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Low ficolin-2 levels in common variable immunodeficiency patients with bronchiectasis.

Clinical and experimental immunology (2014-09-25)
M-L Metzger, I Michelfelder, S Goldacker, K Melkaoui, J Litzman, D Guzman, B Grimbacher, U Salzer
ZUSAMMENFASSUNG

Common variable immunodeficiency (CVID) encompasses a heterogeneous group of antibody deficiencies characterized by susceptibility to recurrent infections and sequelae, including bronchiectasis. We investigated the relevance of the lectin complement pathway in CVID patients by analysing ficolin-2 and ficolin-3 serum levels and genotyping single nucleotide polymorphisms (SNPs) in the FCN2 and FCN3 genes. Our results show that ficolin-2 levels in CVID patients are significantly lower (P < 0.0001) than in controls. The lowest ficolin-2 levels are found in CVID patients with bronchiectasis (P = 0.0004) and autoimmunity (P = 0.04). Although serum levels of ficolin-3 were similar in CVID patients and controls, CVID patients with bronchiectasis again showed lower levels when compared to controls (P = 0.0001). Analysis of single nucleotide polymorphisms in the FCN2 gene confirmed known influences on ficolin-2 serum levels, but did not support a genetic basis for the observed ficolin-2 deficiency in CVID. We found that CVID patients with bronchiectasis have very low levels of ficolin-2. The reason for the deficiency of ficolin-2 in CVID and any possible causal relationship is currently unknown. However, as bronchiectasis is a very important factor for morbidity and mortality in CVID, ficolin-2 could also serve as biomarker for monitoring disease complications such as bronchiectasis.

MATERIALIEN
Produktnummer
Marke
Produktbeschreibung

Sigma-Aldrich
Diethanolamin, BioUltra, ≥99.5% (GC)
Sigma-Aldrich
Diethanolamin, reagent grade, ≥98.0%
Sigma-Aldrich
Diethanolamin, puriss. p.a., ACS reagent, ≥99.0% (GC)
Sigma-Aldrich
Diethanolamin, ACS reagent, ≥98.5%
Sigma-Aldrich
β-D-Allose, rare aldohexose sugar
Supelco
Diethanolamin, analytical standard
Trolamin Unreinheit B, European Pharmacopoeia (EP) Reference Standard