Korean J Pediatr.  2015 Jul;58(7):239-244. 10.3345/kjp.2015.58.7.239.

Complement regulation: physiology and disease relevance

• Cho H

• KMID: 2140664
• DOI: http://doi.org/10.3345/kjp.2015.58.7.239

Abstract

The complement system is part of the innate immune response and as such defends against invading pathogens, removes immune complexes and damaged self-cells, aids organ regeneration, confers neuroprotection, and engages with the adaptive immune response via T and B cells. Complement activation can either benefit or harm the host organism; thus, the complement system must maintain a balance between activation on foreign or modified self surfaces and inhibition on intact host cells. Complement regulators are essential for maintaining this balance and are classified as soluble regulators, such as factor H, and membrane-bound regulators. Defective complement regulators can damage the host cell and result in the accumulation of immunological debris. Moreover, defective regulators are associated with several autoimmune diseases such as atypical hemolytic uremic syndrome, dense deposit disease, age-related macular degeneration, and systemic lupus erythematosus. Therefore, understanding the molecular mechanisms by which the complement system is regulated is important for the development of novel therapies for complement-associated diseases.

Keyword

Complement; Autoimmunity

MeSH Terms

Adaptive Immunity
Antigen-Antibody Complex
Autoimmune Diseases
Autoimmunity
B-Lymphocytes
Complement Activation
Complement Factor H
Complement System Proteins*
Glomerulonephritis, Membranoproliferative
Hemolytic-Uremic Syndrome
Immunity, Innate
Lupus Erythematosus, Systemic
Macular Degeneration
Physiology*
Regeneration
Antigen-Antibody Complex
Complement Factor H
Complement System Proteins