Purpose. To determine the local i/al ion. distribution and structure of fibiillin micro fihnl s in the lens capsule nf normal indil idual.s and of subjects llith the Marian slndrome (MS), in older to gai n a better understanding of the pathogenesLs of eclopia lentis and imopia in MS. Met h tuts Sections and or flat mounts of lens capsules llere prepared from 6 autopsl eves. '< surgical LlipsLit(iioml spec miens, and capsule.s from patients uith MS lli( immunopeiolidase or imniunolluorescence staining lvere used to detect fibrillin llitli a nionoelon.il jntihodl Image anaKsis ol'the amount of fibrillin expression was performed llitli Jandel Scientific Sottware (JAVA). Results. Based on llbrillin staining patterns. lle jdentilled distmet .nul adiacent /ones in the equatorial and peri-equatorial regions of the normal len- capsule /one I - a d.7 mm-lude peripheral ring ol'the anterior capsule contained radial bundles of tlhrillin libers that appeared to suspend I he central part of the capsule /one II - ,i I mm llide me.shuork of fibrillin-rich fibers encircled the equator and ,seiled as an insertion platform for zonular libers, /one ill - radial. 0 I mm-llide bands were arranged in a peiiodk lashion in the most peripheral part of the posterior capsule. Fihrillin libers llere abnormal and disrupted in all three /ones in patients with MS. Overall. Marian capsule had shorter, liiinnu and less compact fibers. I'he amininl of tlhrillin staining per unit aiea was significantl) reduced in Marian capsules ( Id-26% of unit area) ls. normal |4(J6 n of unit aieail p n 001 j. Conclusions. 1- ibrillin is a major constituent of the peripheral and equatorial areas of the lens capsule. Zonular libers, also rich in llhrillin insert into the equatonal /onular lamella (/one II). Fihrillin mal pla> a role in the abiliK ol'lhe lens to change ils i-onhguration during accommodation. I he observed qualitative and quantitative abnormalities m tlhrillin expression in the lens capsule of patients uith MS support a causal relationship to lens abnormalities in these patients.
|Original language||English (US)|
|Journal||Investigative Ophthalmology and Visual Science|
|State||Published - Dec 1 1997|
ASJC Scopus subject areas
- Sensory Systems
- Cellular and Molecular Neuroscience