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I. Masuda, Y. Takada, T. Igarashi, S. Zigler, Y. Yamada, K. Csaky; Lens Pathology in Rescued Perlecan-Knockout Mice. Invest. Ophthalmol. Vis. Sci. 2007;48(13):2001.
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Perlecan is a major heparan sulfatrte proteoglycan (HSPG) found in basement membranes (BMs) and connective tissue. Heparan sulfate(HS) side cahins in the lens capsule has been reported to have a structural function and be essential in the insulation of the lens. The aim of this study is to examine the role for the central core protein of perlecan in lens development.
Perlecan-knock-out mice(prl-/prl-) were rescued by coexpression of perlecan under the control of the mouse type II collagen gene regulatory regions. Paraffin section of eyes from Rescued Perlecan-Knockout mice were stained with hematoxylin-eosin,PAS and In-Situ TUNEL assays. Frozen sentions of eyes were stained with perlecan antibody and expression of adhesion molecules ( cadherin , integrin). ). Lenses from wild-type mice and perlecan-knockout mice were kept in organ culture with either contrtol(DMEM) or perlecan(DMEM+perlecan) for 1,3,7 days. The cataract formation was examined using a dissecting microscope.
Immunostaining for perlecan confirmed the presence of perlecan in lens in prl+/prl+ and prl+/prl- mice but not rescued prl-/prl- mice. Histology of the lens showed evidence of lens degeneration with detachment of the lens epithelium.
The core protein of perlecan play a critical role in lens development, absence of which results in lens epithelial and capsular changes.
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