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- W4317506051 endingPage "269" @default.
- W4317506051 startingPage "269" @default.
- W4317506051 abstract "The visual process begins with the absorption of photons by photopigments of cone and rod photoreceptors in the retina. In this process, the signal is first amplified by a cyclic guanosine monophosphate (cGMP)-based signaling cascade and then converted into an electrical signal by cyclic nucleotide-gated (CNG) channels. CNG channels are purely ligand-gated channels whose activity can be controlled by cGMP, which induces a depolarizing Na+/Ca2+ current upon binding to the channel. Structurally, CNG channels belong to the superfamily of pore-loop cation channels and share structural similarities with hyperpolarization-activated cyclic nucleotide (HCN) and voltage-gated potassium (KCN) channels. Cone and rod photoreceptors express distinct CNG channels encoded by homologous genes. Mutations in the genes encoding the rod CNG channel (CNGA1 and CNGB1) result in retinitis-pigmentosa-type blindness. Mutations in the genes encoding the cone CNG channel (CNGA3 and CNGB3) lead to achromatopsia. Here, we review the molecular properties of CNG channels and describe their physiological and pathophysiological roles in the retina. Moreover, we summarize recent activities in the field of gene therapy aimed at developing the first gene therapies for CNG channelopathies." @default.
- W4317506051 created "2023-01-20" @default.
- W4317506051 creator A5013075360 @default.
- W4317506051 creator A5021803362 @default.
- W4317506051 creator A5083191480 @default.
- W4317506051 creator A5089190614 @default.
- W4317506051 date "2023-01-19" @default.
- W4317506051 modified "2023-10-04" @default.
- W4317506051 title "Biology, Pathobiology and Gene Therapy of CNG Channel-Related Retinopathies" @default.
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