragmed structure are key to their function in glomerular hydraulic permeability and their aberration in disease can contribute to loss of glomerular filtration function. This review provides a comprehensive update of current understanding of the regulation and biogenesis of fenestrae. We consider diseases in which GEnC fenestration loss is recognised or may play a role and discuss methods with potential to facilitate the study of these critical structures. Literature is drawn from GEnC as well as other fenestrated cell types such as liver sinusoidal endothelial cells that most closely parallel GEnC.