Is the expression of [-G93A(+)] human SOD1 a model to study neurodegenerations?

  • R. Stifanese | stifanese@unige.it
  • M. Averna
  • M. Pedrazzi
  • R. De Tullio
  • F. Salamino
  • S. Pontremoli
  • E. Melloni

Abstract

To relate the alterations occurring in neurodegenerations with Ca2+ homeostasis dysregulation, we analyzed the functional properties of the Ca2+-dependent calpain/calpastatin system in neuronal cells of transgenic mice overexpressing human mutated -G93A(+) SOD1. Motor cortex and spinal cord lower segments from transgenic mice show a very large increase in free Ca2+ ions and evident calpain activation, identified onthe basis of its consumption and substrates digestion. Changes in calpastatin intracellular localization and calpain conformation state further support these observations. Moreover, calpastatin is significantly expressed, counteracting its calpain-mediated degradation. Thus, the calpain /calpastatin system may be a target for new therapeutic approaches to neurodegenerative diseases.

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Published
2011-01-30
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Abstract Book
Keywords:
Ca2 -dependent proteolysis, calpain, calpastatin, NOS, ALS
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How to Cite
Stifanese, R., Averna, M., Pedrazzi, M., De Tullio, R., Salamino, F., Pontremoli, S., & Melloni, E. (2011). Is the expression of [-G93A(+)] human SOD1 a model to study neurodegenerations?. Journal of Biological Research - Bollettino Della Società Italiana Di Biologia Sperimentale, 84(1). https://doi.org/10.4081/jbr.2011.4501