Article
Cell culture models to investigate the selective vulnerability of motoneuronal mitochondria to familial ALS-linked G93ASOD1.
The European journal of neuroscience - 1 Jul 2006
Raimondi Andrea, Mangolini Alessandra, Rizzardini Milena, Tartari Silvia, Massari Silvia, Bendotti Caterina, Francolini Maura, Borgese Nica, Cantoni Lavinia, Pietrini Grazia
Abstract excerpt
Mitochondrial damage induced by superoxide dismutase (SOD1) mutants has been proposed to have a causative role in the selective degeneration of motoneurons in amyotrophic lateral sclerosis (ALS). In order to investigate the basis of the tissue specificity of mutant SOD1 we compared the effect of the continuous expression of wild-type or mutant (G93A) human SOD1 on mitochondrial morphology in the NSC-34...
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