The role of GABAA receptor biogenesis, structure and function in epilepsy. (25th October 2006)

• Record Type: Journal Article
• Authors: Mizielinska, S.; Greenwood, S.; Connolly, C.N.
• Is Part Of: Biochemical Society transactions. Volume 34:Number 5(2006)
• Page Start: 863
• Page End: 867
• Publication Date: 2006-10-25
• Subjects: assembly -- benzodiazepine -- biogenesis -- epilepsy -- γ-aminobutyric acid type A receptor (GABAA receptor) -- neurotransmitter; Biochemistry -- Congresses; 572
• DOI: 10.1042/BST0340863
• Languages: English
• ISSNs: 0300-5127

Abstract

Abstract : Maintaining the correct balance in neuronal activation is of paramount importance to normal brain function. Imbalances due to changes in excitation or inhibition can lead to a variety of disorders ranging from the clinically extreme (e.g. epilepsy) to the more subtle (e.g. anxiety). In the brain, the most common inhibitory synapses are regulated by GABAA (γ-aminobutyric acid type A) receptors, a role commensurate with their importance as therapeutic targets. Remarkably, we still know relatively little about GABAA receptor biogenesis. Receptors are constructed as pentameric ion channels, with α and β subunits being the minimal requirement, and the incorporation of a γ subunit being necessary for benzodiazepine modulation and synaptic targeting. Insights have been provided by the discovery of several specific assembly signals within different GABAA receptor subunits. Moreover, a number of recent studies on GABAA receptor mutations associated with epilepsy have further enhanced our understanding of GABAA receptor biogenesis, structure and function.