3D ultrastructural observations provide possible clinical insights in a mouse model of Marfan Syndrome.
Marfan syndrome (MFS) is a genetic condition that impacts connective tissue. While this can have negative consequences throughout the body and organs, it is the cardiovascular damage in particular that can cause death from this condition. Dr. Rodrigo Barbosa is a Professor of Anatomy at Faculdade Santa Marcelina as well as a Research Assistant in the group of Professor Lygia Pereira at the University of São Paulo, Brazil. Professor Pereira established the first knockout mouse model for MFS. Working with Professor Pereira, Professor Barbosa has published work characterizing the kidney in MFS, which plays an important role in the cardiovascular system. Their findings suggest that kidney phenotype may offer clinical insights for patients with Marfan Syndrome. This work incorporates in-depth imaging studies, including volume electron microscopy using SBF-SEM with a ZEISS Sigma FE-SEM with an integrated ultramicrotome.
The kidney plays a critical role in the cardiovascular system through its impact on hemodynamics. We explored the possible contribution of the Fibers of Elastic Fibers System (FEFS) in the glomerulus using ultrastructural analyses, including SBF-SEM.
3D Ultrastructure of the Kidney Glomerulus Capillary and Microfibrils
Imaged with SBF-SEM
Superior Plane
Higher Mag
Lateral Plane
IMAGE 1 OF 3 (Click arrows above to navigate images): The Fibers of the Elastic Fiber System (FEFS) are shown in red, capillary in white, and cells in blue. Wild type (left) presents FEFS arranged in a tubular-shaped network (white arrow) within the capillary. MFS (right) presents fractured FEFS (black arrow), and loss of capillary structure. Image 1: Figures A and B show the superior plane. Image 2: Figures C and D show the superior plane in higher magnification. Image 3: Figures E and F show the lateral plane. Data collected with ZEISS Sigma FE-SEM with integrated ultramicrotome. Data from R. Barbosa de Souza et al.
IMAGE 1 OF 3 (Click arrows above to navigate images): The Fibers of the Elastic Fiber System (FEFS) are shown in red, capillary in white, and cells in blue. Wild type (left) presents FEFS arranged in a tubular-shaped network (white arrow) within the capillary. MFS (right) presents fractured FEFS (black arrow), and loss of capillary structure. Image 1: Figures A and B show the superior plane. Image 2: Figures C and D show the superior plane in higher magnification. Image 3: Figures E and F show the lateral plane. Data collected with ZEISS Sigma FE-SEM with integrated ultramicrotome. Data from R. Barbosa de Souza et al.