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Updated: May 31, 2026
Isolation of Glomeruli and In Vivo Labeling of Glomerular Cell Surface Proteins
Published on: January 18, 2019
Phenotypic expression of ADAMTS13 in glomerular endothelial cells
Ramesh Tati1, Ann-Charlotte Kristoffersson, Anne-Lie Ståhl
1Department of Pediatrics, Clinical Sciences Lund, Lund University, Lund, Sweden.
Background:
ADAMTS13 is the physiological von Willebrand factor (VWF)-cleaving protease. The aim of this study was to examine ADAMTS13 expression in kidneys from ADAMTS13 wild-type (Adamts13⁺/⁺) and deficient (Adamts13⁻/⁻) mice and to investigate the expression pattern and bioactivity in human glomerular endothelial cells.
Methodology/Principal Findings:
Immunohistochemistry was performed on kidney sections from ADAMTS13 wild-type and ADAMTS13-deficient mice. Phenotypic differences were examined by ultramorphology. ADAMTS13 expression in human glomerular endothelial cells and dermal microvascular endothelial cells was investigated by real-time PCR, flow cytometry, immunofluorescence and immunoblotting. VWF cleavage was demonstrated by multimer structure analysis and immunoblotting. ADAMTS13 was demonstrated in glomerular endothelial cells in Adamts13⁺/⁺ mice but no staining was visible in tissue from Adamts13⁻/⁻ mice. Thickening of glomerular capillaries with platelet deposition on the vessel wall was detected in Adamts13⁻/⁻ mice. ADAMTS13 mRNA and protein were detected in both human endothelial cells and the protease was secreted. ADAMTS13 activity was demonstrated in glomerular endothelial cells as cleavage of VWF.
Conclusions/Significance:
Glomerular endothelial cells express and secrete ADAMTS13. The proteolytic activity could have a protective effect preventing deposition of platelets along capillary lumina under the conditions of high shear stress present in glomerular capillaries.
