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A26 Polyamine metabolism in huntington’s disease
  1. Nagehan Ersoy Tunali1,
  2. Mehmet Ali Tüfekçi2
  1. 1İstanbul Medeniyet University, Department of Molecular Biology and Genetics, İstanbul-Turkey
  2. 2Marmara University, Department of Biology, İstanbul-Turkey


Background N-methyl-d-aspartic acid receptor (NMDA) mediated excitotoxic destruction of striatal neurons is one of the models proposed for the pathogenesis of Huntington’s disease. Polyamines (putrescine, spermidine and spermine) may be involved in excitotoxicity by modulating NMDA receptors either in a negative or positive manner.

Aims In the framework of this work, we aimed to investigate the effects of polyamines on the mutant huntingtin mediated excitotoxicity.

Methods HEK293 cells, stably expressing mutant or normal htt were transiently transfected with NMDA receptor heterodimers (NR1/NR2A or NR1/NR2B) and then exposed to polyamines or polyamine biosynthesis inhibitors. Intracellular polyamine levels were determined by HPLC, cell viability was measured by MTT assay and the changes in protein expressions of huntingtin, inhibitor protein kappa B and ornithine decarboxylase were performed by western blotting.

Results It was found that exogenously applied Spd, Spm, α-difluoromethyl ornithine and cyclohexylamine, but not putrescine, cause an increase in the mutant huntingtin aggregates. On the other hand, polyamines and α-difluoromethyl ornithine increased the cell viability in mutant huntingtin expressing cells. Similar results were found for NR1/NR2A and NR1/NR2B expressing cells.

Conclusions The results showed that polyamines may contribute to HD pathology. However, future experiments are required to determine their contribution in detail.

  • polyamines
  • excitotoxicity

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