PLOS ONE · 14 authors, 7 centres
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This preclinical study characterized kidney structure and vascular dynamics in the mgΔlpn mouse model of Marfan syndrome, finding significant reductions in fibrillin-1, fibronectin, and glomerular dimensions alongside increased collagen deposition and vascular remodeling. These structural and hemodynamic alterations suggest kidney remodeling and vascular resistance that may contribute to the cardiovascular phenotype of MFS.
This study used histology, transmission electron microscopy, 3D-ultrastructure reconstruction, SDF video-microscopy, and ultrasound flow analysis to characterize kidney changes in the mgΔlpn mouse model of Marfan syndrome. MFS mice showed significant reductions in fibrillin-1 and fibronectin in the glomerulus, decreased glomerular dimensions, and fragmented microfibrils. Increased collagen types I and III, MMP-9, and α-smooth muscle actin indicated tissue remodeling, while reduced Cx43 expression suggested some compensatory mechanism. Microvessel distribution was increased with reduced blood-flow velocity, and ultrasound revealed lower blood flow in the renal artery and vein. Elastic fiber fragmentation in the renal artery and duplication of the basal lamina in glomerular capillaries were also observed. These changes indicate kidney remodeling and vascular resistance. However, no direct measurement of renal function, blood pressure, or plasma RAAS components was performed, limiting interpretation.
