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Related Experiment Videos

Functional deficiency of complement factor D in a monozygous twin.

H C Kluin-Nelemans, H van Velzen-Blad, H P van Helden

    Clinical and Experimental Immunology
    |December 1, 1984
    PubMed
    Summary

    A partial deficiency in complement factor D caused recurrent bacterial infections in an adult twin. Supplementing with purified factor D restored immune function, indicating its crucial role in the complement system.

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    Area of Science:

    • Immunology
    • Biochemistry

    Background:

    • The complement system is vital for innate immunity, protecting against pathogens.
    • Complement factor D is a key serine protease in the alternative pathway.

    Observation:

    • An adult twin presented with recurrent bacterial infections.
    • The patient exhibited a partial functional deficiency of complement factor D.

    Findings:

    • Reconstitution of patient's serum with purified complement factor D fully restored alternative pathway activity.
    • Zymosan- and cobra venom factor-induced consumption of C3 and factor B was normalized post-supplementation.
    • Family studies showed normal complement factor D levels in other family members.
    • No precursor activity for complement factor D was detected in the patient's serum fractions.

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    Implications:

    • This case highlights the critical role of complement factor D in host defense against bacterial infections.
    • Understanding complement factor D deficiency aids in diagnosing and managing immunodeficiencies.
    • Further research into complement factor D precursor molecules may be warranted.