Extracellular matrix and vascular dynamics in the kidney of a murine model for Marfan syndrome

• DOI: 10.1371/journal.pone.0285418
• Publication Date: 2023-05-09T22:38:53Z
• Authors: Rodrigo Barbosa d...; Renan Barbosa Lemes; Orestes Foresto-Neto; Luara Lucena Cass...; Dieter P. Reinhardt; Keith M. Meek; Ivan Hong Jun Koh; Philip N. Lewis; Lygia V. Pereira
• Keywords: Glomerulus; Fibrillin; Renal glomerulus; Kidney Glomerulus

Fibrillin-1 is a pivotal structural component of the kidney’s glomerulus and peritubular tissue. Mutations in fibrillin-1 gene result Marfan syndrome (MFS), an autosomal dominant disease connective Although kidney not considered classically affected organ MFS, several case reports describe glomerular patients. Therefore, this study aimed to characterize mgΔ lpn -mouse model MFS. Affected animals presented significant reduction glomerulus, glomerulus-capillary, urinary space, fibronectin glomerulus. Transmission electron microscopy 3D-ultrastructure analysis revealed decreased amounts microfibrils which also appeared fragmented MFS mice. Increased collagen fibers types I III, MMP-9, α-actin were observed animals, suggesting tissue-remodeling process kidney. Video showed increase microvessel distribution coupled with blood-flow velocity, while ultrasound flow significantly lower blood artery vein The hemodynamic changes indicate presence remodeling vascular resistance model. Both processes are associated hypertension expected worsen cardiovascular phenotype