Hereditary Angioedema: Insights into inflammation and allergy
| Autor: | Alberto López-Lera, Coen Maas |
|---|---|
| Rok vydání: | 2019 |
| Předmět: |
0301 basic medicine
Angioedemas Hereditary/immunology Allergy Fibrinolysis/immunology medicine.medical_treatment Immunology Blood Coagulation/immunology Bradykinin Inflammation Disease Review Research Support 03 medical and health sciences chemistry.chemical_compound 0302 clinical medicine Fibrinolysis medicine Bradykinin/immunology Journal Article Hypersensitivity Humans Hereditary/immunology Non-U.S. Gov't Molecular Biology Blood Coagulation Complement System Proteins/immunology business.industry Research Support Non-U.S. Gov't Angioedemas Hereditary Angioedemas Complement System Proteins Inflammation/immunology medicine.disease Phenotype 030104 developmental biology chemistry Coagulation Hereditary angioedema medicine.symptom business 030215 immunology Hypersensitivity/immunology |
| Zdroj: | Molecular Immunology, 112, 378. Elsevier Limited |
| ISSN: | 1872-9142 0161-5890 |
| Popis: | Hereditary Angioedema (HAE) is a rare autosomal recessive bradykinin (BK)-mediated disease characterized by local episodes of non-pitting swelling. Initially considered a complement-mediated disease, novel pathogenic mechanisms uncovered in the last decade have revealed new HAE-associated genes and tight physiological relationships among complement, contact, coagulation, fibrinolysis and inflammation. Uncontrolled production of BK due to inefficient regulation of the plasma contact system, increased activity of contact and coagulation factors or a deficient regulation of BK receptor-triggered intracellular signalling are on the basis of HAE pathology. In this new scenario, HAE can result from different mechanisms that may generate distinct clinical phenotypes of the disease. This review focuses in the recent advances and unsolved challenges in our comprehension of this ever increasingly complex pathology. |
| Databáze: | OpenAIRE |
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