H-ficolin (ficolin-3) concentrations and FCN3 gene polymorphism in neonates

Immunobiology. 2012 Jul;217(7):730-7. doi: 10.1016/j.imbio.2011.12.004. Epub 2011 Dec 16.

Abstract

Serum H-ficolin (ficolin-3) concentrations (n=613) and FCN3 genotypes (n=529) from a large group of neonates are presented. Both pre-term deliveries and low birthweight (independently of gestational age) were significantly associated with low H-ficolin concentrations but not with heterozygosity for the FCN3 1637delC frameshift mutation. The presence of the variant allele, however, apparently influenced the protein level. No association of FCN3 gene heterozygosity or relative functional H-ficolin insufficiency (determined as serum level ≤8.6 μg/ml) with perinatal infections was found. One premature newborn, with confirmed infection caused by Streptococcus agalactiae, was H-ficolin-deficient (FCN3 variant homozygote, no detectable protein). We present what is only the fourth case report of total H-ficolin deficiency in the world literature. This neonate was however previously found to be mannan-binding lectin (MBL) as well as MBL-associated serine protease-2 (MASP-2) deficient and also had low serum L-ficolin.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Alleles
  • Female
  • Frameshift Mutation
  • Genotype
  • Gestational Age
  • Glycoproteins / deficiency
  • Glycoproteins / genetics*
  • Heterozygote
  • Homozygote
  • Humans
  • Infant, Low Birth Weight
  • Infant, Newborn
  • Lectins / deficiency
  • Lectins / genetics*
  • Male
  • Mannose-Binding Lectin / deficiency
  • Mannose-Binding Lectin / genetics
  • Mannose-Binding Protein-Associated Serine Proteases / deficiency
  • Mannose-Binding Protein-Associated Serine Proteases / genetics
  • Polymorphism, Genetic*
  • Premature Birth / genetics*
  • Premature Birth / immunology
  • Premature Birth / microbiology
  • Streptococcal Infections / genetics*
  • Streptococcal Infections / immunology
  • Streptococcal Infections / microbiology
  • Streptococcus agalactiae / immunology

Substances

  • FCN3 protein, human
  • Glycoproteins
  • Lectins
  • Mannose-Binding Lectin
  • MASP2 protein, human
  • Mannose-Binding Protein-Associated Serine Proteases