title: Lack of evidence for oxidative stress in sporadic amyotrophic lateral sclerosis fibroblasts.
abstract: BACKGROUND
  It is conceivable that an early therapeutic intervention in amyotrophic lateral sclerosis (ALS) would lead to better results in terms of disease progression for these patients. One possible strategy to increase the sensitivity of the diagnosis is represented by the use of biological parameters reflecting, for example, oxidative stress alterations associated with ALS. Such biomarkers would be valuable tools both for a better diagnostic evaluation and for studying the impact of therapeutic interventions on the disease course. A special category of experimental models is represented by peripheral cells obtained directly from patients (ex vivo).
  
  
  OBJECTIVE
  In this study, primary fibroblasts obtained from 10 sporadic ALS (SALS) patients and 10 healthy matched controls were used to investigate a panel of parameters related to the oxidative status.
  
  
  METHODS
  Reactive oxygen species production, protein carbonylation and nitration, susceptibility to hydrogen peroxide exposure, p38-mitogen-activated protein kinase activation and adenosine triphosphate intracellular content were evaluated.
  
  
  RESULTS
  No significant difference was observed in all investigated parameters between patient and control cells, and no correlation with the disease severity was found.
  
  
  CONCLUSIONS
  Collectively, our data show no major alterations of the oxidative and bioenergetic status in SALS cultured fibroblasts, suggesting that these cells do not represent a useful model to study the oxidative dysfunction associated with SALS.
authors: Department of Neuroscience and Biomedical Technologies, University of Milano-Bicocca, Milano, Italy. gessica.sala@unimib.it
authors: Gessica
authors: G
authors: Sala
authors: Federica
authors: F
authors: Trombin
authors: Laura
authors: L
authors: Mattavelli
authors: Simone
authors: S
authors: Beretta
authors: Lucio
authors: L
authors: Tremolizzo
authors: Simona
authors: S
authors: Andreoni
authors: Elena
authors: E
authors: Calabrese
authors: Lara
authors: L
authors: Sanvito
authors: Carlo
authors: C
authors: Ferrarese
mesh_terms: D000255:Adenosine Triphosphate / Q000378:metabolism; D000368:Aged; D000690:Amyotrophic Lateral Sclerosis / Q000473:pathology / Q000503:physiopathology*; D002470:Cell Survival / Q000187:drug effects; D002478:Cells, Cultured; D005260:Female; D005347:Fibroblasts / Q000166:cytology / Q000473:pathology / Q000502:physiology*; D005452:Fluoresceins / Q000494:pharmacology; D006801:Humans; D008297:Male; D008875:Middle Aged; D018384:Oxidative Stress; D017382:Reactive Oxygen Species / Q000378:metabolism; D012016:Reference Values
chemical_list: D005452:Fluoresceins; D017382:Reactive Oxygen Species; C029569:diacetyldichlorofluorescein; D000255:Adenosine Triphosphate