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Challenges in diagnostic testing of nephritic factors
Nephritic factors (NeFs) are autoantibodies promoting the activity of the central enzymes of the complement cascade, an important first line of defense of our innate immune system. NeFs stabilize the complement convertase complexes and prevent their natural and regulator-mediated decay. They are mos...
Autores principales: | , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Frontiers Media S.A.
2022
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9702996/ https://www.ncbi.nlm.nih.gov/pubmed/36451820 http://dx.doi.org/10.3389/fimmu.2022.1036136 |
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author | Michels, Marloes A. H. M. Volokhina, Elena B. van de Kar, Nicole C. A. J. van den Heuvel, Lambertus P.W. J. |
author_facet | Michels, Marloes A. H. M. Volokhina, Elena B. van de Kar, Nicole C. A. J. van den Heuvel, Lambertus P.W. J. |
author_sort | Michels, Marloes A. H. M. |
collection | PubMed |
description | Nephritic factors (NeFs) are autoantibodies promoting the activity of the central enzymes of the complement cascade, an important first line of defense of our innate immune system. NeFs stabilize the complement convertase complexes and prevent their natural and regulator-mediated decay. They are mostly associated with rare complement-mediated kidney disorders, in particular with C3 glomerulopathy and related diseases. Although these autoantibodies were already described more than 50 years ago, measuring NeFs for diagnostic purposes remains difficult, and this also complicates our understanding of their clinical associations. In this review, we address the multifactorial challenges of NeF diagnostics. We describe the diseases NeFs are associated with, the heterogenic mechanisms of action of different NeF types, the different methods available in laboratories used for their detection, and efforts for standardization. Finally, we discuss the importance of proper NeF diagnostics for understanding the clinical impact of these autoantibodies in disease pathophysiology and for considering future complement-directed therapy. |
format | Online Article Text |
id | pubmed-9702996 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2022 |
publisher | Frontiers Media S.A. |
record_format | MEDLINE/PubMed |
spelling | pubmed-97029962022-11-29 Challenges in diagnostic testing of nephritic factors Michels, Marloes A. H. M. Volokhina, Elena B. van de Kar, Nicole C. A. J. van den Heuvel, Lambertus P.W. J. Front Immunol Immunology Nephritic factors (NeFs) are autoantibodies promoting the activity of the central enzymes of the complement cascade, an important first line of defense of our innate immune system. NeFs stabilize the complement convertase complexes and prevent their natural and regulator-mediated decay. They are mostly associated with rare complement-mediated kidney disorders, in particular with C3 glomerulopathy and related diseases. Although these autoantibodies were already described more than 50 years ago, measuring NeFs for diagnostic purposes remains difficult, and this also complicates our understanding of their clinical associations. In this review, we address the multifactorial challenges of NeF diagnostics. We describe the diseases NeFs are associated with, the heterogenic mechanisms of action of different NeF types, the different methods available in laboratories used for their detection, and efforts for standardization. Finally, we discuss the importance of proper NeF diagnostics for understanding the clinical impact of these autoantibodies in disease pathophysiology and for considering future complement-directed therapy. Frontiers Media S.A. 2022-11-14 /pmc/articles/PMC9702996/ /pubmed/36451820 http://dx.doi.org/10.3389/fimmu.2022.1036136 Text en Copyright © 2022 Michels, Volokhina, van de Kar and van den Heuvel https://creativecommons.org/licenses/by/4.0/This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms. |
spellingShingle | Immunology Michels, Marloes A. H. M. Volokhina, Elena B. van de Kar, Nicole C. A. J. van den Heuvel, Lambertus P.W. J. Challenges in diagnostic testing of nephritic factors |
title | Challenges in diagnostic testing of nephritic factors |
title_full | Challenges in diagnostic testing of nephritic factors |
title_fullStr | Challenges in diagnostic testing of nephritic factors |
title_full_unstemmed | Challenges in diagnostic testing of nephritic factors |
title_short | Challenges in diagnostic testing of nephritic factors |
title_sort | challenges in diagnostic testing of nephritic factors |
topic | Immunology |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9702996/ https://www.ncbi.nlm.nih.gov/pubmed/36451820 http://dx.doi.org/10.3389/fimmu.2022.1036136 |
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