Plasma-derived mannose-binding lectin shows a direct interaction with C1-inhibitor |
| |
| Authors: | Mischa P. Keizer Angela M. Kamp Nannette Brouwer Marianne D. van de Wetering Diana Wouters Taco W. Kuijpers |
| |
| Affiliation: | 1. Emma Children''s Hospital, Academic Medical Center (AMC), University of Amsterdam, Amsterdam, The Netherlands;2. Department of Immunopathology, Sanquin Research and Landsteiner Laboratory, AMC, University of Amsterdam, Amsterdam, The Netherlands;3. Department of Blood Cell Research, Sanquin Research and Landsteiner Laboratory, AMC, University of Amsterdam, Amsterdam, The Netherlands |
| |
| Abstract: | MBL-deficiency has been associated with an increased frequency and severity of infection, in particular in children and under immunocompromized conditions. In an open uncontrolled safety and pharmacokinetic MBL-substitution study using plasma-derived MBL (pdMBL) in MBL-deficient pediatric oncology patients, we found that despite MBL trough levels above 1.0 μg/ml MBL functionality was not efficiently restored upon ex vivo testing. |
| |
| Keywords: | AP, alternative pathway C1-inh, C1-inhibitor CP, classical pathway HPE, high performance ELISA buffer LP, lectin pathway MASPs, MBL associated serine proteases Mass-Spec, mass-spectrometry MBL, mannan binding lectin MMC, MBL/MASP/C1-inh pAb, polyclonal antibodies pdMBL, plasma-derived MBL polyHRP, polymerized Horseradish peroxidase rhMASP-2, recombinant human MASP-2 rhMBL, recombinant human MBL TMB, tetramethylbenzidine TP, terminal pathway |
| 本文献已被 ScienceDirect 等数据库收录！ |
|
