Pathological atx3 expression induces cell perturbations in e. Coli as revealed by biochemical and biophysical investigations

Diletta Ami, Barbara Sciandrone, Paolo Mereghetti, Jacopo Falvo, Tiziano Catelani, Cristina Visentin, Paolo Tortora, Salvador Ventura, Antonino Natalello*, Maria Elena Regonesi*

*Corresponding author for this work

Research output: Contribution to journalArticleResearchpeer-review

5 Citations (Scopus)


Amyloid aggregation of human ataxin-3 (ATX3) is responsible for spinocerebellar ataxia type 3, which belongs to the class of polyglutamine neurodegenerative disorders. It is widely accepted that the formation of toxic oligomeric species is primarily involved in the onset of the disease. For this reason, to understand the mechanisms underlying toxicity, we expressed both a physiological (ATX3-Q24) and a pathological ATX3 variant (ATX3-Q55) in a simplified cellular model, Escherichia coli. It has been observed that ATX3-Q55 expression induces a higher reduction of the cell growth compared to ATX3-Q24, due to the bacteriostatic effect of the toxic oligomeric species. Furthermore, the Fourier transform infrared microspectroscopy investigation, supported by multivariate analysis, made it possible to monitor protein aggregation and the induced cell perturbations in intact cells. In particular, it has been found that the toxic oligomeric species associated with the expression of ATX3-Q55 are responsible for the main spectral changes, ascribable mainly to the cell envelope modifications. A structural alteration of the membrane detected through electron microscopy analysis in the strain expressing the pathological form supports the spectroscopic results.

Original languageEnglish
Article number943
Pages (from-to)1-21
Number of pages21
JournalInternational journal of molecular sciences
Issue number2
Publication statusPublished - 2 Jan 2021


  • Amyloids
  • Ataxin-3 expression
  • Escherichia coli
  • FTIR microspectroscopy
  • Multivariate analysis
  • Oligomer toxicity
  • Protein aggregation


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