IBRO Reports · 2017 · 41 citations · 19 references
Life SciencesNeurologyNeuroscienceMolecular NeuroscienceNeurobiology Of DiseaseNeurodegenerationAmyotrophic Lateral SclerosisNeurodegenerative Disease PharmacotherapyNeurodegenerative DiseasesNeurodegenerative DisordersNeurological Disease ModelsNeuroprotectionAmyotrophic Lateral Sclerosis ModelsAmyotrophic Lateral Sclerosis PathologyCopper Chaperone ProteinsCopper ChaperonesCopper HomeostasisDisease Onset TimingKnockout Mouse ModelsTransgenic Mouse Models
A copper chelator known as diacetylbis(N(4)-methylthiosemicarbazonato) copper II (CuATSM), has been reported to be efficacious in multiple transgenic SOD1 models of amyotrophic lateral sclerosis (ALS), a fatal neurodegenerative disorder affecting motor neurons. Here we report that we also observed CuATSM efficacy on disease onset and progression in a standardized litter-matched and gender-balanced efficacy study using B6SJL-SOD1G93A/1Gur mice. We also report improved survival trends with CuATSM treatment. In addition, we report a lack of efficacy by unmetallated ATSM in the same model using the same standardized study design. These results add to existing evidence supporting an efficacious role for copper delivery using chaperone molecules in mouse models of ALS.
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Motor Neuron Degeneration in Mice that Express a Human Cu,Zn Superoxide Dismutase Mutation
Mark E. Gurney, Haifeng Pu, Arlene Y. Chiu et al. · Science · 1994 · 4.1K citations
Increased 3‐nitrotyrosine in both sporadic and familial amyotrophic lateral sclerosis
M. Flint Beal, Robert J. Ferrante, Susan Browne et al. · Annals of Neurology · 1997 · 595 citations · Full text