POSH participates in epileptogenesis by increasing the surface expression of the NMDA receptor: a promising therapeutic target for epilepsy

Expert Opin Ther Targets. 2017 Dec;21(12):1083-1094. doi: 10.1080/14728222.2017.1394456. Epub 2017 Oct 27.

Abstract

Objectives: Plenty of SH3 (POSH) was originally found to be a key regulator of neuronal apoptosis, axon outgrowth, and neuronal migration. However, the role of POSH in epilepsy has not been reported.

Methods: We investigated the expression of POSH in patients with intractable temporal epilepsy (TLE) and in a kainic acid (KA)-induced mouse model, and then we performed behavioral, electrophysiological and biochemical analyses after the lentivirus (LV)-mediated knockdown or overexpression of POSH in the KA-induced model.

Results: POSH overexpression shortened the latency of seizure onset, increased the frequency of spontaneous recurrent seizures, and increased the frequency of electrical epileptic discharges, while POSH knockdown had contrasting effects. Whole-cell patch-clamp recordings confirmed that POSH overexpression and knockdown were associated with increased and decreased miniature excitatory postsynaptic currents (mEPSCs) and N-methyl-D-aspartate receptor (NMDAR)-mediated currents, respectively. Finally, co-immunoprecipitation showed that POSH and NMDA receptor subunit 1 (NMDAR1) precipitated with each other, and western blot analysis revealed that the surface expression of NMDAR1 was altered in the hippocampus of epileptic mice.

Conclusion: These results show that POSH plays a critical role in the progression of epileptic seizures via NMDAR trafficking and suggest that the protein is a novel target for the treatment of human epilepsy.

Keywords: NMDA receptor; POSH; electrophysiology; epilepsy; lentivirus; patch clamp; therapeutic target.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Animals
  • Disease Models, Animal
  • Epilepsy / genetics
  • Epilepsy / physiopathology
  • Epilepsy / therapy
  • Epilepsy, Temporal Lobe / genetics
  • Epilepsy, Temporal Lobe / physiopathology*
  • Epilepsy, Temporal Lobe / therapy
  • Female
  • Gene Knockdown Techniques
  • Hippocampus / pathology
  • Humans
  • Kainic Acid / toxicity
  • Lentivirus / genetics
  • Male
  • Mice
  • Mice, Inbred C57BL
  • Molecular Targeted Therapy*
  • Patch-Clamp Techniques
  • Receptors, N-Methyl-D-Aspartate / metabolism*
  • Ubiquitin-Protein Ligases / genetics
  • Ubiquitin-Protein Ligases / metabolism*
  • Young Adult

Substances

  • NMDA receptor A1
  • Receptors, N-Methyl-D-Aspartate
  • SH3RF1 protein, human
  • Ubiquitin-Protein Ligases
  • Kainic Acid