Hijacking of GABAA Receptors by Mutant Glycine Receptors

Robert J Harvey

doi:10.1016/j.molmed.2019.08.010

Hijacking of GABA_A Receptors by Mutant Glycine Receptors

Trends Mol Med. 2019 Oct;25(10):823-825. doi: 10.1016/j.molmed.2019.08.010. Epub 2019 Sep 11.

Author

Robert J Harvey¹

Affiliation

¹ School of Health and Sport Sciences, University of the Sunshine Coast, Locked Bag 4, Maroochydore DC, QLD 4558, Australia; Sunshine Coast Health Institute, Birtinya, QLD 4575, Australia. Electronic address: rharvey2@usc.edu.au.

PMID: 31521561
DOI: 10.1016/j.molmed.2019.08.010

Abstract

Startle disease results from mutations in genes encoding inhibitory GlyR α1 and β subunits or the presynaptic glycine transporter GlyT2. However, the most effective therapies are benzodiazepines that potentiate inhibitory GABA_AR function. A recent publication by Zou et al. adds further complexity by suggesting that dominant GlyR α1 mutants assemble into pre- and extrasynaptic GABA_ARs.

Keywords: GABA(A) receptors; GlyT2; benzodiazepines; glycine receptors; hyperekplexia; startle disease.

MeSH terms

Animals
Genes, Dominant
Humans
Hyperekplexia / genetics*
Hyperekplexia / metabolism
Mutation
Receptors, GABA-A / genetics
Receptors, GABA-A / metabolism*
Receptors, Glycine / genetics*
Receptors, Glycine / metabolism

Substances

Receptors, GABA-A
Receptors, Glycine