Treatment for Amyotrophic lateral sclerosis (ALS)
Amyotrophic Lateral Sclerosis (ALS)
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Amyotrophic lateral sclerosis (ALS) or motoneuron disease (MND) is a fatal neurodegenerative disease selectively involving brain and spinal cord motoneurons..
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Motor neurons in the brain and spinal cord are selectively destroyed, and most patients die within three years of onset.
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In 97% of patients, aggregates of a protein called TDP-43 are observed in the brain and spinal cord.
G93A SOD1 mutant mouse
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SOD1 mutation is the most prevalent genetic cause in human ALS.
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SOD1 mutant mice are the most widely used animal model for ALS.
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We tested the lysosomal activator (ZCS1) developed by Zincure in these mice, and found that the drug substantially increased the survival and reduced SOD1 and TDP-43 accumulation.
