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Purpose: We characterized the response to the extremely low frequency magnetic field (ELF-MF) in an in vitro model of familial Amyotrophic Lateral Sclerosis (fALS), carrying two mutant variants of the superoxide dismutase 1 (SOD1) gene. Materials and methods: SH-SY5Y human neuroblastoma cells, stably over-expressing the wild type, the G93A or the H46R mutant SOD1 cDNA, were exposed to either the ELF-MF (50 Hz, 1 mT) or the sham control field, up to 72 h. Analysis of (i) viability, proliferation and apoptosis, (ii) reactive oxygen species generation, and (iii) assessment of the iron metabolism, were carried out in all clones in response to the MF exposure. Results: We report that 50-Hz MF exposure induces: (i) no change in proliferation and viability; (ii) no modulation of the intracellular superoxide and H2O2 levels; (iii) a significant deregulation in the expression of iron-related genes IRP1, MFRN1 and TfR1, this evidence being exclusive for the SOD1G93A clone and associated with a slight (p =.0512) difference in the total iron content. Conclusions: 50-Hz MF affects iron homeostasis in the in vitro SOD1G93A ALS model. [ABSTRACT FROM AUTHOR]

Detailed assessment of Nogo-A and its receptor NgR in the spinal cord of amyotrophic lateral sclerosis (ALS) models or patients has not been reported previously, and we examined the expression and distribution patterns of Nogo-A and NgR in an ALS mouse model to determine whether these molecules play a role in this disease. As compared with wild-type (WT) mice, transgenic (Tg) mice showed that the expression levels of Nogo-A transiently increased in motor neurons at an age of 10 weeks old (W), while it progressively decreased from 15 to 18 W. NgR expression in motor neurons of the Tg mice increased at 10 W, then progressively decreased from 15 to 18 W. In contrast, there was no significant change in the dorsal lumbar cord or the cerebellum of Tg mice throughout the progression of ALS. This study suggests that the function of Nogo-A may alter under certain conditions and locations, and thus transient overexpression of Nogo-A and NgR in motor neurons of this ALS mouse model at 10 W may represent a survival reaction of these cells under stressful conditions. [ABSTRACT FROM AUTHOR]

In the present study, we used the SOD1 (G93A) mutant transgenic mice as a model of amyotrophic lateral sclerosis (ALS). This model is widely used as a laboratory tool to study experimental treatments in vivo for ALS to investigate new therapeutic strategies for this neurodegenerative disease. Such studies require the objective quantification of different parameters while mice develop the disease. We have applied a battery of different and specific tests: scoring of motor deficits by a trained observer, weighing, survival measure, hanging wire test, rotarod task and electromyography, most of them commonly used to evaluate G93A animals. We have critically compared these methods, showing the significant influence of gender on the onset of symptoms, and the optimal moment to apply each test. These results should be taken into account in future therapeutic assays on this ALS model. [ABSTRACT FROM AUTHOR]