The Role of Complement in Hereditary Angioedema
1University College London Hospitals NHS Foundation Trust, Department of Medicine, UK; Cardiometabolic Programme-NIHR UCLH/UCL BRC, London, UK; Amsterdam UMC, University of Amsterdam, Department of Vascular Medicine, Amsterdam, the Netherlands.
Transfusion Medicine Reviews
|November 3, 2019
Summary
Hereditary angioedema (HAE) is caused by low C1 inhibitor levels, leading to attacks. New insights reveal bradykinin
Area of Science:
- Immunology
- Complement System
- Plasma Proteases
Background:
- Hereditary angioedema (HAE) is linked to low C1 inhibitor levels, causing debilitating attacks.
- C1 inhibitor regulates complement, coagulation, and fibrinolytic systems.
- Bradykinin overproduction, due to insufficient C1 inhibitor regulation, is key in HAE pathogenesis.
Purpose of the Study:
- To review recent findings on HAE pathophysiology.
- To discuss the role of bradykinin and C1 inhibitor in HAE.
- To highlight novel therapeutic strategies for HAE.
Main Methods:
- Review of recent scientific literature on hereditary angioedema.
- Analysis of the role of C1 inhibitor in complement, coagulation, and fibrinolysis.
- Examination of the kallikrein-kinin system in HAE pathogenesis.
Main Results:
- Low C1 inhibitor levels lead to angioedema attacks.
- Bradykinin plays a critical role in HAE pathophysiology.
- C1 inhibitor deficiency can activate coagulation and fibrinolytic pathways.
Conclusions:
- Understanding C1 inhibitor's role is crucial for HAE management.
- Bradykinin pathway modulation offers new therapeutic avenues for HAE.
- Novel treatments, including C1 inhibitor concentrates and bradykinin antagonists, are available for HAE.
Related Concept Videos
01:27Complement System
9.3K
The complement system is a group of approximately 20 plasma proteins that strengthen the body's defenses against infections through opsonization, inflammation, and cell lysis. Opsonization involves coating pathogens with complement proteins, making them more recognizable and facilitating phagocyte engulfment. Certain complement proteins induce inflammation that attracts immune cells to the site of infection. Cell lysis involves the destruction of pathogens through the formation of a...
9.3K
00:49Complementation Tests
6.0K
A complementation test is a simple cross to identify whether the two mutations are located on the same gene or different genes. It was first performed by Edward Lewis in the 1940s while working on fruit flies. He developed the test to identify the location and arrangement of different mutations on chromosomes.
Organisms heterozygous for different mutations are crossed pairwise in all combinations. If present on different genes, the mutations can complement each other by providing the missing...
Organisms heterozygous for different mutations are crossed pairwise in all combinations. If present on different genes, the mutations can complement each other by providing the missing...
6.0K
01:30Anticoagulant Drugs: Low-Molecular-Weight Heparins
1.6K
Hemostasis is a crucial process that prevents excessive blood loss from damaged blood vessels. It involves various mechanisms such as vasoconstriction, platelet adhesion and activation, and fibrin formation. The importance of each mechanism depends on the type of vessel injury. In contrast, thrombosis is the abnormal formation of a blood clot within the blood vessels, leading to potential complications if the clot obstructs blood flow. Thrombosis can be caused by increased coagulability of the...
1.6K
01:26Antibody Actions
2.2K
Antibodies, or immunoglobulins, are critical players in the immune system's arsenal against invading pathogens. Produced by B cells and plasma cells, their primary role is to detect and bind to specific antigens, molecules found on the surface of pathogens like bacteria or viruses. Beyond antigen recognition, antibodies perform several vital functions that contribute to immune defense.
Neutralization
Antibodies can bind to pathogens, preventing them from infecting host cells. This process...
Neutralization
Antibodies can bind to pathogens, preventing them from infecting host cells. This process...
2.2K
01:30Antihypertensive Drugs: Angiotensin-Converting Enzyme Inhibitors
2.2K
Angiotensin-converting enzyme (ACE), a vital component of the renin-angiotensin-aldosterone system, is abundant in lung endothelial cells. ACE converts the inactive decapeptide, angiotensin I, into the active octapeptide, angiotensin II. This potent vasoconstrictor narrows blood vessels, increasing resistance to blood flow and elevating blood pressure. Angiotensin II also stimulates aldosterone production, encouraging kidney cells to reabsorb more sodium and water from urine, thereby increasing...
2.2K
02:06Allergic Reactions
31.9K
Overview
31.9K