The mannan-binding lectin pathway and lung disease in cystic fibrosis—dysfunction of mannan-binding lectin-associated serine protease 2 (MASP-2) may be a major …
HV Olesen, JC Jensenius, R Steffensen, S Thiel… - Clinical …, 2006 - Elsevier
The lectin pathway of complement activation is initiated by mannan-binding lectin (MBL) or
the ficolins through the common MBL-associated serine protease-2 (MASP-2). Deficiency of
MBL has been associated with poorer outcome in cystic fibrosis (CF). We investigated the
MBL pathway further by analysis of the MASP-2 deficiency mutation (D105G) as well as MBL-
2 genotypes. Concentrations and genotypes of MASP-2 and MBL in 109 CF patients were
correlated to lung function and chronic infections. We describe the first CF patient …
the ficolins through the common MBL-associated serine protease-2 (MASP-2). Deficiency of
MBL has been associated with poorer outcome in cystic fibrosis (CF). We investigated the
MBL pathway further by analysis of the MASP-2 deficiency mutation (D105G) as well as MBL-
2 genotypes. Concentrations and genotypes of MASP-2 and MBL in 109 CF patients were
correlated to lung function and chronic infections. We describe the first CF patient …