title: Synthesis and structural organization of zonular fibers during development and aging.
abstract: Zonular fibers are a specific form of extracellular matrix composed mainly of fibrillins. The purpose of this study was to determine which cells secrete fibrillin-1 during development and aging. A specific guinea pig fibrillin-1 mRNA probe was designed and cloned in order to identify fibrillin-secreting cells in guinea pig eye, using in situ hybridization. Immunofluorescence, with a specific guinea pig monoclonal antibody, was used to compare protein levels at different stages from birth to 35 months of age. Electron microscopy and immunolabeling were used to investigate the organization of zonular microfibril bundles. We identified the cells of non-pigmented epithelium of the ciliary body as the main source of fibrillin secreted into the zonule. Moreover, while mRNA expression decreased during aging, there was no decrease in fibrillin immunoreactivity, as previously described in human aorta. These data indicate a very slow turnover of the zonular microfibrils which can be correlated with the appearance during aging of a new periodic fibrillar structure. This new structure may reflect an increased cross-linking in the long-lived zonular microfibrillar bundles.
authors.affiliation: Institut de Biologie et Chimie des Protéines, UMR 5086 CNRS/UCBL, 7, Passage du Vercors, 69367 Lyon Cedex 07, France.
authors.forename: E
authors.initials: E
authors.lastname: Hanssen
authors.forename: S
authors.initials: S
authors.lastname: Franc
authors.forename: R
authors.initials: R
authors.lastname: Garrone
mesh_terms: D000375:Aging / Q000378:metabolism*; D000595:Amino Acid Sequence; D000818:Animals; D001483:Base Sequence; D018076:DNA, Complementary; D005109:Extracellular Matrix / Q000378:metabolism* / Q000648:ultrastructure; D005123:Eye / Q000254:growth & development / Q000378:metabolism; D000071838:Fibrillin-1; D000071837:Fibrillins; D006168:Guinea Pigs; D006801:Humans; D015151:Immunoblotting; D017403:In Situ Hybridization; D051379:Mice; D008807:Mice, Inbred BALB C; D008840:Microfilament Proteins / Q000235:genetics / Q000378:metabolism*; D008854:Microscopy, Electron / Q000379:methods; D008969:Molecular Sequence Data; D054392:Adipokines
chemical_list: D018076:DNA, Complementary; D000071838:Fibrillin-1; D000071837:Fibrillins; D008840:Microfilament Proteins; C000605722:FBN1 protein, human; D054392:Adipokines; C000605724:Fbn1 protein, mouse