Datasets for neuronal imaging, extracellular recordings and behavioral rig code

Rod and cone photoreceptors degenerate in retinitis pigmentosa (RP). While
downstream neurons survive, they undergo physiological changes, including
accelerated spontaneous firing in retinal ganglion cells (RGCs). Retinoic
acid (RA) is the molecular trigger of RGC hyperactivity, but whether this
interferes with visual perception is unknown. Here we show that inhibiting
RA synthesis with disulfiram, a deterrent of human alcohol abuse, improves
behavioral image detection in vision-impaired mice. In vivo Ca2+ imaging
shows that disulfiram sharpens orientation-tuning of visual cortical
neurons and strengthens fidelity of responses to natural scenes. An RA
receptor inhibitor also reduces RGC hyperactivity, sharpens cortical
representations, and improves image detection. These findings suggest that
photoreceptor degeneration is not the only cause of vision loss in RP.
RA-induced corruption of retinal information processing also degrades
vision, pointing to RA synthesis and signaling inhibitors as potential
therapeutic tools for improving sight in RP and other retinal degenerative
disorders.