Severely decreased ADAMTS13 unbound to VWF may play a key role in the pathogenesis of HELLP syndrome.A qualitative ADAMTS13 assay may be important for diagnosing HELLP syndrome.
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http://dx.doi.org/10.1182/bloodadvances.2017006767 | DOI Listing |
Clin Pharmacokinet
May 2022
Department of Clinical Pharmacology, Medical University of Vienna, Vienna, Austria.
The pathophysiology of sepsis alters drug pharmacokinetics, resulting in inadequate drug exposure and target-site concentration. Suboptimal exposure leads to treatment failure and the development of antimicrobial resistance. Therefore, we seek to optimize antimicrobial therapy in sepsis by selecting the right drug and the correct dosage.
View Article and Find Full Text PDFJ Mol Biol
June 2021
Proteolysis Laboratory, Department of Structural Biology, Molecular Biology Institute of Barcelona (CSIC), Barcelona Science Park, c/Baldiri Reixac, 15-21, 08028 Barcelona, Catalonia, Spain. Electronic address:
Von Willebrand Factor (vWF), a 300-kDa plasma protein key to homeostasis, is cleaved at a single site by multi-domain metallopeptidase ADAMTS-13. vWF is the only known substrate of this peptidase, which circulates in a latent form and becomes allosterically activated by substrate binding. Herein, we characterised the complex formed by a competent peptidase construct (AD13-MDTCS) comprising metallopeptidase (M), disintegrin-like (D), thrombospondin (T), cysteine-rich (C), and spacer (S) domains, with a 73-residue functionally relevant vWF-peptide, using nine complementary techniques.
View Article and Find Full Text PDFSeverely decreased ADAMTS13 unbound to VWF may play a key role in the pathogenesis of HELLP syndrome.A qualitative ADAMTS13 assay may be important for diagnosing HELLP syndrome.
View Article and Find Full Text PDFTransfusion
December 2013
Department of Blood Transfusion Medicine, Nara Medical University, Kashihara, Nara, Japan; The Chemo-Sero-Therapeutic Research Institute, Kikuchi, Kumamoto, Japan.
Background: Thrombotic thrombocytopenic purpura (TTP) is characterized by deficient ADAMTS13 activity. Treatment involves plasma exchange (PE). Both fresh-frozen plasma (FFP) and cryosupernatant (CSP) are used, but it remains to be determined which is more effective.
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