Complement regulators in human disease: lessons from modern genetics
| Autor: | M K Liszewski, John P. Atkinson |
|---|---|
| Rok vydání: | 2014 |
| Předmět: |
Blood Protein Disorders
medicine.drug_class Complement Membrane Attack Complex Monoclonal antibody Antibodies Monoclonal Humanized Macular Degeneration Immune system Internal Medicine medicine Humans Complement Activation Genetics Polymorphism Genetic biology Complement System Proteins Eculizumab Complement system Complement (complexity) Complement Factor H Immunology Hemolytic-Uremic Syndrome Mutation Alternative complement pathway biology.protein Antibody Complement membrane attack complex medicine.drug |
| Zdroj: | Journal of internal medicine. 277(3) |
| ISSN: | 1365-2796 |
| Popis: | First identified in human serum in the late 19th century as a ‘complement’ to antibodies in mediating bacterial lysis, the complement system emerged more than a billion years ago probably as the first humoral immune system. The contemporary complement system consists of nearly 60 proteins in three activation pathways (classical, alternative and lectin) and a terminal cytolytic pathway common to all. Modern molecular biology and genetics have not only led to further elucidation of the structure of complement system components, but have also revealed function-altering rare variants and common polymorphisms, particularly in regulators of the alternative pathway, that predispose to human disease by creating ‘hyperinflammatory complement phenotypes’. To treat these ‘complementopathies’, a monoclonal antibody against the initiator of the membrane attack complex, C5, has received approval for use. Additional therapeutic reagents are on the horizon. |
| Databáze: | OpenAIRE |
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