title: Fibrillin-1 and fibrillin-2 in human embryonic and early fetal development.
abstract: The extracellular glycoproteins fibrillin-1 and fibrillin-2 are major components of connective tissue microfibrils. Mutations in the fibrillin-1 and fibrillin-2 genes are responsible for the phenotypical manifestations of Marfan syndrome and congenital contractural arachnodactyly respectively, which emphasizes their essential roles in developmental processes of various tissues. Consistent with this last notion, organ culture experiments have indirectly suggested morphogenic roles for fibrillins in lung and kidney development. In order to contribute to the understanding of the roles of fibrillins in developmental and morphogenetic events, we have investigated the distribution of fibrillin-1 and fibrillin-2 in human embryonic and early fetal tissues between the 5th and the 12th gestational week, i.e. at the beginning of organogenesis. Fibrillin-1 and fibrillin-2 were localized immunohistochemically using specific monoclonal antibodies, mAb 69 and mAb 48, respectively. Both fibrillins are widely distributed in various human anlagen, from early developmental stages. In most embryonic and early fetal human organs such as skin, lung, heart, aorta, central nervous system anlage, nerves, and ganglia, fibrillin-1 and fibrillin-2 follow the same temporo-spatial pattern of distribution. However, in other organs such as kidney, liver, rib anlagen, notochord fibrillin-1 and fibrillin-2 are distributed differentially. The present paper is focused on this aspect. These results suggest different roles for fibrillin-1 and -2 in the development of these structures.
authors.affiliation: Department of Histology, University of Göttingen, Kreuzbergring 36, Germany. fquonda@gwdg.de
authors.forename: Fabio
authors.initials: F
authors.lastname: Quondamatteo
authors.forename: Dieter P
authors.initials: DP
authors.lastname: Reinhardt
authors.forename: Noe L
authors.initials: NL
authors.lastname: Charbonneau
authors.forename: Gabriele
authors.initials: G
authors.lastname: Pophal
authors.forename: Lynn Y
authors.initials: LY
authors.lastname: Sakai
authors.forename: Rainer
authors.initials: R
authors.lastname: Herken
mesh_terms: D004622:Embryo, Mammalian / Q000378:metabolism*; D005314:Embryonic and Fetal Development; D005260:Female; D000071838:Fibrillin-1; D000071840:Fibrillin-2; D000071837:Fibrillins; D006321:Heart / Q000196:embryology; D006801:Humans; D007668:Kidney / Q000196:embryology; D008099:Liver / Q000196:embryology; D008168:Lung / Q000196:embryology; D008840:Microfilament Proteins / Q000378:metabolism*; D011247:Pregnancy; D011261:Pregnancy Trimester, First; D012272:Ribs / Q000196:embryology; D013131:Spine / Q000196:embryology; D014018:Tissue Distribution; D054392:Adipokines
chemical_list: D000071838:Fibrillin-1; D000071840:Fibrillin-2; D000071837:Fibrillins; D008840:Microfilament Proteins; C000605722:FBN1 protein, human; D054392:Adipokines; C000605723:FBN2 protein, human