Learn More
The protein neural retina leucine zipper (Nrl) is a basic motif-leucine zipper transcription factor that is preferentially expressed in rod photoreceptors. It acts synergistically with Crx to regulate rhodopsin transcription. Missense mutations in human NRL have been associated with autosomal dominant retinitis pigmentosa. Here we report that deletion of(More)
PURPOSE To correlate retinal functional changes with structural changes in P23H rhodopsin transgenic rats as a model of autosomal dominant retinitis pigmentosa. METHODS P23H heterozygote (lines 1 and 3) and Sprague-Dawley control rats were studied at 4 to 29 weeks by retinal histology, electroretinogram (ERG), and a-wave transduction modeling. RESULTS(More)
MicroRNA-124a (miR-124a) is the most abundant microRNA expressed in the vertebrate CNS. Despite past investigations into the role of miR-124a, inconsistent results have left the in vivo function of miR-124a unclear. We examined the in vivo function of miR-124a by targeted disruption of Rncr3 (retinal non-coding RNA 3), the dominant source of miR-124a.(More)
PURPOSE To study how the photoreceptoral and postreceptoral ON- and OFF-components contribute to the photopic sine-wave flicker ERG in the monkey by isolating the components with glutamate analogs. METHODS Monkey photopic flicker ERGs were elicited with sine wave stimuli (mean luminance, 2.66 log cd/m(2); 80% modulation depth, on a 40 cd/m(2) white(More)
PURPOSE The b-wave of the human photopic electroretinogram (ERG) elicited by a short-flash increases in amplitude with increasing stimulus intensities at lower stimulus levels, but then decreases at higher stimulus intensities. The purpose of the present study was to explore this phenomenon in more detail, using short- and long-flash stimuli. METHODS The(More)
Exquisitely precise synapse formation is crucial for the mammalian CNS to function correctly. Retinal photoreceptors transfer information to bipolar and horizontal cells at a specialized synapse, the ribbon synapse. We identified pikachurin, an extracellular matrix-like retinal protein, and observed that it localized to the synaptic cleft in the(More)
PURPOSE At lower stimulus intensities, the amplitude of the photopic flash ERG b-wave increases with increasing stimulus intensities, but then plateaus and decreases at higher stimulus intensities (the "photopic hill"). The purpose of this study was to determine the mechanism underlying this unusual phenomenon. METHODS Five adult monkeys (Macaca mulatta(More)
Cilia function as cell sensors in many organs, and their disorders are referred to as "ciliopathies." Although ciliary components and transport machinery have been well studied, regulatory mechanisms of ciliary formation and maintenance are poorly understood. Here we show that male germ cell-associated kinase (Mak) regulates retinal photoreceptor ciliary(More)
PURPOSE To evaluate the relative contributions of photoreceptors and postphotoreceptoral activity to the primate 32-Hz flicker electroretinogram (ERG) elicited by sine-wave, square-wave, and pulse stimuli. METHODS Flicker 32-Hz ERGs were evoked from four adult rhesus (Macaca mulatta) monkeys using sine-wave, square-wave, and 4-ms pulse trains and xenon(More)
Retinal photoreceptor degeneration takes many forms. Mutations in rhodopsin genes or disorders of the retinal pigment epithelium, defects in the adenosine triphosphate binding cassette transporter, ABCR gene defects, receptor tyrosine kinase defects, ciliopathies and transport defects, defects in both transducin and arrestin, defects in rod cyclic guanosine(More)