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Systemic Lupus Erythematosus and Deficiencies of Early Components of the Complement Classical Pathway
The complement system plays an important role in the innate and acquired immune response against pathogens. It consists of more than 30 proteins found in soluble form or attached to cell membranes. Most complement proteins circulate in inactive forms and can be sequentially activated by the classica...
Autores principales: | , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
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Frontiers Media S.A.
2016
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Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4764694/ https://www.ncbi.nlm.nih.gov/pubmed/26941740 http://dx.doi.org/10.3389/fimmu.2016.00055 |
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author | Macedo, Ana Catarina Lunz Isaac, Lourdes |
author_facet | Macedo, Ana Catarina Lunz Isaac, Lourdes |
author_sort | Macedo, Ana Catarina Lunz |
collection | PubMed |
description | The complement system plays an important role in the innate and acquired immune response against pathogens. It consists of more than 30 proteins found in soluble form or attached to cell membranes. Most complement proteins circulate in inactive forms and can be sequentially activated by the classical, alternative, or lectin pathways. Biological functions, such as opsonization, removal of apoptotic cells, adjuvant function, activation of B lymphocytes, degranulation of mast cells and basophils, and solubilization and clearance of immune complex and cell lysis, are dependent on complement activation. Although the activation of the complement system is important to avoid infections, it also can contribute to the inflammatory response triggered by immune complex deposition in tissues in autoimmune diseases. Paradoxically, the deficiency of early complement proteins from the classical pathway (CP) is strongly associated with development of systemic lupus erythematous (SLE) – mainly C1q deficiency (93%) and C4 deficiency (75%). The aim of this review is to focus on the deficiencies of early components of the CP (C1q, C1r, C1s, C4, and C2) proteins in SLE patients. |
format | Online Article Text |
id | pubmed-4764694 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2016 |
publisher | Frontiers Media S.A. |
record_format | MEDLINE/PubMed |
spelling | pubmed-47646942016-03-03 Systemic Lupus Erythematosus and Deficiencies of Early Components of the Complement Classical Pathway Macedo, Ana Catarina Lunz Isaac, Lourdes Front Immunol Immunology The complement system plays an important role in the innate and acquired immune response against pathogens. It consists of more than 30 proteins found in soluble form or attached to cell membranes. Most complement proteins circulate in inactive forms and can be sequentially activated by the classical, alternative, or lectin pathways. Biological functions, such as opsonization, removal of apoptotic cells, adjuvant function, activation of B lymphocytes, degranulation of mast cells and basophils, and solubilization and clearance of immune complex and cell lysis, are dependent on complement activation. Although the activation of the complement system is important to avoid infections, it also can contribute to the inflammatory response triggered by immune complex deposition in tissues in autoimmune diseases. Paradoxically, the deficiency of early complement proteins from the classical pathway (CP) is strongly associated with development of systemic lupus erythematous (SLE) – mainly C1q deficiency (93%) and C4 deficiency (75%). The aim of this review is to focus on the deficiencies of early components of the CP (C1q, C1r, C1s, C4, and C2) proteins in SLE patients. Frontiers Media S.A. 2016-02-24 /pmc/articles/PMC4764694/ /pubmed/26941740 http://dx.doi.org/10.3389/fimmu.2016.00055 Text en Copyright © 2016 Macedo and Isaac. http://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) or licensor 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 Macedo, Ana Catarina Lunz Isaac, Lourdes Systemic Lupus Erythematosus and Deficiencies of Early Components of the Complement Classical Pathway |
title | Systemic Lupus Erythematosus and Deficiencies of Early Components of the Complement Classical Pathway |
title_full | Systemic Lupus Erythematosus and Deficiencies of Early Components of the Complement Classical Pathway |
title_fullStr | Systemic Lupus Erythematosus and Deficiencies of Early Components of the Complement Classical Pathway |
title_full_unstemmed | Systemic Lupus Erythematosus and Deficiencies of Early Components of the Complement Classical Pathway |
title_short | Systemic Lupus Erythematosus and Deficiencies of Early Components of the Complement Classical Pathway |
title_sort | systemic lupus erythematosus and deficiencies of early components of the complement classical pathway |
topic | Immunology |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4764694/ https://www.ncbi.nlm.nih.gov/pubmed/26941740 http://dx.doi.org/10.3389/fimmu.2016.00055 |
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