title: Lessons learned from mice deficient in lectin complement pathway molecules.
abstract: The lectin pathway of the complement system is initiated when the pattern-recognition molecules, mannose-binding lectin (MBL), ficolins or collectin-11, bind to invading pathogens or damaged host cells. This leads to activation of MBL/ficolin/collectin-11 associated serine proteases (MASPs), which in turn activate downstream complement components, ultimately leading to elimination of the pathogen. Mice deficient in the key molecules of lectin pathway of complement have been generated in order to build knowledge of the molecular mechanisms of the lectin pathway in health and disease. Despite differences in the genetic arrangements of murine and human orthologues of lectin pathway molecules, the knockout mice have proven to be valuable models to explore the effect of deficiency states in humans. In addition, new insight and unexpected findings on the diverse roles of lectin pathway molecules in complement activation, pathogen infection, coagulation, host tissue injury and developmental biology have been revealed by in vivo investigations. This review provides an overview of the mice deficient in lectin pathway molecules and highlights some of the most important findings that have resulted from studies of these.
authors: Laboratory of Molecular Medicine, Department of Clinical Immunology, Section 7631 Rigshospitalet, Faculty of Health and Medical Sciences, University of Copenhagen, Copenhagen, Denmark.
authors: Ninette
authors: N
authors: Genster
authors: Department of Immunology, Fukushima Medical University School of Medicine, Fukushima, Japan.
authors: Minoru
authors: M
authors: Takahashi
authors: Department of Immunology, Fukushima Medical University School of Medicine, Fukushima, Japan.
authors: Hideharu
authors: H
authors: Sekine
authors: Radioisotope Center, Fukushima Medical University School of Medicine, Fukushima, Japan.
authors: Yuichi
authors: Y
authors: Endo
authors: Laboratory of Molecular Medicine, Department of Clinical Immunology, Section 7631 Rigshospitalet, Faculty of Health and Medical Sciences, University of Copenhagen, Copenhagen, Denmark. Electronic address: garred@post5.tele.dk.
authors: Peter
authors: P
authors: Garred
authors: Fukushima General Hygiene Institute, Fukushima, Japan.
authors: Teizo
authors: T
authors: Fujita
mesh_terms: D000818:Animals; D037582:Complement Pathway, Mannose-Binding Lectin / Q000502:physiology*; D006801:Humans; D037102:Lectins / Q000235:genetics / Q000378:metabolism; D037241:Mannose-Binding Lectins / Q000235:genetics / Q000378:metabolism; D050606:Mannose-Binding Protein-Associated Serine Proteases / Q000235:genetics / Q000378:metabolism; D051379:Mice; D018345:Mice, Knockout; D000097604:Ficolins
chemical_list: D037102:Lectins; D037241:Mannose-Binding Lectins; D050606:Mannose-Binding Protein-Associated Serine Proteases; D000097604:Ficolins
keywords: Complement; Ficolin; Knockout mice; Lectin pathway; MASP; Mannose-binding lectin