GEOapplication/xmlftp://ftp.ncbi.nlm.nih.gov/geo/series/GSE314nnn/GSE314562/primaryOK200TranscriptomicsMus musculusExpression profiling by high throughput sequencinghttps://www.ncbi.nlm.nih.gov/geo/query/acc.cgi?acc=GSE314562GEOGSEfalseTranscription co-repressors Panky and Panky-like are essential for retinal cone photoreceptor integrity and survival by modulating ganglioside metabolismTo investigate the in vivo functions of mouse Panky and its paralog, Panky-like (Ankrd33b), we generated and analyzed the Panky/Panky-like double knockout (PPL DKO) mice. PPL DKO mice exhibited decreased light-evoked activities, impaired morphology of cone photoreceptors, and disrupted membrane integrity in the cone outer segments and synaptic terminals, followed by cone death at a later stage. RNA-seq analysis revealed that Cerkl gene, whose mutations in human ortholog CERKL cause retinitis pigmentosa (RP26), was upregulated in Panky/Panky-like/Nrl triple KO mouse retinas, in which loss of Nrl converts rods into cones. Our findings suggest that the regulation of Cerkl expression in cone photoreceptors by Panky and Panky-like is essential for cone photoreceptor integrity and survival.2026/07/06GSE314562GSM9401884GSM9401883GSM9401882GSM9401881GSM9401886GSM940188524247314562Mus musculus