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Complement System

The document discusses the complement system, including how complement proteins become active, which immunoglobulins can activate complement, and the main complement pathways. It also covers complement deficiencies and associated diseases.

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Jess Delapaz
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32 views23 pages

Complement System

The document discusses the complement system, including how complement proteins become active, which immunoglobulins can activate complement, and the main complement pathways. It also covers complement deficiencies and associated diseases.

Uploaded by

Jess Delapaz
Copyright
© © All Rights Reserved
We take content rights seriously. If you suspect this is your content, claim it here.
Available Formats
Download as PDF, TXT or read online on Scribd

COMPLEMENT SYSTEM

Cascade Activation
Complement proteins are often designated by an uppercase letter C
and are inactive until they are split into products.
Example: C1

When the products are split they become active. The active products
are usually designated with a lower case a or b.
Example: C1a and C1b

All classes of immunoglobulin do not fix complement only IgM, IgG3, IgG1
and IgG2 fix in the respective order.

IgG4, IgA, IgD and IgE do not fix complement.

Complement does not bind to free antigen and antibody


Four Important Functions
Overview of the Complement Pathways
General Functions of Complement
Alternative Pathway
Alternative Pathway
Mannose-binding Lectin Pathway
• Lectin is a protein that binds to CHO
• MBL binds to mannose on many bacterial cells
• MBL is produced by liver in acute phase inflammatory reactions
• Once MBL binds to target cell, 2 serine proteases MASP-1, MASP-2
bind
• Acts like C1
• MBL, an acute phase protein, binds to mannose residues
• When MBL complex binds to a pathogen surface, MASP-2 is
activated to cleave C4 and C2
• The MBL pathway is of importance in innate host defense
mechanism in early childhood
Mannose-binding Lectin Pathway
Complement Deficiency and Associated Diseases
Key Facts
Genetic Deficiencies in Classical Pathway Components
Deficiencies in Components of Alternative Pathway
Mutation of the Gene Encoding the Mannose-Binding Lectin
Deficiencies in Complement Regulatory Protein
C1 Inhibitor Deficiency
Deficiencies in Complement Regulatory Protein
CD 59 Deficiency and
Paroxysmal Nocturnal Hemoglobinuria (PNH)

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