Cargando…
Retinal Cyclic Nucleotide-Gated Channels: From Pathophysiology to Therapy
The first step in vision is the absorption of photons by the photopigments in cone and rod photoreceptors. After initial amplification within the phototransduction cascade the signal is translated into an electrical signal by the action of cyclic nucleotide-gated (CNG) channels. CNG channels are lig...
Autores principales: | , , |
---|---|
Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
MDPI
2018
|
Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5877610/ https://www.ncbi.nlm.nih.gov/pubmed/29518895 http://dx.doi.org/10.3390/ijms19030749 |
_version_ | 1783310731420106752 |
---|---|
author | Michalakis, Stylianos Becirovic, Elvir Biel, Martin |
author_facet | Michalakis, Stylianos Becirovic, Elvir Biel, Martin |
author_sort | Michalakis, Stylianos |
collection | PubMed |
description | The first step in vision is the absorption of photons by the photopigments in cone and rod photoreceptors. After initial amplification within the phototransduction cascade the signal is translated into an electrical signal by the action of cyclic nucleotide-gated (CNG) channels. CNG channels are ligand-gated ion channels that are activated by the binding of cyclic guanosine monophosphate (cGMP) or cyclic adenosine monophosphate (cAMP). Retinal CNG channels transduce changes in intracellular concentrations of cGMP into changes of the membrane potential and the Ca(2+) concentration. Structurally, the CNG channels belong to the superfamily of pore-loop cation channels and share a common gross structure with hyperpolarization-activated cyclic nucleotide-gated (HCN) channels and voltage-gated potassium channels (KCN). In this review, we provide an overview on the molecular properties of CNG channels and describe their physiological role in the phototransduction pathways. We also discuss insights into the pathophysiological role of CNG channel proteins that have emerged from the analysis of CNG channel-deficient animal models and human CNG channelopathies. Finally, we summarize recent gene therapy activities and provide an outlook for future clinical application. |
format | Online Article Text |
id | pubmed-5877610 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2018 |
publisher | MDPI |
record_format | MEDLINE/PubMed |
spelling | pubmed-58776102018-04-09 Retinal Cyclic Nucleotide-Gated Channels: From Pathophysiology to Therapy Michalakis, Stylianos Becirovic, Elvir Biel, Martin Int J Mol Sci Review The first step in vision is the absorption of photons by the photopigments in cone and rod photoreceptors. After initial amplification within the phototransduction cascade the signal is translated into an electrical signal by the action of cyclic nucleotide-gated (CNG) channels. CNG channels are ligand-gated ion channels that are activated by the binding of cyclic guanosine monophosphate (cGMP) or cyclic adenosine monophosphate (cAMP). Retinal CNG channels transduce changes in intracellular concentrations of cGMP into changes of the membrane potential and the Ca(2+) concentration. Structurally, the CNG channels belong to the superfamily of pore-loop cation channels and share a common gross structure with hyperpolarization-activated cyclic nucleotide-gated (HCN) channels and voltage-gated potassium channels (KCN). In this review, we provide an overview on the molecular properties of CNG channels and describe their physiological role in the phototransduction pathways. We also discuss insights into the pathophysiological role of CNG channel proteins that have emerged from the analysis of CNG channel-deficient animal models and human CNG channelopathies. Finally, we summarize recent gene therapy activities and provide an outlook for future clinical application. MDPI 2018-03-07 /pmc/articles/PMC5877610/ /pubmed/29518895 http://dx.doi.org/10.3390/ijms19030749 Text en © 2018 by the authors. Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (http://creativecommons.org/licenses/by/4.0/). |
spellingShingle | Review Michalakis, Stylianos Becirovic, Elvir Biel, Martin Retinal Cyclic Nucleotide-Gated Channels: From Pathophysiology to Therapy |
title | Retinal Cyclic Nucleotide-Gated Channels: From Pathophysiology to Therapy |
title_full | Retinal Cyclic Nucleotide-Gated Channels: From Pathophysiology to Therapy |
title_fullStr | Retinal Cyclic Nucleotide-Gated Channels: From Pathophysiology to Therapy |
title_full_unstemmed | Retinal Cyclic Nucleotide-Gated Channels: From Pathophysiology to Therapy |
title_short | Retinal Cyclic Nucleotide-Gated Channels: From Pathophysiology to Therapy |
title_sort | retinal cyclic nucleotide-gated channels: from pathophysiology to therapy |
topic | Review |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5877610/ https://www.ncbi.nlm.nih.gov/pubmed/29518895 http://dx.doi.org/10.3390/ijms19030749 |
work_keys_str_mv | AT michalakisstylianos retinalcyclicnucleotidegatedchannelsfrompathophysiologytotherapy AT becirovicelvir retinalcyclicnucleotidegatedchannelsfrompathophysiologytotherapy AT bielmartin retinalcyclicnucleotidegatedchannelsfrompathophysiologytotherapy |