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ADAMTS13 phenotype in plasma from normal individuals and patients with thrombotic thrombocytopenic purpura.

Manea Hedström, Minola LU ; Kristoffersson, Ann-Charlotte LU ; Tsai, Han-Mou; Zhou, Wenhua; Winqvist, Ingemar; Oldaeus, Goran; Billstrom, Rolf; Björk, Peter LU ; Holmberg, Lars LU and Karpman, Diana LU (2007) In European Journal of Pediatrics 166(3). p.249-257
Abstract
The activity of ADAMTS 13, the von Willebrand factor cleaving protease, is deficient in patients with thrombotic thrombocytopenic purpura (TTP). In the present study, the phenotype of ADAMTS13 in TTP and in normal plasma was demonstrated by immunoblotting. Normal plasma (n=20) revealed a single band at 190 kD under reducing conditions using a polyclonal antibody, and a single band at 150 kD under non-reducing conditions using a monoclonal antibody. ADAMTS 13 was not detected in the plasma from patients with congenital TTP (n=5) by either antibody, whereas patients with acquired TTP (n=2) presented the normal phenotype. Following immunoadsorption of immunoglobulins, the ADAMTS 13 band was removed from the plasma of the patients with... (More)
The activity of ADAMTS 13, the von Willebrand factor cleaving protease, is deficient in patients with thrombotic thrombocytopenic purpura (TTP). In the present study, the phenotype of ADAMTS13 in TTP and in normal plasma was demonstrated by immunoblotting. Normal plasma (n=20) revealed a single band at 190 kD under reducing conditions using a polyclonal antibody, and a single band at 150 kD under non-reducing conditions using a monoclonal antibody. ADAMTS 13 was not detected in the plasma from patients with congenital TTP (n=5) by either antibody, whereas patients with acquired TTP (n=2) presented the normal phenotype. Following immunoadsorption of immunoglobulins, the ADAMTS 13 band was removed from the plasma of the patients with acquired TTP, but not from that of normal individuals. This indicates that ADAMTS13 is complexed with immunoglobulin in these patients. The lack of ADAMTS13 expression in the plasma from patients with hereditary TTP may indicate defective synthesis, impaired cellular secretion, or enhanced degradation in the circulation. This study differentiated between normal and TTP plasma, as well as between congenital and acquired TTP. This method may, therefore, be used as a complement in the diagnosis of TTP. (Less)
Please use this url to cite or link to this publication:
author
organization
publishing date
type
Contribution to journal
publication status
published
subject
keywords
von Willebrand factor cleaving protease, von Willebrand factor, thrombotic thrombocytopenic purpura, ADAMTS13, plasma, mmunoblotting
in
European Journal of Pediatrics
volume
166
issue
3
pages
249 - 257
publisher
Springer
external identifiers
  • wos:000244645100011
  • scopus:33847675391
ISSN
1432-1076
DOI
10.1007/s00431-006-0354-2
language
English
LU publication?
yes
id
65ed6783-2492-4dcd-b648-9dd2fac7fc7c (old id 163787)
alternative location
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=17187257&dopt=Abstract
date added to LUP
2007-07-23 15:31:18
date last changed
2017-01-01 04:39:40
@article{65ed6783-2492-4dcd-b648-9dd2fac7fc7c,
  abstract     = {The activity of ADAMTS 13, the von Willebrand factor cleaving protease, is deficient in patients with thrombotic thrombocytopenic purpura (TTP). In the present study, the phenotype of ADAMTS13 in TTP and in normal plasma was demonstrated by immunoblotting. Normal plasma (n=20) revealed a single band at 190 kD under reducing conditions using a polyclonal antibody, and a single band at 150 kD under non-reducing conditions using a monoclonal antibody. ADAMTS 13 was not detected in the plasma from patients with congenital TTP (n=5) by either antibody, whereas patients with acquired TTP (n=2) presented the normal phenotype. Following immunoadsorption of immunoglobulins, the ADAMTS 13 band was removed from the plasma of the patients with acquired TTP, but not from that of normal individuals. This indicates that ADAMTS13 is complexed with immunoglobulin in these patients. The lack of ADAMTS13 expression in the plasma from patients with hereditary TTP may indicate defective synthesis, impaired cellular secretion, or enhanced degradation in the circulation. This study differentiated between normal and TTP plasma, as well as between congenital and acquired TTP. This method may, therefore, be used as a complement in the diagnosis of TTP.},
  author       = {Manea Hedström, Minola and Kristoffersson, Ann-Charlotte and Tsai, Han-Mou and Zhou, Wenhua and Winqvist, Ingemar and Oldaeus, Goran and Billstrom, Rolf and Björk, Peter and Holmberg, Lars and Karpman, Diana},
  issn         = {1432-1076},
  keyword      = {von Willebrand factor cleaving protease,von Willebrand factor,thrombotic thrombocytopenic purpura,ADAMTS13,plasma,mmunoblotting},
  language     = {eng},
  number       = {3},
  pages        = {249--257},
  publisher    = {Springer},
  series       = {European Journal of Pediatrics},
  title        = {ADAMTS13 phenotype in plasma from normal individuals and patients with thrombotic thrombocytopenic purpura.},
  url          = {http://dx.doi.org/10.1007/s00431-006-0354-2},
  volume       = {166},
  year         = {2007},
}