Expression of a Cu,Zn superoxide dismutase typical of familial amyotrophic lateral sclerosis induces mitochondrial alteration and increase of cytosolic Ca2+ concentration in transfected neuroblastoma SH-SY5Y cells

Maria Teresa Carrì, Alberto Ferri, Andrea Battistoni, Laila Famhy, Roberta Gabbianelli, Fabrizio Poccia, Giuseppe Rotilio

Research output: Contribution to journalArticle


We have set up a model system for familial amyotrophic lateral sclerosis (FALS) by transfecting human neuroblastoma cell line SH-SY5Y with plasmids directing constitutive expression of either wild-type human Cu,Zn superoxide dismutase (Cu,ZnSOD) or a mutant of this enzyme (G93A) associated with FALS. We have tested mitochondrial function and determined cytosolic Ca2+ concentration in control cells (untransfected) and in cells expressing either wild-type Cu,ZnSOD or G93A. We report that G93A induces a significant loss of mitochondrial membrane potential, an increased sensitivity toward valinomycin and a parallel increase in cytosolic Ca2+ concentration. The above phenomena are not related to total Cu,ZnSOD content and activity in the cell.

Original languageEnglish
Pages (from-to)365-368
Number of pages4
JournalFEBS Letters
Issue number2
Publication statusPublished - Sep 8 1997



  • Amyotrophic lateral sclerosis
  • Cytosolic calcium
  • Mitochondrial membrane potential
  • Neurodegeneration
  • Oxidative damage
  • Superoxide dismutase

ASJC Scopus subject areas

  • Biochemistry
  • Biophysics
  • Molecular Biology

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