Restoration of MBL-deficiency: Redefining the safety, efficacy and viability of MBL-substitution therapy

M. P. Keizer; D. Wouters; L. J. Schlapbach; T. W. Kuijpers

doi:https://doi.org/10.1016/j.molimm.2014.06.005

Restoration of MBL-deficiency: Redefining the safety, efficacy and viability of MBL-substitution therapy

M. P. Keizer, D. Wouters, L. J. Schlapbach, T. W. Kuijpers

Research output: Contribution to journal › Review article › Academic › peer-review

33 Citations (Scopus)

Abstract

MBL-deficiency is a commonly occurring deficiency of the innate immune system, affecting a substantial part of the population and has been extensively studied. MBL appears to function as a disease modifier. The role of MBL in different conditions is context-dependent. Many clinical studies show conflicting results, which can be partially explained by different definitions of MBL-deficiency, including phenotype- and genotype-based approaches. In this review we give an overview of literature of MBL, its role in different pathologies, diseases and patient populations. We review MBL replacement studies, and discuss the potential of MBL substitution therapy. We finally suggest that new MBL substitution trials should be conducted within a predefined patient population. MBL-deficiency should be based on serum levels and confirmed by genotyping. (C) 2014 Elsevier Ltd. All rights reserved

Original language	English
Pages (from-to)	174-184
Journal	Molecular immunology
Volume	61
Issue number	2
DOIs	https://doi.org/10.1016/j.molimm.2014.06.005
Publication status	Published - 2014

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https://doi.org/10.1016/j.molimm.2014.06.005

Cite this

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title = "Restoration of MBL-deficiency: Redefining the safety, efficacy and viability of MBL-substitution therapy",

abstract = "MBL-deficiency is a commonly occurring deficiency of the innate immune system, affecting a substantial part of the population and has been extensively studied. MBL appears to function as a disease modifier. The role of MBL in different conditions is context-dependent. Many clinical studies show conflicting results, which can be partially explained by different definitions of MBL-deficiency, including phenotype- and genotype-based approaches. In this review we give an overview of literature of MBL, its role in different pathologies, diseases and patient populations. We review MBL replacement studies, and discuss the potential of MBL substitution therapy. We finally suggest that new MBL substitution trials should be conducted within a predefined patient population. MBL-deficiency should be based on serum levels and confirmed by genotyping. (C) 2014 Elsevier Ltd. All rights reserved",

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AU - Kuijpers, T. W.

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AB - MBL-deficiency is a commonly occurring deficiency of the innate immune system, affecting a substantial part of the population and has been extensively studied. MBL appears to function as a disease modifier. The role of MBL in different conditions is context-dependent. Many clinical studies show conflicting results, which can be partially explained by different definitions of MBL-deficiency, including phenotype- and genotype-based approaches. In this review we give an overview of literature of MBL, its role in different pathologies, diseases and patient populations. We review MBL replacement studies, and discuss the potential of MBL substitution therapy. We finally suggest that new MBL substitution trials should be conducted within a predefined patient population. MBL-deficiency should be based on serum levels and confirmed by genotyping. (C) 2014 Elsevier Ltd. All rights reserved

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