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 modifier
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...
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| Published in: | Clinical Immunology Vol. 121; no. 3; pp. 324 - 331 |
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| Language: | English |
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| Abstract | 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 homozygous for the mutation, a girl with extremely severe lung disease with no other precipitating factors. We suspect total MASP-2 dysfunction to be a major modifier of CF lung disease. However, heterozygosity for the D105G mutation of MASP-2 had no correlation to MBL pathway function or poor lung function. Lung function was higher in the MBL deficiency determining genotypes (XA/YO
+
YO/YO) than in the other genotypes. |
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| AbstractList | 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 homozygous for the mutation, a girl with extremely severe lung disease with no other precipitating factors. We suspect total MASP-2 dysfunction to be a major modifier of CF lung disease. However, heterozygosity for the D105G mutation of MASP-2 had no correlation to MBL pathway function or poor lung function. Lung function was higher in the MBL deficiency determining genotypes (XA/YO+YO/YO) than in the other genotypes. 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 homozygous for the mutation, a girl with extremely severe lung disease with no other precipitating factors. We suspect total MASP-2 dysfunction to be a major modifier of CF lung disease. However, heterozygosity for the D105G mutation of MASP-2 had no correlation to MBL pathway function or poor lung function. Lung function was higher in the MBL deficiency determining genotypes (XA/YO + YO/YO) than in the other genotypes. |
| Author | Schiøtz, P.O. Jensenius, J.C. Olesen, H.V. Steffensen, R. Thiel, S. |
| Author_xml | – sequence: 1 givenname: H.V. surname: Olesen fullname: Olesen, H.V. email: hvo@dadlnet.dk organization: Department of Pediatrics A, Aarhus University Hospital, Skejby Sygehus, Brendstrupgaardsvej 100, DK-8200 Aarhus N, Denmark – sequence: 2 givenname: J.C. surname: Jensenius fullname: Jensenius, J.C. organization: Department of Medical Microbiology and Immunology, University of Aarhus, Denmark – sequence: 3 givenname: R. surname: Steffensen fullname: Steffensen, R. organization: Department of Clinical Immunology, Aalborg Hospital, Aalborg, Denmark – sequence: 4 givenname: S. surname: Thiel fullname: Thiel, S. organization: Department of Medical Microbiology and Immunology, University of Aarhus, Denmark – sequence: 5 givenname: P.O. surname: Schiøtz fullname: Schiøtz, P.O. organization: Department of Pediatrics A, Aarhus University Hospital, Skejby Sygehus, Brendstrupgaardsvej 100, DK-8200 Aarhus N, Denmark |
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| Keywords | Mannan-binding lectin Spirometry Cystic fibrosis Complement activation MBL-associated serine proteases Modifier genes Mannose binding lectin Complement Modifier gene Immunology Pancreatic disease Human Immunopathology Lung disease Serine endopeptidases Respiratory disease Enzyme Exploration Metabolic diseases Genetic disease Peptidases Pulmonary fibrosis Dysfunction Digestive diseases Hydrolases |
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| SubjectTerms | Biological and medical sciences Complement activation Cystic fibrosis Fundamental and applied biological sciences. Psychology Fundamental immunology Immunopathology Mannan-binding lectin MBL-associated serine proteases Medical sciences Modifier genes Spirometry |
| Title | 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 modifier |
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