Mannan-binding lectin-associated serine protease-2 (MASP-2) deficiency in two patients with pulmonary tuberculosis and one healthy control

摘要

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 of other factors of the lectin pathway seems to be very rare.