Combining Valosin-containing Protein (VCP) Inhibition and Suberanilohydroxamic Acid (SAHA) Treatment Additively Enhances the Folding, Trafficking, and Function of Epilepsy-associated γ-Aminobutyric Acid, Type A (GABAA) Receptors

GABAA receptors are the primary inhibitory ion channels in the mammalian central nervous system. The A322D mutation in the α1 subunit results in its excessive endoplasmic reticulum-associated degradation at the expense of plasma membrane trafficking, leading to autosomal dominant juvenile myoclonic...

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Veröffentlicht in:	The Journal of biological chemistry 2015-01, Vol.290 (1), p.325-337
Hauptverfasser:	Han, Dong-Yun, Di, Xiao-Jing, Fu, Yan-Lin, Mu, Ting-Wei
Format:	Artikel
Sprache:	eng
Schlagworte:	Action Potentials - drug effects Action Potentials - physiology Adenosine Triphosphatases - antagonists & inhibitors Adenosine Triphosphatases - genetics Adenosine Triphosphatases - metabolism Adolescent Cell Cycle Proteins - antagonists & inhibitors Cell Cycle Proteins - genetics Cell Cycle Proteins - metabolism Cell Line, Tumor Chlorides - metabolism Drug Synergism Dynamin I - genetics Dynamin I - metabolism Endocytosis - drug effects Endoplasmic Reticulum - drug effects Endoplasmic Reticulum - metabolism Endoplasmic Reticulum-Associated Degradation - drug effects Endoplasmic Reticulum-Associated Degradation - genetics Epilepsy ER Quality Control ER-associated Degradation GABA Receptor gamma-Aminobutyric Acid - metabolism HEK293 Cells Humans Hydrazones - pharmacology Hydroxamic Acids - pharmacology Hydroxyurea - analogs & derivatives Hydroxyurea - pharmacology Myoclonic Epilepsy, Juvenile - genetics Myoclonic Epilepsy, Juvenile - metabolism Myoclonic Epilepsy, Juvenile - pathology Neurons - drug effects Neurons - metabolism Neurons - pathology Patch-Clamp Techniques Proteasome Endopeptidase Complex - drug effects Proteasome Endopeptidase Complex - metabolism Protein Folding - drug effects Protein Misfolding Protein Stability - drug effects Protein Synthesis and Degradation Proteostasis Receptors, GABA-A - chemistry Receptors, GABA-A - genetics Receptors, GABA-A - metabolism SAHA Signal Transduction Valosin Containing Protein VCP Vorinostat
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