Effects of cholestanol feeding on corneal dystrophy in mice.
A cholestanol-enriched diet administered for 8 months to BALB/c mice produced in 20% two kinds of corneal opacities resembling calcific band keratopathy and Schnyder's crystalline dystrophy in humans. The concentrations of cholestanol in serum, liver and cornea of the corneal opacity bearing mice were 30-40-times higher than those of normal mice. On the other hand, brain cholestanol level increased only 7-times in the opacity group as compared with that of control group. There was no significant difference in the cholesterol concentrations of serum and several tissues among opacity, non-opacity and the control group. The crystal particles were observed between epithelial basement membrane and superficial stroma by the electron microscopy. Energy dispersive analysis of the particles revealed that the deposits were composed principally of calcium and phosphorus with other crystalline materials, which was presumed to be cholestanol. These results suggest that the cholestanol may deposit in the cornea from elevated serum levels. Deposition of cholestanol in cornea and related area may be a cause of corneal dystrophy in CTX.
Kim
K S
KS
Department of Physiological Chemistry and Nutrition, Faculty of Medicine, University of Tokyo, Japan.
Kano
K
K
Kasama
T
T
Ishii
Y
Y
Yamashita
H
H
Seyama
Y
Y
D000818:Animals; D004083:Cholestanol / Q000009:adverse effects* / Q000737:chemistry; D002851:Chromatography, High Pressure Liquid; D003316:Corneal Diseases / Q000209:etiology* / Q000473:pathology; D003318:Corneal Opacity / Q000209:etiology / Q000473:pathology; D004041:Dietary Fats / Q000009:adverse effects*; D005260:Female; D008297:Male; D051379:Mice; D008807:Mice, Inbred BALB C; D014973:Xanthomatosis / Q000209:etiology / Q000473:pathology
D004041:Dietary Fats; D004083:Cholestanol