Endothelial cell-specific collagen type IV-alpha_3 expression does not rescue Alport syndrome in Col4a3~/~ mice

摘要

The primary pathology of the renal disease aspect of Alport syndrome is localized to the glomeruli, which are capillary tufts with an outer parietal epithelium and intervening Bowman's space. The tuft is a network of feneslrated endothelial cell (EC)-lined capillaries anchored by mesangial cells and covered by specialized epithelial cells called podocytes that reside in Bowman's space (33). A glomerular basement membrane (GBM) constructed by and secreted between the ECs and podocytes is thought to impart size-selective filtration (5) as well as provide attachment sites and signals to ECs, podocytes, and mesangial cells (7). Plasma components pass through the fenestrated endothelium, GBM, and specialized cell-cell junctions between podocytes called slit diaphragms to form the ultrafiltrate.