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Biologically active ADAMTS13 is expressed in renal tubular epithelial cells.

Manea Hedström, Minola LU ; Tati, Ramesh LU ; Karlsson, Jessica; Békassy, Zivile LU and Karpman, Diana LU (2010) In Pediatric Nephrology 25. p.87-96
Abstract
ADAMTS13 mRNA, which encodes the von Willebrand factor-cleaving protease, has been detected in a variety of tissues, including the kidney. The aim of our study was to characterize tubular expression and bioactivity of ADAMTS13. ADAMTS13 mRNA was detected in cultured primary human renal tubular epithelial cells (HRTEC) and in A498 cells, a human renal carcinoma cell line, by real-time PCR. Protein was detected using immunofluorescence and immunoblotting. Immunoblots demonstrated that the protein was secreted. The protease was proteolytically active in both cell lysates and cleaved the FRETS-VWF73 substrate. ADAMTS13 was demonstrated in situ in the renal cortex by immunohistochemistry. Protease was detected in both the proximal and distal... (More)
ADAMTS13 mRNA, which encodes the von Willebrand factor-cleaving protease, has been detected in a variety of tissues, including the kidney. The aim of our study was to characterize tubular expression and bioactivity of ADAMTS13. ADAMTS13 mRNA was detected in cultured primary human renal tubular epithelial cells (HRTEC) and in A498 cells, a human renal carcinoma cell line, by real-time PCR. Protein was detected using immunofluorescence and immunoblotting. Immunoblots demonstrated that the protein was secreted. The protease was proteolytically active in both cell lysates and cleaved the FRETS-VWF73 substrate. ADAMTS13 was demonstrated in situ in the renal cortex by immunohistochemistry. Protease was detected in both the proximal and distal renal tubules in normal renal tissue (n = 3) as well as in patients with tubular disorders (n = 3). Immunoblotting revealed that ADAMTS13 was present in the urine of patients with tubulopathy (n = 5) but not in normal urine. ADAMTS13 in urine had a molecular size similar to that in plasma, which indicates that the protease originates in the tubuli because such large proteins do not normally pass the glomerular filter. In conclusion, human renal tubular epithelial cells synthesize biologically active ADAMTS13 which may, after release from tubuli, regulate hemostasis in the local microenvironment. (Less)
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author
organization
publishing date
type
Contribution to journal
publication status
published
subject
in
Pediatric Nephrology
volume
25
pages
87 - 96
publisher
Springer
external identifiers
  • wos:000271961000010
  • pmid:19644711
  • scopus:76149104791
ISSN
1432-198X
DOI
10.1007/s00467-009-1262-2
language
English
LU publication?
yes
id
4d164828-fd5d-4066-a76c-48bb64a9242f (old id 1470211)
alternative location
http://www.ncbi.nlm.nih.gov/pubmed/19644711?dopt=Abstract
date added to LUP
2009-09-04 10:52:51
date last changed
2017-08-13 04:40:22
@article{4d164828-fd5d-4066-a76c-48bb64a9242f,
  abstract     = {ADAMTS13 mRNA, which encodes the von Willebrand factor-cleaving protease, has been detected in a variety of tissues, including the kidney. The aim of our study was to characterize tubular expression and bioactivity of ADAMTS13. ADAMTS13 mRNA was detected in cultured primary human renal tubular epithelial cells (HRTEC) and in A498 cells, a human renal carcinoma cell line, by real-time PCR. Protein was detected using immunofluorescence and immunoblotting. Immunoblots demonstrated that the protein was secreted. The protease was proteolytically active in both cell lysates and cleaved the FRETS-VWF73 substrate. ADAMTS13 was demonstrated in situ in the renal cortex by immunohistochemistry. Protease was detected in both the proximal and distal renal tubules in normal renal tissue (n = 3) as well as in patients with tubular disorders (n = 3). Immunoblotting revealed that ADAMTS13 was present in the urine of patients with tubulopathy (n = 5) but not in normal urine. ADAMTS13 in urine had a molecular size similar to that in plasma, which indicates that the protease originates in the tubuli because such large proteins do not normally pass the glomerular filter. In conclusion, human renal tubular epithelial cells synthesize biologically active ADAMTS13 which may, after release from tubuli, regulate hemostasis in the local microenvironment.},
  author       = {Manea Hedström, Minola and Tati, Ramesh and Karlsson, Jessica and Békassy, Zivile and Karpman, Diana},
  issn         = {1432-198X},
  language     = {eng},
  pages        = {87--96},
  publisher    = {Springer},
  series       = {Pediatric Nephrology},
  title        = {Biologically active ADAMTS13 is expressed in renal tubular epithelial cells.},
  url          = {http://dx.doi.org/10.1007/s00467-009-1262-2},
  volume       = {25},
  year         = {2010},
}