ArticleClinical and experimental immunology2005
Deficiency of the mannan-binding lectin pathway of complement and poor outcome in cystic fibrosis: bacterial colonization may be decisive for a relationship.
Article in Clinical and experimental immunology, 2005. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 25 papers, 1 of them a synthesis that pooled it.
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
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Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
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Who cites it
25 citing papers in PubMed, 1 synthesis or guideline pooled it, 78 citations in OpenAlex.
- Impact of Gene Modifiers on Cystic Fibrosis Phenotypic Profiles: A Systematic Review.Human mutation · 2024Pooled it
- Role of Mannose-binding Lectin and Association with Microbial Sensitization in a Cohort of Patients with Atopic Dermatitis.Acta dermato-venereologica · 2023Article
- Investigation ofPathogens and global health · 2022Article
- Review
- Cystic Fibrosis Lung Disease Modifiers and Their Relevance in the New Era of Precision Medicine.Genes · 2021Review
- Development of a vaccine against Staphylococcus aureus invasive infections: Evidence based on human immunity, genetics and bacterial evasion mechanisms.FEMS microbiology reviews · 2020Review
- MBL-associated serine proteases (MASPs) and infectious diseases.Molecular immunology · 2015Review
- Primary immunodeficiency in infection-prone children in southern Sweden: occurrence, clinical characteristics and immunological findings.BMC immunology · 2014Article
- Genetic influences on cystic fibrosis lung disease severity.Frontiers in pharmacology · 2013Article
- Defective activities, but not secretions, resulting from gene point mutations of human mannan-binding lectin.Molecular medicine reports · 2012Article
- Mannose-binding lectin (MBL) as prognostic factor in paediatric oncology patients.Clinical and experimental immunology · 2011Article
- Modifier genes in Mendelian disorders: the example of cystic fibrosis.Annals of the New York Academy of Sciences · 2010Review
- Infections of people with complement deficiencies and patients who have undergone splenectomy.Clinical microbiology reviews · 2010Review
- Use of a modeling framework to evaluate the effect of a modifier gene (MBL2) on variation in cystic fibrosis.European journal of human genetics : EJHG · 2010Article
- Update on gene modifiers in cystic fibrosis.Current opinion in pulmonary medicine · 2008Review
- Complex two-gene modulation of lung disease severity in children with cystic fibrosis.The Journal of clinical investigation · 2008Article
- Deficiency of mannan-binding lectin associated serine protease-2 due to missense polymorphisms.Genes and immunity · 2007Article
- Strategies for identifying modifier genes in cystic fibrosis.Proceedings of the American Thoracic Society · 2007Review
- Disease modifying genes in cystic fibrosis: therapeutic option or one-way road?Naunyn-Schmiedeberg's archives of pharmacology · 2006Review
- Genetic susceptibility to infectious diseases: big is beautiful, but will bigger be even better?The Lancet. Infectious diseases · 2006Review
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
7 authors at 2 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
In cystic fibrosis (CF) prognosis concerning lung damage development is highly variable and difficult to predict. Mannan-binding lectin (MBL) deficiency has been reported to be associated with poor outcome in CF lung disease. MBL is a recognition molecule of the MBL pathway of the complement system and is encoded by a gene characterized by a high degree of polymorphism. Some genotypes result in low serum concentrations of MBL. MBL-associated serine protease 2 (MASP-2) is another protein belonging to the MBL pathway. A mutation resulting in low levels of MASP-2 in serum has been described recently. In the present study, 112 CF patients aged 4-54 years were investigated for MBL and MASP-2 genotypes, serum levels of MBL and MASP-2 and the MBL pathway function in serum. No correlation to reduced lung function or need for lung transplantation was seen, either for MBL deficiency, MASP-2 gene mutation or reduced MBL pathway function. However, in the 27 patients colonized with Staphylococcus aureus, MBL-deficient genotypes were associated with decreased lung function. As expected, MBL pathway function in serum was reduced both in MBL-deficient patients and in patients carrying a mutant MASP-2 allele. An unexpected finding was that CF patients had higher serum levels of MBL than healthy controls when corrected for MBL genotype. In conclusion, MBL pathway function was affected both by MBL and by MASP-2 genotypes. However, MBL or MASP-2 levels in serum did not affect the clinical outcome in the cohort of CF patients studied.
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Registered trials
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