Jens Christian Jensenius

The mannan-binding lectin pathway and lung disease in cystic fibrosis--disfunction of mannan-binding lectin-associated serine protease 2 (MASP-2) may be a major modifier.

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  • The Department of Paediatrics
  • Department of Medical Microbiology and Immunology
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.
Udgivelsesdato: 2006-Dec
Original languageEnglish
JournalClinical Immunology
Volume121
Issue3
Pages (from-to)324-31
Number of pages7
ISSN1521-6616
DOIs
Publication statusPublished - 2006

    Research areas

  • Adolescent, Adult, Child, Child, Preschool, Cystic Fibrosis, Female, Genotype, Humans, Lung, Male, Mannose-Binding Lectin, Mannose-Binding Protein-Associated Serine Proteases

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