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Phenotypic Expression of ADAMTS13 in Glomerular Endothelial Cells

Tati, Ramesh LU ; Kristoffersson, Ann-Charlotte LU ; Ståhl, Anne-lie LU ; Mörgelin, Matthias LU ; Motto, David; Satchell, Simon; Mathieson, Peter; Manea Hedström, Minola LU and Karpman, Diana LU (2011) In PLoS ONE 6(6).
Abstract
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... (More)
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. (Less)
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published
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in
PLoS ONE
volume
6
issue
6
publisher
Public Library of Science
external identifiers
  • wos:000292036900059
  • pmid:21720563
  • scopus:79959610720
ISSN
1932-6203
DOI
10.1371/journal.pone.0021587
language
English
LU publication?
yes
id
73c83b40-5889-4c7e-87ea-07b65ac78bf9 (old id 2032571)
alternative location
http://www.ncbi.nlm.nih.gov/pubmed/21720563?dopt=Abstract
date added to LUP
2011-08-02 08:58:56
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2017-08-06 04:03:18
@article{73c83b40-5889-4c7e-87ea-07b65ac78bf9,
  abstract     = {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.},
  author       = {Tati, Ramesh and Kristoffersson, Ann-Charlotte and Ståhl, Anne-lie and Mörgelin, Matthias and Motto, David and Satchell, Simon and Mathieson, Peter and Manea Hedström, Minola and Karpman, Diana},
  issn         = {1932-6203},
  language     = {eng},
  number       = {6},
  publisher    = {Public Library of Science},
  series       = {PLoS ONE},
  title        = {Phenotypic Expression of ADAMTS13 in Glomerular Endothelial Cells},
  url          = {http://dx.doi.org/10.1371/journal.pone.0021587},
  volume       = {6},
  year         = {2011},
}