AIMS To determine whether the short wavelength sensitive (S) cone electroretinogram (ERG) is selectively altered in diabetic patients with and without retinopathy. METHODS Ganzfeld spectral flashes in the presence of bright white background illumination were used to elicit S cone ERGs is 15 non-retinopathic diabetics, 16 background retinopathic diabetics, and 16 age matched normal controls. ...

PURPOSE To investigate macular photoreceptor structure in patients with inherited retinal degeneration using high-resolution images and to correlate the findings with clinical phenotypes and genetic mutations. METHODS Adaptive optics scanning laser ophthalmoscopy (AOSLO) images of photoreceptors were obtained in 16 eyes: five with retinitis pigmentosa (RP), three with cone-rod dystrophy (CRD)...

PURPOSE To examine the involvement of the long (L) and middle (M) wavelength-sensitive cone opsin genes in cone-dominated phenotypes. METHODS Clinical and molecular analyses included family history, color vision testing, full-field electroretinography (ERG), linkage analysis, and mutation detection. RESULTS Eighteen families were recruited that had X-linked retinal disease characterized by ...

PURPOSE To assess macular photoreceptor abnormalities in eyes with retinitis pigmentosa (RP) with preserved central vision using adaptive optics scanning laser ophthalmoscopy (AO-SLO). METHODS Fourteen eyes of 14 patients with RP (best-corrected visual acuity 20/20 or better) and 12 eyes of 12 volunteers underwent a full ophthalmologic examination, fundus autofluorescence, spectral-domain opt...

We consider germs of smooth maps f : (R l ; 0) ! (R n ; c); c 2 C where C is the standard nondegenerate cone of some signature and classify their singularities under the actions of two natural groups of diieomorphisms preserving C. Occuring singularities are subdivided into 3 classes: regular, semiregular and irregular. In the regular case the classiication of singularities is reduced to the cl...

Focal electroretinograms (ERGs) from the green and red cone systems in combination were elicited with a method of rod silent substitution from three young patients with dominant retinitis pigmentosa with reduced penetrance. With this method, two alternating lights of different wavelength, matched in brightness for the rods, were presented to the same retinal area. The brighter light for the gre...

Mutations in the CNGB3 gene account for >50% of all known cases of achromatopsia. Although of early onset, its stationary character and the potential for rapid assessment of restoration of retinal function following therapy renders achromatopsia a very attractive candidate for gene therapy. Here we tested the efficacy of an rAAV2/8 vector containing a human cone arrestin promoter and a human CN...