Complement as a diagnostic tool in immunopathology

The complement system is a complex and autoregulated multistep cascade at theinterface of innate and adaptive immunity. It is activated by immune complexes or apoptotic cells (classical pathway), pathogen-associated glycoproteins (lectin pathway) or a variety of molecular and cellular surfaces (alte...

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Detalles Bibliográficos
Autores: López-Lera, Alberto, Corvillo, Fernando, Nozal, Pilar, Regueiro, José Ramón, Sánchez-Corral, Pilar, López Trascasa, Margarita
Tipo de recurso: artículo
Fecha de publicación:2018
País:España
Institución:Universidad Autónoma de Madrid
Repositorio:Biblos-e Archivo. Repositorio Institucional de la UAM
Idioma:inglés
OAI Identifier:oai:repositorio.uam.es:10486/684665
Acceso en línea:http://hdl.handle.net/10486/684665
https://dx.doi.org/10.1016/j.semcdb.2017.12.017
Access Level:acceso abierto
Palabra clave:Complement system
Immune deficiency
Infection
Autoimmunity
Molecular diagnosis
Monitoring
Medicina
Descripción
Sumario:The complement system is a complex and autoregulated multistep cascade at theinterface of innate and adaptive immunity. It is activated by immune complexes or apoptotic cells (classical pathway), pathogen-associated glycoproteins (lectin pathway) or a variety of molecular and cellular surfaces (alternative pathway). Upon activation, complement triggers the generation of proteolytic fragments that allow the elimination of the activating surface by enhancing inflammation, opsonization, phagocytosis, and cellular lysis. Moreover, complement efficiently discriminates self from non-self surfaces by means of soluble and membrane-bound complement regulators which are critical for innate self-tolerance. Complement deficiency or dysfunction disturb complement homeostasis and give rise to diseases as diverse as bacterial infections, autoimmunity, or renal and neurological disorders. Research on complement-targeted therapies is an expanding field that has already improved the prognosis of severe diseases such as atypical Haemolytic Uremic syndrome or Paroxysmal Nocturnal Haemoglobinuria. Therefore, complement analysis and monitoring provides valuable information with deep implications for diagnosis and therapy. In addition to its important role as an extracellular defense system, it has now become evident that complement is also present intracellularly, and its activation has profound implications for leukocyte survival and function. In this review, we summarize the essential, up-todate information on the use of complement as a diagnostic and therapeutic tool in the clinics