Hereditary complement deficiencies are usually associated with increased susceptibility to infections and/ or autoimmune diseases. Generally, they are rare： the majority have been detected in no more than a few dozen...Hereditary complement deficiencies are usually associated with increased susceptibility to infections and/ or autoimmune diseases. Generally, they are rare： the majority have been detected in no more than a few dozen individuals. However, due to lack of population-based studies, such cases are usually detected by chance, e.g., in severely ill persons and their family members. An exception is mannan-binding lectin （MBL） deficiency, affecting perhaps 5%-10% of the population. MBL, like collectin-10, -11 and the ficolins （M-, L-, H-）, is a pattern-recognition molecule, cooperating with MBL- associated serine proteases （MASPs） in the initiation of complement activation via the lectin pathway. In contrast, deficiency展开更多
文摘Hereditary complement deficiencies are usually associated with increased susceptibility to infections and/ or autoimmune diseases. Generally, they are rare： the majority have been detected in no more than a few dozen individuals. However, due to lack of population-based studies, such cases are usually detected by chance, e.g., in severely ill persons and their family members. An exception is mannan-binding lectin （MBL） deficiency, affecting perhaps 5%-10% of the population. MBL, like collectin-10, -11 and the ficolins （M-, L-, H-）, is a pattern-recognition molecule, cooperating with MBL- associated serine proteases （MASPs） in the initiation of complement activation via the lectin pathway. In contrast, deficiency
