'Tissue' transglutaminase ablation reduces neuronal death and prolongs survival in a mouse model of Huntington's disease

P. G. Mastroberardino, C. Iannicola, R. Nardacci, F. Bernassola, V. De Laurenzi, G. Melino, S. Moreno, F. Pavone, S. Oliverio, L. Fesus, M. Piacentini

Research output: Contribution to journalArticlepeer-review

Abstract

By crossing Huntington's disease (HD) R6/1 transgenic mice with 'tissue' transglutaminase (TG2) knock-out mice, we have demonstrated that this multifunctional enzyme plays an important role in the neuronal death characterising this disorder in vivo. In fact, a large reduction in cell death is observed in R6/1, TG2-/- compared with R6/1 transgenic mice. In addition, we have shown that the formation of neuronal intranuclear inclusions (NII) is potentiated in absence of the 'tissue' transglutaminase. These phenomena are paralleled by a significant improvement both in motor performances and survival of R6/1, TG2-/- versusR6/1 mice. Taken together these findings suggest an important role for tissue transglutaminase in the regulation of neuronal cell death occurring in Huntington's disease.

Original languageEnglish
Pages (from-to)873-880
Number of pages8
JournalCell Death and Differentiation
Volume9
Issue number9
DOIs
Publication statusPublished - Sep 2002

Keywords

  • ε(γ-glutamyl)lysine crosslinks
  • Autophagy
  • Huntingtin
  • Neurodegeneration
  • Nuclear inclusions

ASJC Scopus subject areas

  • Cell Biology

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