PURPOSE: Here, we examined the development, composition, and structural organization of the ciliary zonule of the mouse. Fibrillin 1, a large glycoprotein enriched in force-bearing tissues, is a prominent constituent of the mouse zonule. In humans, mutations in the gene for fibrillin 1 (FBN1) underlie Marfan syndrome (MS), a disorder characterized by lens dislocation and other ocular symptoms. METHODS: Fibrillin expression was analyzed by in situ hybridization. The organization of the zonule was visualized using antibodies to Fbn1, Fbn2, and microfibril-associated glycoprotein-1 (Magp1) in conjunction with 5-ethynyl-2'-deoxyuridine (EdU), an S-phase marker. RESULTS: Microfibrils, enriched in Fbn2 and Magp1, were prominent components of the temporary vascular tunic of the embryonic lens. Fbn2 expression by nonpigmented ciliary epithelial cells diminished postnatally and there was a concomitant increase in Fbn1 expression, especially in cells located in valleys between the ciliary folds. Zonular fibers projected from the posterior pars plicata to the lens in anterior, equatorial, and posterior groupings. The attachment point of the posterior zonular fibers consisted of a dense meshwork of radially oriented microfibrils that we termed the fibrillar girdle. The fibrillar girdle was located directly above the transition zone, a region of the lens epithelium in which cells commit to terminal differentiation. CONCLUSIONS: The development and arrangement of the murine ciliary zonule are similar to those of humans, and consequently the mouse eye may be a useful model in which to study ocular complications of MS.
PURPOSE: Here, we examined the development, composition, and structural organization of the ciliary zonule of the mouse. Fibrillin 1, a large glycoprotein enriched in force-bearing tissues, is a prominent constituent of the mouse zonule. In humans, mutations in the gene for fibrillin 1 (FBN1) underlie Marfan syndrome (MS), a disorder characterized by lens dislocation and other ocular symptoms. METHODS: Fibrillin expression was analyzed by in situ hybridization. The organization of the zonule was visualized using antibodies to Fbn1, Fbn2, and microfibril-associated glycoprotein-1 (Magp1) in conjunction with 5-ethynyl-2'-deoxyuridine (EdU), an S-phase marker. RESULTS: Microfibrils, enriched in Fbn2 and Magp1, were prominent components of the temporary vascular tunic of the embryonic lens. Fbn2 expression by nonpigmented ciliary epithelial cells diminished postnatally and there was a concomitant increase in Fbn1 expression, especially in cells located in valleys between the ciliary folds. Zonular fibers projected from the posterior pars plicata to the lens in anterior, equatorial, and posterior groupings. The attachment point of the posterior zonular fibers consisted of a dense meshwork of radially oriented microfibrils that we termed the fibrillar girdle. The fibrillar girdle was located directly above the transition zone, a region of the lens epithelium in which cells commit to terminal differentiation. CONCLUSIONS: The development and arrangement of the murine ciliary zonule are similar to those of humans, and consequently the mouse eye may be a useful model in which to study ocular complications of MS.
Authors: Gwenaëlle Collod-Béroud; Saga Le Bourdelles; Lesley Ades; Leena Ala-Kokko; Patrick Booms; Maureen Boxer; Anne Child; Paolo Comeglio; Anne De Paepe; James C Hyland; Katerine Holman; Ilkka Kaitila; Bart Loeys; Gabor Matyas; Lieve Nuytinck; Leena Peltonen; Terhi Rantamaki; Peter Robinson; Beat Steinmann; Claudine Junien; Christophe Béroud; Catherine Boileau Journal: Hum Mutat Date: 2003-09 Impact factor: 4.878
Authors: Tomas Majtan; Wendell Jones; Jakub Krijt; Insun Park; Warren D Kruger; Viktor Kožich; Steven Bassnett; Erez M Bublil; Jan P Kraus Journal: Mol Ther Date: 2017-12-19 Impact factor: 11.454