Primary motor neurons from spinal cords of E14 rat embryos were transfected with plasmid DNA usingt he Magnetofectamine kit.
This paper demonstrated the high efficiency of Magnetofectamine kit from OZ Biosciences for DNA delivery into primary motor neurons.article reference: Amyotroph Lateral Scler Frontotemporal Degener. 2014 Oct 22:1-4.
A novel p.E121G SOD1 mutation in slowly progressive form of amyotrophic lateral sclerosis induces cytoplasmic aggregates in cultured motor neurons and reduces cell viability.
Dangoumau A1, Deschamps R, Veyrat-Durebex C, Pettmann B, Corcia P, Andres CR, Vourc'h PAbstract
Mutations
in the SOD1 gene encoding the Cu/Zn superoxide dismutase-1 protein are
responsible for amyotrophic lateral sclerosis (ALS), a fatal
neurodegenerative disease. To date a large number of mutations have been
reported in SOD1, but only few of them have been studied and validated
by functional studies. We present a novel mutation in SOD1 in a female
suffering from slowly progressive ALS. This dominant mutation (c.365A
> G) in exon 5 resulted in a substitution of a highly conserved amino
acid (p.E121G) of the protein. Functional studies in the motor neuronal
cell line NSC34 and in primary culture of mouse motor neurons revealed
that this mutation p.E121G induced aggregates positive for SOD1 and
ubiquitin, as well as reduced cell viability. These findings identified a
novel causal mutation in ALS in close proximity with one of the three
histidine residues (H120) of SOD1 interacting with copper.
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