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)
Please use this url to cite or link to this publication:
https://lup.lub.lu.se/record/2032571
- author
- 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
- organization
- publishing date
- 2011
- type
- Contribution to journal
- publication status
- published
- subject
- in
- PLoS ONE
- volume
- 6
- issue
- 6
- publisher
- Public Library of Science (PLoS)
- external identifiers
-
- wos:000292036900059
- pmid:21720563
- scopus:79959610720
- pmid:21720563
- 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
- 2016-04-01 13:41:57
- date last changed
- 2022-01-27 20:31:52
@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 (PLoS)}},
series = {{PLoS ONE}},
title = {{Phenotypic Expression of ADAMTS13 in Glomerular Endothelial Cells}},
url = {{https://lup.lub.lu.se/search/files/3537654/2203225.pdf}},
doi = {{10.1371/journal.pone.0021587}},
volume = {{6}},
year = {{2011}},
}