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...
Ausführliche Beschreibung

Gespeichert in:

Autor*in:	Marloes A. H. M. Michels [verfasserIn] Elena B. Volokhina [verfasserIn] Nicole C. A. J. van de Kar [verfasserIn] Lambertus P.W. J. van den Heuvel [verfasserIn]

Format:	E-Artikel
Sprache:	Englisch

Erschienen:	2022

Schlagwörter:	nephritic factor complement system complement dysregulation complement alternative pathway assay standardization C3 glomerulopathy

Übergeordnetes Werk:	In: Frontiers in Immunology - Frontiers Media S.A., 2011, 13(2022)
Übergeordnetes Werk:	volume:13 ; year:2022

Links:	Link aufrufen Link aufrufen Link aufrufen Journal toc

DOI / URN:	10.3389/fimmu.2022.1036136

Katalog-ID:	DOAJ02641421X

Internformat


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520			\|a 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.
650		4	\|a nephritic factor
650		4	\|a complement system
650		4	\|a complement dysregulation
650		4	\|a complement alternative pathway
650		4	\|a assay standardization
650		4	\|a C3 glomerulopathy
653		0	\|a Immunologic diseases. Allergy
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allfields	10.3389/fimmu.2022.1036136 doi (DE-627)DOAJ02641421X (DE-599)DOAJ7e09eefc2a954f0190b874c0085fbf9e DE-627 ger DE-627 rakwb eng RC581-607 Marloes A. H. M. Michels verfasserin aut Challenges in diagnostic testing of nephritic factors 2022 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier 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. nephritic factor complement system complement dysregulation complement alternative pathway assay standardization C3 glomerulopathy Immunologic diseases. Allergy Elena B. Volokhina verfasserin aut Elena B. Volokhina verfasserin aut Nicole C. A. J. van de Kar verfasserin aut Lambertus P.W. J. van den Heuvel verfasserin aut Lambertus P.W. J. van den Heuvel verfasserin aut Lambertus P.W. J. van den Heuvel verfasserin aut Lambertus P.W. J. van den Heuvel verfasserin aut In Frontiers in Immunology Frontiers Media S.A., 2011 13(2022) (DE-627)657998354 (DE-600)2606827-8 16643224 nnns volume:13 year:2022 https://doi.org/10.3389/fimmu.2022.1036136 kostenfrei https://doaj.org/article/7e09eefc2a954f0190b874c0085fbf9e kostenfrei https://www.frontiersin.org/articles/10.3389/fimmu.2022.1036136/full kostenfrei https://doaj.org/toc/1664-3224 Journal toc kostenfrei GBV_USEFLAG_A SYSFLAG_A GBV_DOAJ SSG-OLC-PHA GBV_ILN_11 GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_73 GBV_ILN_74 GBV_ILN_95 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_206 GBV_ILN_213 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_602 GBV_ILN_2003 GBV_ILN_2014 GBV_ILN_4012 GBV_ILN_4037 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4249 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4700 AR 13 2022
spelling	10.3389/fimmu.2022.1036136 doi (DE-627)DOAJ02641421X (DE-599)DOAJ7e09eefc2a954f0190b874c0085fbf9e DE-627 ger DE-627 rakwb eng RC581-607 Marloes A. H. M. Michels verfasserin aut Challenges in diagnostic testing of nephritic factors 2022 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier 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. nephritic factor complement system complement dysregulation complement alternative pathway assay standardization C3 glomerulopathy Immunologic diseases. Allergy Elena B. Volokhina verfasserin aut Elena B. Volokhina verfasserin aut Nicole C. A. J. van de Kar verfasserin aut Lambertus P.W. J. van den Heuvel verfasserin aut Lambertus P.W. J. van den Heuvel verfasserin aut Lambertus P.W. J. van den Heuvel verfasserin aut Lambertus P.W. J. van den Heuvel verfasserin aut In Frontiers in Immunology Frontiers Media S.A., 2011 13(2022) (DE-627)657998354 (DE-600)2606827-8 16643224 nnns volume:13 year:2022 https://doi.org/10.3389/fimmu.2022.1036136 kostenfrei https://doaj.org/article/7e09eefc2a954f0190b874c0085fbf9e kostenfrei https://www.frontiersin.org/articles/10.3389/fimmu.2022.1036136/full kostenfrei https://doaj.org/toc/1664-3224 Journal toc kostenfrei GBV_USEFLAG_A SYSFLAG_A GBV_DOAJ SSG-OLC-PHA GBV_ILN_11 GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_73 GBV_ILN_74 GBV_ILN_95 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_206 GBV_ILN_213 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_602 GBV_ILN_2003 GBV_ILN_2014 GBV_ILN_4012 GBV_ILN_4037 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4249 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4700 AR 13 2022
allfields_unstemmed	10.3389/fimmu.2022.1036136 doi (DE-627)DOAJ02641421X (DE-599)DOAJ7e09eefc2a954f0190b874c0085fbf9e DE-627 ger DE-627 rakwb eng RC581-607 Marloes A. H. M. Michels verfasserin aut Challenges in diagnostic testing of nephritic factors 2022 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier 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. nephritic factor complement system complement dysregulation complement alternative pathway assay standardization C3 glomerulopathy Immunologic diseases. Allergy Elena B. Volokhina verfasserin aut Elena B. Volokhina verfasserin aut Nicole C. A. J. van de Kar verfasserin aut Lambertus P.W. J. van den Heuvel verfasserin aut Lambertus P.W. J. van den Heuvel verfasserin aut Lambertus P.W. J. van den Heuvel verfasserin aut Lambertus P.W. J. van den Heuvel verfasserin aut In Frontiers in Immunology Frontiers Media S.A., 2011 13(2022) (DE-627)657998354 (DE-600)2606827-8 16643224 nnns volume:13 year:2022 https://doi.org/10.3389/fimmu.2022.1036136 kostenfrei https://doaj.org/article/7e09eefc2a954f0190b874c0085fbf9e kostenfrei https://www.frontiersin.org/articles/10.3389/fimmu.2022.1036136/full kostenfrei https://doaj.org/toc/1664-3224 Journal toc kostenfrei GBV_USEFLAG_A SYSFLAG_A GBV_DOAJ SSG-OLC-PHA GBV_ILN_11 GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_73 GBV_ILN_74 GBV_ILN_95 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_206 GBV_ILN_213 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_602 GBV_ILN_2003 GBV_ILN_2014 GBV_ILN_4012 GBV_ILN_4037 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4249 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4700 AR 13 2022
allfieldsGer	10.3389/fimmu.2022.1036136 doi (DE-627)DOAJ02641421X (DE-599)DOAJ7e09eefc2a954f0190b874c0085fbf9e DE-627 ger DE-627 rakwb eng RC581-607 Marloes A. H. M. Michels verfasserin aut Challenges in diagnostic testing of nephritic factors 2022 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier 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. nephritic factor complement system complement dysregulation complement alternative pathway assay standardization C3 glomerulopathy Immunologic diseases. Allergy Elena B. Volokhina verfasserin aut Elena B. Volokhina verfasserin aut Nicole C. A. J. van de Kar verfasserin aut Lambertus P.W. J. van den Heuvel verfasserin aut Lambertus P.W. J. van den Heuvel verfasserin aut Lambertus P.W. J. van den Heuvel verfasserin aut Lambertus P.W. J. van den Heuvel verfasserin aut In Frontiers in Immunology Frontiers Media S.A., 2011 13(2022) (DE-627)657998354 (DE-600)2606827-8 16643224 nnns volume:13 year:2022 https://doi.org/10.3389/fimmu.2022.1036136 kostenfrei https://doaj.org/article/7e09eefc2a954f0190b874c0085fbf9e kostenfrei https://www.frontiersin.org/articles/10.3389/fimmu.2022.1036136/full kostenfrei https://doaj.org/toc/1664-3224 Journal toc kostenfrei GBV_USEFLAG_A SYSFLAG_A GBV_DOAJ SSG-OLC-PHA GBV_ILN_11 GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_73 GBV_ILN_74 GBV_ILN_95 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_206 GBV_ILN_213 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_602 GBV_ILN_2003 GBV_ILN_2014 GBV_ILN_4012 GBV_ILN_4037 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4249 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4700 AR 13 2022
allfieldsSound	10.3389/fimmu.2022.1036136 doi (DE-627)DOAJ02641421X (DE-599)DOAJ7e09eefc2a954f0190b874c0085fbf9e DE-627 ger DE-627 rakwb eng RC581-607 Marloes A. H. M. Michels verfasserin aut Challenges in diagnostic testing of nephritic factors 2022 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier 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. nephritic factor complement system complement dysregulation complement alternative pathway assay standardization C3 glomerulopathy Immunologic diseases. Allergy Elena B. Volokhina verfasserin aut Elena B. Volokhina verfasserin aut Nicole C. A. J. van de Kar verfasserin aut Lambertus P.W. J. van den Heuvel verfasserin aut Lambertus P.W. J. van den Heuvel verfasserin aut Lambertus P.W. J. van den Heuvel verfasserin aut Lambertus P.W. J. van den Heuvel verfasserin aut In Frontiers in Immunology Frontiers Media S.A., 2011 13(2022) (DE-627)657998354 (DE-600)2606827-8 16643224 nnns volume:13 year:2022 https://doi.org/10.3389/fimmu.2022.1036136 kostenfrei https://doaj.org/article/7e09eefc2a954f0190b874c0085fbf9e kostenfrei https://www.frontiersin.org/articles/10.3389/fimmu.2022.1036136/full kostenfrei https://doaj.org/toc/1664-3224 Journal toc kostenfrei GBV_USEFLAG_A SYSFLAG_A GBV_DOAJ SSG-OLC-PHA GBV_ILN_11 GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_73 GBV_ILN_74 GBV_ILN_95 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_206 GBV_ILN_213 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_602 GBV_ILN_2003 GBV_ILN_2014 GBV_ILN_4012 GBV_ILN_4037 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4249 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4700 AR 13 2022
language	English
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topic_facet	nephritic factor complement system complement dysregulation complement alternative pathway assay standardization C3 glomerulopathy Immunologic diseases. Allergy
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container_title	Frontiers in Immunology
authorswithroles_txt_mv	Marloes A. H. M. Michels @@aut@@ Elena B. Volokhina @@aut@@ Nicole C. A. J. van de Kar @@aut@@ Lambertus P.W. J. van den Heuvel @@aut@@
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callnumber-first	R - Medicine
author	Marloes A. H. M. Michels
spellingShingle	Marloes A. H. M. Michels misc RC581-607 misc nephritic factor misc complement system misc complement dysregulation misc complement alternative pathway misc assay standardization misc C3 glomerulopathy misc Immunologic diseases. Allergy Challenges in diagnostic testing of nephritic factors
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topic_title	RC581-607 Challenges in diagnostic testing of nephritic factors nephritic factor complement system complement dysregulation complement alternative pathway assay standardization C3 glomerulopathy
topic	misc RC581-607 misc nephritic factor misc complement system misc complement dysregulation misc complement alternative pathway misc assay standardization misc C3 glomerulopathy misc Immunologic diseases. Allergy
topic_unstemmed	misc RC581-607 misc nephritic factor misc complement system misc complement dysregulation misc complement alternative pathway misc assay standardization misc C3 glomerulopathy misc Immunologic diseases. Allergy
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title	Challenges in diagnostic testing of nephritic factors
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title_full	Challenges in diagnostic testing of nephritic factors
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title_sort	challenges in diagnostic testing of nephritic factors
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title_auth	Challenges in diagnostic testing of nephritic factors
abstract	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.
abstractGer	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.
abstract_unstemmed	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.
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title_short	Challenges in diagnostic testing of nephritic factors
url	https://doi.org/10.3389/fimmu.2022.1036136 https://doaj.org/article/7e09eefc2a954f0190b874c0085fbf9e https://www.frontiersin.org/articles/10.3389/fimmu.2022.1036136/full https://doaj.org/toc/1664-3224
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