My name is Francesco Tedesco.
I am a retired professor of Immunology of
the University of Trieste, Italy, and currently,
member of the immuno-rheumatology unit of the Clinical Research Institute,
Instituto Auxologico Italiano, Milan, Italy.
I'm going to talk about complement deficiencies in human,
and I shall specifically discuss the frequency of these deficiencies,
their clinical association, their molecular abnormalities,
the diagnostic tools used for their identification and
the therapeutic approaches currently available to prevent the associated diseases.
To date, inherited deficiencies of nearly all complement components and
regulators have been identified and found to be associated with severe diseases.
This is not unexpected because complement is a major component of
the innate immune system and has developed as
an important defense system throughout
evolution to reach a high degree of complexity in humans.
The system is made up of
more than 30 proteins including components, regulators and receptors.
It plays an important role in host protection against bacteria, viruses,
virus infected cells and fungi,
all of which express pathogen-associated molecular patterns, PAMP.
It contributes to maintain a host homeostasis,
clearing immune complexes from blood and
extravascular fluids and removing apoptotic necrotic cells and cell debris,
expressing damage-associated molecular paterns, DAMP.