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Artur Cideciyan; Human Melanopic Circuit in Isolation from Photoreceptor Input:Light Sensitivity and Temporal Profile. Journal of Vision 2018;18(10):1347. doi: 10.1167/18.10.1347.
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Leber congenital amaurosis refers to a group of severe early-onset inherited retinopathies. There are more than 20 causative genes with varied pathophysiological mechanisms resulting in vision loss at the level of the photoreceptors. Some eyes retain near normal photoreceptor and inner retinal structure despite the severe retina-wide loss of photoreceptor function. High luminance stimuli allow recording of pupillary responses driven directly by melanopsin-expressing intrinsically photosensitive retinal ganglion cells. Analyses of these pupillary responses help clarify the fidelity of transmission of light signals from the retina to the brain for patients with no light perception undergoing early phase clinical treatment trials. In addition, these responses serve to define the sensitivity and temporal profile of the human melanopic circuit in isolation from photoreceptor input.
Meeting abstract presented at VSS 2018
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