Retinal dysfunction, photoreceptor protein dysregulation and neuronal remodelling in the R6/1 mouse model of Huntington's disease

► Retinal phenotype in mouse model of Huntington's disease. ► Retinal change appeared by 13 weeks of age, co-incident with motor dysfunction. ► A functional cone deficit likely arose from loss of cone opsin and transducin protein. ► Ectopic rod photoreceptors and remodelled rod and cone bipolar cells were observed. ► There was an increase in Müller cell gliosis, yet limited cell loss.