A systematic analysis of the complement pathways in patients with neuromyelitis optica indicates alteration but no activation during remission

Nóra Veszeli, G. Füst, Dorottya Csuka, Anita Trauninger, Laszlo Bors, C. Rózsa, Zsuzsanna Nagy, Zita Jobbágy, Kornélia Eizler, Z. Prohászka, L. Varga, Zsolt Illes

Research output: Contribution to journalArticle

8 Citations (Scopus)

Abstract

Neuromyelitis optica (NMO) is an autoimmune demyelinating inflammatory disorder, mediated by pathogenic autoantibodies against aquaporin 4 (AQP4), the main water channel of the central nervous system (CNS). NMO is characterized by local IgG deposition and complement activation within the CNS, but the three complement pathways have not been systematically investigated.We evaluated the overall activation of the classical, alternative, and MBL-lectin pathways in the peripheral blood of 25 patients with AQP4-seropositive NMO spectrum during remission and 113 healthy controls by three ways: (1) we measured the concentrations of native complement proteins of the three pathways [C1-inhibitor (C1-inh), C1q, C4, C3, C5, factor I, factor B, properdin]; (2) the concentrations of complement products suggesting in vivo activation (C1rC1sC1-inh, C3a, C3bBbP, and SC5b-9); and (3) the total activity of the three complement pathways. Additionally we measured levels of C1rC1sC1-inh, C3a, C3bBbP in cerebrospinal fluid (CSF) of 6 patients with relapsing NMO and of 18 patients with relapsing multiple sclerosis (MS).The serological studies indicated that total complement activity of the classical [median (interquartile range) 72 (61-82) vs. 65 (56-73) CH50/mL; p= 0.0122] and of the lectin pathways [73 (59-111) vs. 49 (3-92)%; p= 0.0078)] were elevated compared with the controls, whereas that of the alternative pathway was not significantly different. The levels of C3 [1.1 (0.9-1.3) vs. 1.4 (1.2-1.5). g/L; p

Original languageEnglish
Pages (from-to)200-209
Number of pages10
JournalMolecular Immunology
Volume57
Issue number2
DOIs
Publication statusPublished - Feb 2014

Fingerprint

Neuromyelitis Optica
Aquaporin 4
Lectins
Central Nervous System
Complement C2
Aquaporins
Complement Factor B
Complement Activation
Demyelinating Diseases
Autoantibodies
Multiple Sclerosis
Cerebrospinal Fluid
Complement System Proteins
Immunoglobulin G

Keywords

  • Aquaporin-4
  • C1rC1sC1-inh
  • C3a
  • C3bBbP
  • Cerebrospinal fluid
  • Neuromyelitis optica

ASJC Scopus subject areas

  • Molecular Biology
  • Immunology

Cite this

A systematic analysis of the complement pathways in patients with neuromyelitis optica indicates alteration but no activation during remission. / Veszeli, Nóra; Füst, G.; Csuka, Dorottya; Trauninger, Anita; Bors, Laszlo; Rózsa, C.; Nagy, Zsuzsanna; Jobbágy, Zita; Eizler, Kornélia; Prohászka, Z.; Varga, L.; Illes, Zsolt.

In: Molecular Immunology, Vol. 57, No. 2, 02.2014, p. 200-209.

Research output: Contribution to journalArticle

Veszeli, Nóra ; Füst, G. ; Csuka, Dorottya ; Trauninger, Anita ; Bors, Laszlo ; Rózsa, C. ; Nagy, Zsuzsanna ; Jobbágy, Zita ; Eizler, Kornélia ; Prohászka, Z. ; Varga, L. ; Illes, Zsolt. / A systematic analysis of the complement pathways in patients with neuromyelitis optica indicates alteration but no activation during remission. In: Molecular Immunology. 2014 ; Vol. 57, No. 2. pp. 200-209.
@article{f689b911876c46989fcf79e2bd68ead7,
title = "A systematic analysis of the complement pathways in patients with neuromyelitis optica indicates alteration but no activation during remission",
abstract = "Neuromyelitis optica (NMO) is an autoimmune demyelinating inflammatory disorder, mediated by pathogenic autoantibodies against aquaporin 4 (AQP4), the main water channel of the central nervous system (CNS). NMO is characterized by local IgG deposition and complement activation within the CNS, but the three complement pathways have not been systematically investigated.We evaluated the overall activation of the classical, alternative, and MBL-lectin pathways in the peripheral blood of 25 patients with AQP4-seropositive NMO spectrum during remission and 113 healthy controls by three ways: (1) we measured the concentrations of native complement proteins of the three pathways [C1-inhibitor (C1-inh), C1q, C4, C3, C5, factor I, factor B, properdin]; (2) the concentrations of complement products suggesting in vivo activation (C1rC1sC1-inh, C3a, C3bBbP, and SC5b-9); and (3) the total activity of the three complement pathways. Additionally we measured levels of C1rC1sC1-inh, C3a, C3bBbP in cerebrospinal fluid (CSF) of 6 patients with relapsing NMO and of 18 patients with relapsing multiple sclerosis (MS).The serological studies indicated that total complement activity of the classical [median (interquartile range) 72 (61-82) vs. 65 (56-73) CH50/mL; p= 0.0122] and of the lectin pathways [73 (59-111) vs. 49 (3-92){\%}; p= 0.0078)] were elevated compared with the controls, whereas that of the alternative pathway was not significantly different. The levels of C3 [1.1 (0.9-1.3) vs. 1.4 (1.2-1.5). g/L; p",
keywords = "Aquaporin-4, C1rC1sC1-inh, C3a, C3bBbP, Cerebrospinal fluid, Neuromyelitis optica",
author = "N{\'o}ra Veszeli and G. F{\"u}st and Dorottya Csuka and Anita Trauninger and Laszlo Bors and C. R{\'o}zsa and Zsuzsanna Nagy and Zita Jobb{\'a}gy and Korn{\'e}lia Eizler and Z. Proh{\'a}szka and L. Varga and Zsolt Illes",
year = "2014",
month = "2",
doi = "10.1016/j.molimm.2013.09.010",
language = "English",
volume = "57",
pages = "200--209",
journal = "Molecular Immunology",
issn = "0161-5890",
publisher = "Elsevier Limited",
number = "2",

}

TY - JOUR

T1 - A systematic analysis of the complement pathways in patients with neuromyelitis optica indicates alteration but no activation during remission

AU - Veszeli, Nóra

AU - Füst, G.

AU - Csuka, Dorottya

AU - Trauninger, Anita

AU - Bors, Laszlo

AU - Rózsa, C.

AU - Nagy, Zsuzsanna

AU - Jobbágy, Zita

AU - Eizler, Kornélia

AU - Prohászka, Z.

AU - Varga, L.

AU - Illes, Zsolt

PY - 2014/2

Y1 - 2014/2

N2 - Neuromyelitis optica (NMO) is an autoimmune demyelinating inflammatory disorder, mediated by pathogenic autoantibodies against aquaporin 4 (AQP4), the main water channel of the central nervous system (CNS). NMO is characterized by local IgG deposition and complement activation within the CNS, but the three complement pathways have not been systematically investigated.We evaluated the overall activation of the classical, alternative, and MBL-lectin pathways in the peripheral blood of 25 patients with AQP4-seropositive NMO spectrum during remission and 113 healthy controls by three ways: (1) we measured the concentrations of native complement proteins of the three pathways [C1-inhibitor (C1-inh), C1q, C4, C3, C5, factor I, factor B, properdin]; (2) the concentrations of complement products suggesting in vivo activation (C1rC1sC1-inh, C3a, C3bBbP, and SC5b-9); and (3) the total activity of the three complement pathways. Additionally we measured levels of C1rC1sC1-inh, C3a, C3bBbP in cerebrospinal fluid (CSF) of 6 patients with relapsing NMO and of 18 patients with relapsing multiple sclerosis (MS).The serological studies indicated that total complement activity of the classical [median (interquartile range) 72 (61-82) vs. 65 (56-73) CH50/mL; p= 0.0122] and of the lectin pathways [73 (59-111) vs. 49 (3-92)%; p= 0.0078)] were elevated compared with the controls, whereas that of the alternative pathway was not significantly different. The levels of C3 [1.1 (0.9-1.3) vs. 1.4 (1.2-1.5). g/L; p

AB - Neuromyelitis optica (NMO) is an autoimmune demyelinating inflammatory disorder, mediated by pathogenic autoantibodies against aquaporin 4 (AQP4), the main water channel of the central nervous system (CNS). NMO is characterized by local IgG deposition and complement activation within the CNS, but the three complement pathways have not been systematically investigated.We evaluated the overall activation of the classical, alternative, and MBL-lectin pathways in the peripheral blood of 25 patients with AQP4-seropositive NMO spectrum during remission and 113 healthy controls by three ways: (1) we measured the concentrations of native complement proteins of the three pathways [C1-inhibitor (C1-inh), C1q, C4, C3, C5, factor I, factor B, properdin]; (2) the concentrations of complement products suggesting in vivo activation (C1rC1sC1-inh, C3a, C3bBbP, and SC5b-9); and (3) the total activity of the three complement pathways. Additionally we measured levels of C1rC1sC1-inh, C3a, C3bBbP in cerebrospinal fluid (CSF) of 6 patients with relapsing NMO and of 18 patients with relapsing multiple sclerosis (MS).The serological studies indicated that total complement activity of the classical [median (interquartile range) 72 (61-82) vs. 65 (56-73) CH50/mL; p= 0.0122] and of the lectin pathways [73 (59-111) vs. 49 (3-92)%; p= 0.0078)] were elevated compared with the controls, whereas that of the alternative pathway was not significantly different. The levels of C3 [1.1 (0.9-1.3) vs. 1.4 (1.2-1.5). g/L; p

KW - Aquaporin-4

KW - C1rC1sC1-inh

KW - C3a

KW - C3bBbP

KW - Cerebrospinal fluid

KW - Neuromyelitis optica

UR - http://www.scopus.com/inward/record.url?scp=84886417184&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=84886417184&partnerID=8YFLogxK

U2 - 10.1016/j.molimm.2013.09.010

DO - 10.1016/j.molimm.2013.09.010

M3 - Article

C2 - 24172223

AN - SCOPUS:84886417184

VL - 57

SP - 200

EP - 209

JO - Molecular Immunology

JF - Molecular Immunology

SN - 0161-5890

IS - 2

ER -