Comprehensive Characterization of Specific Transcriptional Alterations in the Retina from SCA7 Mice

<div><p>mRNA levels were quantified by real-time PCR of reverse transcribed RNA from 2-mo-old WT and R7E retina. Expression levels are represented as a percentage of the mean of WT littermate mice after normalization to<i>Ppia</i> or<i>Arbp</i> levels. In each panel, each bar represents the mean value ± the standard error of the mean (SEM) (<i>n</i> = 3–6).</p> <p>(A) Down-regulation of genes encoding transcription factors,<i>Crx, Nrl,</i> and<i>Nr2e3,</i> involved in photoreceptor differentiation and maintenance.</p> <p>(B) Expression analysis of cone-specific genes,<i>Bcp, Gnat2,</i> and<i>Carr,</i> in WT and R7E animals.</p> <p>(C) Severe down-regulation of rod-specific genes,<i>Rho, Gnat1, Pde6b, Rhok, Rom,</i> and<i>Rbp3,</i> encoding components of the phototransduction machinery.</p>