期刊
PLOS ONE
卷 6, 期 6, 页码 -出版社
PUBLIC LIBRARY SCIENCE
DOI: 10.1371/journal.pone.0021587
关键词
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资金
- The Swedish Research Council [K2010-65X-14008, 2008-7480]
- Torsten and Ragnar Soderberg Foundation
- Crown Princess Lovisa's Society for Child Care
- The Konung Gustaf V:s 80-arsfond
- Fanny Ekdahl's Foundation
- Greta and Johan Kock Foundation
- Crafoord Foundation
- Royal Swedish Academy of Sciences
- Medical Research Council [G0800200] Funding Source: researchfish
- MRC [G0800200] Funding Source: UKRI
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.
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