INSERM U952, Paris, France
CNRS UMR 7224, Paris, France
Université Pierre et Marie Curie, Paris, France
Fachbereich Biologie, Chemie, Pharmazie, Freie Universität Berlin, Berlin, Germany
RNA Editing and Hyperexcitability Disorders, Max Delbrück Center for Molecular Medicine, Berlin-Buch, Germany
Developmental Neurobiology, Max Delbrück Center for Molecular Medicine, Berlin-Buch, Germany
Information processing in the brain requires a delicate balance between excitation and inhibition. Glycine receptors (GlyR) are involved in inhibitory mechanisms mainly at a synaptic level, but potential novel roles for these receptors recently emerged due to the discovery of posttranscriptional processing. GLR transcripts are edited through enzymatic modification of a single nucleotide leading to amino acid substitution within the neurotransmitter binding domain. RNA editing produces gain-of-function receptors well suited for generation and maintenance of tonic inhibition of neuronal excitability. As neuronal activity deprivation in early stages of development or in epileptic tissue is detrimental to neurons and because RNA editing of GlyR is up-regulated in temporal lobe epilepsy patients with a severe course of disease a pathophysiological role of these receptors emerges. This review contains a state-of-the-art discussion of (patho)physiological implications of GlyR RNA editing.