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Molecular Neurobiology

, Volume 35, Issue 3, pp 266–277 | Cite as

Function and Dysfunction of CNG Channels: Insights from Channelopathies and Mouse Models

  • Martin BielEmail author
  • Stylianos Michalakis
Article

Abstract

Channels directly gated by cyclic nucleotides (CNG channels) are important cellular switches that mediate influx of Na+ and Ca2+ in response to increases in the intracellular concentration of cAMP and cGMP. In photoreceptors and olfactory receptor neurons, these channels serve as final targets for cGMP and cAMP signaling pathways that are initiated by the absorption of photons and the binding of odorants, respectively. CNG channels have been also found in other types of neurons and in non-excitable cells. However, in most of these cells, the physiological role of CNG channels has yet to be determined. CNG channels have a complex heteromeric structure. The properties of individual subunits that assemble in specific stoichiometries to the native channels have been extensively investigated in heterologous expression systems. Recently, mutations in human CNG channel genes leading to inherited diseases (so-called channelopathies) have been functionally characterized. Moreover, mouse knockout models were generated to define the role of CNG channel proteins in vivo. In this review, we will summarize recent insights into the physiological and pathophysiological role of CNG channel proteins that have emerged from genetic studies in mice and humans.

Keywords

CNG Cyclic nucleotide-gated channel Knockout Channelopathies Photoreceptor Olfactory receptor neuron Cyclic nucleotides Retinitis pigmentosa Achromatopsia Vision Olfaction 

Notes

Acknowledgment

This work was supported by the Deutsche Forschungsgemeinschaft.

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Copyright information

© Humana Press Inc. 2007

Authors and Affiliations

  1. 1.Center for Integrated Protein Science, CIPSM and Department Pharmazie—Zentrum für Pharmaforschung, Pharmakologie für NaturwissenschaftenLudwig-Maximilians-Universität MünchenMünchenGermany

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