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Related Concept Videos

Humoral Immune Responses01:36

Humoral Immune Responses

Overview
Complementation Tests00:49

Complementation Tests

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...
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The hosts' susceptibility to infection depends on several factors. The integrity of the skin and mucous membranes helps protect the body against microbial attacks. When the skin is altered, the chance of infection, limb loss, and even death increases.
The integrity and count of the white blood cells help the body resist pathogens and fight infection. When impaired, it reduces the body's resistance to pathogens. The acidic pH levels of the gastrointestinal, genitourinary tracts, and skin create...
Disorders of Hemostasis01:24

Disorders of Hemostasis

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Immunodeficiency Diseases01:25

Immunodeficiency Diseases

Immunodeficiency disorders are conditions in which the immune system's ability to fight infectious disease and cancer is compromised or entirely absent. The immune system comprises a complex network of cells, tissues, and organs that work together to protect the body from potentially harmful invaders. When this system is deficient or not functioning properly, it leaves the body susceptible to infections, diseases, or other complications.
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Complement System01:27

Complement System

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 membrane...

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Related Experiment Video

Updated: Jun 21, 2026

Enrichment of Bruch's Membrane from Human Donor Eyes
10:22

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Published on: November 15, 2015

Inherited deficiencies of complement components in man.

C A Alper

    Immunology Letters
    |February 1, 1987
    PubMed
    Summary

    Inherited complement protein deficiencies increase risks for lupus, bacterial infections, or neisserial infections. Hereditary angioedema, linked to C1 inhibitor deficiency, can be managed with androgens.

    Area of Science:

    • Immunology
    • Genetics
    • Complement System

    Background:

    • Isolated inherited deficiencies of complement proteins are recognized, mostly as autosomal recessive traits.
    • Deficiencies in early components (C1, C4, C2) elevate the risk of immune complex diseases like lupus.
    • Deficiencies in later components (C3, factor I, factor H, properdin, C5-C8) lead to increased susceptibility to specific bacterial or neisserial infections.

    Purpose of the Study:

    • To review the clinical manifestations and genetic basis of inherited complement protein deficiencies.
    • To highlight the association between specific complement deficiencies and distinct disease risks.
    • To discuss the pathophysiology and management of hereditary angioedema due to C1 inhibitor deficiency.

    Main Methods:

    • Review of existing literature on complement deficiencies and associated clinical outcomes.

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  • Analysis of inheritance patterns (autosomal recessive, autosomal dominant) for various deficiencies.
  • Examination of the role of C1 inhibitor deficiency in hereditary angioedema and potential treatments.

    • Main Results:

    • Complement deficiencies are linked to specific health risks: immune complex disease, pyogenic bacterial infections, or neisserial infections.
    • C1 inhibitor deficiency, an autosomal dominant trait, causes angioedema and C4/C2 consumption.
    • A plasmin-modified C2 fragment is implicated in angioedema; androgens may help manage symptoms.

    Conclusions:

    • Inherited complement deficiencies represent a spectrum of immune dysregulation with varied clinical consequences.
    • Understanding these deficiencies is crucial for diagnosis, risk assessment, and targeted management.
    • Hereditary angioedema management may involve addressing biochemical abnormalities and preventing attacks, with androgens showing therapeutic potential.