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Prion diseases are a group of neurodegenerative disorders that are caused by the deposition of abnormally folded proteins in the brain. Prion diseases primarily involve the brain, but also can affect the eyes and other organs. A new study by scientists at the National Institutes of Health reports that the earliest eye damage from prion disease takes place in the cone photoreceptor cells, specifically in the cilia and the ribbon synapses. Their findings may offer insights on human retinitis pigmentosa, an inherited disease with similar photoreceptor degeneration leading to blindness. Scrapie is closely related to human prion diseases, such as variant, familial, and sporadic Creutzfeldt-Jakob disease CJD. Their work suggests that by interfering with transport through cilia, these aggregates may provide an important early mechanism by which prion infection selectively destroys photoreceptors. Prion protein also was deposited in cones and rods adjacent to ribbon synapses just before the destruction of these structures and death of photoreceptors. Ribbon synapses are specialized neuron connections found in ocular and auditory neural pathways, and their health is critical to the function of retinal photoreceptors in the eye, as well as hair cells in the ear. The identification of disease-associated prion protein, and the correlation with retinal damage, has not been seen previously and is likely to occur in all prion-susceptible species, including people. Rfid Engineering And Its Effects

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