vision/eye
• lamellar structure is disrupted, more severely in the posterior stroma
• relatively normal lamellae are separated by abnormal zones in which collagen fibrils are irregularly packed and embedded in an electron lucent substance
• collagen fibrillogenesis is altered, interfibril spacing is increased, and in electron lucent zones fibril diameters are reduced
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• disruption of the lamellar structure is more severe in the posterior stroma
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• the extent of stromal opacity varies from hardly visualized cloudiness to overt opacity
• generally develop by about 1 month of age with a slow progression of opacity afterward
• opacity is more severe in the central cornea
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Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
congenital stromal corneal dystrophy | DOID:0060445 |
OMIM:610048 |
J:177392 |