Kj. Alghoul et al., THE STRUCTURE OF POSTERIOR SUBCAPSULAR CATARACTS IN THE ROYAL-COLLEGEOF SURGEONS (RCS) RATS, Experimental Eye Research, 67(2), 1998, pp. 163-177
The Royal College of Surgeons (RCS) rat is an animal model for human a
utosomal recessive retinitis pigmentosa. As the retinas of these anima
ls degenerate from two to six weeks after birth, posterior subcapsular
cataracts (PSCs) develop, presumably in response to toxic lipid perox
ides formed by degenerating rod outer segments. Morphologically, these
PSCs are thought to be characterized by a proliferation of dysplastic
bladder-like fibers, or Wedl cells, in the meridional region of the l
ens, that subsequently migrate to, and aggregate at, the posterior pol
e as the PSC. This report presents the results of correlative scanning
(SEM) and transmission (TEM) electron microscopic as well as light mi
croscopic (LM) analysis of the ultrastructure of RCS PSCs, SEM analysi
s of two, four and six week old lenses (n = 6-10 specimens per age gro
up) demonstrated that the PSCs of RCS rats resulted from a growth malf
ormation of the posterior fiber ends from four to six weeks. The PSC i
s composed of markedly enlarged and irregular posterior fiber ends abe
rrantly curved away from the polar axis toward the vitreous rather tha
n overlapping and abutting to form suture branches within and between
concentric growth shells. LM analysis revealed evidence of progressive
ly more numerous, enlarged, and irregular, ovate cellular profiles at
the posterior pole from four to six weeks. However, there was no evide
nce of Wedl cells either within the meridional row region or along a m
igratory path from the equator to the posterior pole at any age. TEM a
nalysis confirmed that the size and abnormal shapes of cellular profil
es were consistent with SEM analysis and that nuclei were never observ
ed within the plaque. In addition, there was considerable variation in
cytoplasmic densities between cells, Also, dense deposits were freque
ntly noted between cells and beneath the capsule. The orientation of p
osterior fiber end profiles to the posterior capsule was 45, 70 and 90
degrees at respectively two, four and six weeks of age. These results
show that RCS PSCs are a consequence of abnormal posterior fiber end
growth culminating in a posterior opacity. (C) 1998 Academic Press.