Animal Models of Light Chain Deposition Disease Provide a Better Understanding of Nodular Glomerulosclerosis

Nephron
G A HerreraJiamin Teng

Abstract

Light chain deposition disease (LCDD) is a model of glomerulosclerosis. The mature lesion of LCDD mimics nodular glomerulosclerosis in diabetic nephropathy. The pathogenetic mechanisms involved are similar in both disorders, though the causative factors are entirely different. This fact highlights the generic response of the mesangium to varied stimuli. In-vitro work has provided much insight into the pathogenesis of glomerulosclerosis in LCDD where the mesangium is the main target for initiation and progression of the disease. The lack of animal models has prevented the development of further therapeutic approaches to be tested in platforms such as ex-vivo and in-vivo preparing the way for human studies. Light chains (LCs) obtained from the urine of patients with renal biopsy proven LCDD were delivered to glomeruli using ex-vivo and in-vivo approaches to address whether in-vitro information could be validated in-vivo. Selected in-vitro studies were conducted to address specific issues dealing with mesangial cell (MC) differentiation and composition of extracellular matrix to add additional data to the existing vast literature. Using light, electron and scanning microscopy together with immunohistochemistry and ultrastructural ...Continue Reading

Citations

Feb 20, 2018·Nature Reviews. Nephrology·Christophe SiracFrank Bridoux

❮ Previous
Next ❯

Related Concepts

Related Feeds

Advanced Imaging of Cellular Signaling

Cell signaling is a vital mechanism for communication within cells and outside with the environment. Several different signaling pathways have been found and advanced imaging techniques are being developed to visualize the molecules involved in these signaling pathways. Find the latest research in advanced imaging of cellular signaling here.

Caveolins & Signal Transduction

Caveolins are small proteins with a hairpin loop conformation that are located in the plasma membrane of various cell types where they bind cholesterol and interact with receptors essential for several signal transduction pathways. Here is the latest research.